Hirschprung’s disease in Ghanaian children
Jumah K.B., Yeboah A.A.
Department of Radiology, University of Ghana Medical School
Korle Bu Teaching Hospital, Accra, Ghana
Abstract
This was a retrospective study involving 808 children with the age range of one week to 13 years and mean age of 2.5 years presenting with chronic constipation, referred for barium enema at the Department of Radiology,
Korle Bu Teaching Hospital. Between January 1988 to January 1999, 403 patients (49,9%) from the series showed radiological signs of Hirschprung’s disease as demonstrated on the barium enema : transitional zone, post-stenotic dilatation and significant retention of barium in the 24 hour post evacuation film.
Boys were nearly four times affected than girls and the oldest boy affected was 12 years. Hirschprung’s disease is therefore not uncommon cause of chronic constipation in Ghanaian children.
Key words : Hirschprung’s disease, colon anomalies, constipation.
Hirschprung’s disease or aganglionic megacolon is a congenital anomaly of the nervous innervation of the bowel that affects varying lengths of colon and in some instances, the distal small bowel. Normal developmental innervation of the intestine proceeds from the mouth to the anus as the neuroblasts from the neural crests migrate caudal.
Why the neuroblasts, the precursors of mature ganglion cells, fail to complete their migration to the anus in patients with Hirschprung’s disease is not understood (1,2,3). Clinically, the disease presents with chronic constipation from birth.
Method
We reviewed 808 barium enemas in children with the age range of 1 week to 13 years with the mean age of 2,5 years presenting with chronic constipation at the Korle Bu Teaching Hospital, Department of Radiology and Paediatric Surgery between January, 1998 and January 1999.
The main indication for the enema was to exclude or confirm Hirschprung’s disease. Some patients did have plain abdomen films prior to the barium enema. The barium enemas were performed by radiologists, no bowel preparation procedures were adopted. A straight tipped lubricated catheter was inserted to a point just beyond the anal sphincter. With the patient in the lateral position, barium suspension was introduced slowly by hand injected under flouroscopy and image intensifier, the films were taken in the lateral position for the recto-sigmoid region and then in the supine with the barium filling the descending colon and the proximal colon. Immediate post-evacuation and 24 hour post-evacuation films were obtained routinely.
Results
Of the 808 patients examined 405 (50,1%) were normal, 403 (49,9%) showed radiological signs of Hirschprung’s disease : the demonstration of transitional zone, post-stenotic dilatation and significant retention of barium on the 24 hour post-evacuation films. ( Fig. 1-3.) The plain film findings were constipation or signs of intestinal obstruction. There were 420 (75%) males to 142 (35%) females in the study. The majority of the children (310, 52%) were examined within the first twelve months of age with the youngest being one week old boy while the oldest patients were 12 years old and were only 13 (2,3%) in number. Table 1. shows Histogram demonstrating Number of Patients and Age in years.
Discussion
In 85% of patients with Hirschprung’s disease, there is a short segment of involvement of the rectum or rectosigmoid. In another 10% of patients, the length of bowel lacking innervation extends to the level of splenic flexure.
In the remaining patients, variable segments of the rest of the colon and distal ilieum are affected. Rare instances of significant, even total, small bowel involvement have been reported (3). Our series showed all the lesions confined to the rectum and rectosigmoid.
Gans (3) mentioned two other forms of the disease, ultrashort agangliosis limited to a segment of the anus above the sphincter and a skip variety with one or more normally innervated segments of colon interposed between aganglionic portions of bowel, remain topics of ongoing controversy, claimed by some investigators and questioned by others (3).
The absence of the ganglion cells interrupts the normal propagation of colonic peristalsis. With shorter lengths of colon invovement, patients may present with a history of intermittent constipation or bouts of constipation and diarrhoea, from birth. Some infants who have obstructive signs may develop superimposed becterial entero colitis, which is a potentially life threatening complication.
Aganglionosis of the distal colon results in hypertonic contraction of the affected bowel. The more proximal, normal bowel becomes dilated. Between the two segments, a transition zone of bowel that lacks innervation but that partially distends under the influence of the peristaltic activity of the normal bowel pushing the bowel contents into the contracted distal segment develops. This transition zone generally is funnel-shaped, which is an important diagnostic feature (Figs. 1,2.). Less characteristic variations do occur. In some instances, the transformation from dilated bowel to narrowed bowel is abrupt (4). In other cases, the funneling of the bowel occurs incrementally over a long segment of bowel to appear almost imperceptible because of the gradual change in calibre of the bowel.
In our series, male patients outnumbered female patients by four to one, which is not different from most published series (4,5,6,7). It has been reported that black race is affected less often, but in our series we recorded an average annual incidence of 70 patients. It is however known that the prevalence among patients with trisomy 21 is higher than in the general population but non of our patients were in this category.
The radiologic examination of the colon is an important element in the diagnosis of Hirschprung’s disease. The plain radiograph can document constipation or evidence of obstruction when present, particularly is small infants, the small bowel cannot be distinguished from colonic loops on plain radiograph. The main contribution of plain radiographs is that in the lateral view gas in the colon may occasionally outline the transition zone and the narrowed lumen of the distal colon. Lateral views, which are designed to facilitate the detection of gas in the distal colon, can be helpful. Some authorities recommend invertograms, whereas others opt for prone cross-table lateral views of the abdomen (1). Regardless of the plain radiographic findings, a carefully performed barium enema is the radiographic sine qua non for the diagnosis of Hirschprung’s disease. It is a general rule that, the examination be performed on an unprepared patient who has not undergone cleansing enemas (1,4,7). It is preferable that the patient has not had recent digital examination of the rectum, as it may distort the transition zone. For the same reason, the enema is never performed with an inflated Foley Catheter in the rectum.
Experience has shown that the ionic water soluble contrast agents, especially in the presence of enterocolitis with increased amounts of fluid in the bowel lumen, may fail to define the transition zone (1,7). One serious pitfall is that the rapid infusion of barium suspension can distend and mask the transition zone. The importance of post evacuation and 24 hour post-evacuation delayed radiographs of the abdomen after a barium enema, particularly in questionable cases of megacolon, cannot be overemphasized (Fig.3.). After evacuation of the bowel, an abnormal aganglionis segment, previously distended, may assume a narrowed appearance because of hypertonic contraction. Delayed radiographs may show prolonged retention of barium, which is a strong indicator of Hirschprung’s disease when enema findings have been inconclusive (table 2). In such cases, a rectal biopsy is necessary.
In conclusion, contrary to reports of fewer occurances in black population Hirschprung’s disease is not an uncommon disease in Ghanaian children presenting with chronic constipation (1,4,7).
References
Cremin B :The radiologic assessment of anorectal anomalies.
Clin Radiol 22 : 239, 1971
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Viral encephalitis caused by Ebstein Barr virus infection
Case report
Urbanek K., Gyarmati J., *Budai J.
HUNIKO Diagnostic Imaging Center, Kistarcsa ; * Szent László Hospital
Abstract
A case of a 19 years old girl with Ebstein Barr virus infection is presented. EBV is a very rare pathogene of encephalitis and may mimic tumor symptoms.
Authors discuss the characteristic clinical symptoms and MRI findings.
MRI findings suggested a tumor like lesion in the right central parietal lobe and the final diagnosis was revealed by a cerebral biopsy.
Key words: Encephalitis, EB virus, infection.
EB virus encephalitis is a very rare cause of encephalitis.
EBV infections are occasionally accompained by acute neurological impairment. The pathogenesis of neurological manifestation with EBV infection consists of primary inflammation of EBV infection and secondary immunologic reactions (1).
Case report
A 19 years old girl was admitted following few hours of haedache and vomiting. Some hours later she developed left sided hemiplegia and facial paresis. There was no mention of previous disease in the history, urgent cranial MRI was performed. On T1 weighted images a hypointense and on T2 weighted images a hyperintense, inhomogenious lesion was seen in the right central parietal lobe which suggested a haemorrhage. Minimal contrast enchancement was seen at the adjacent meninx.(Fig.1)
Cerebral fluid and EEG investigation did not support infectious disease and biopsy was decided upon. Histologically, fibrotic tissue, haemorrhage and white blood cells were seen but tumor cells were not found.
Despite the dehydration therapy increased intracranial pressure (30 - 45 Hgmm) and coma developed. Therefore, fronto - temporo - parieto - occipital craniectomy was done. Finding of follow up MRI is seen on figure 2.
After the surgery the patient got better and the paretic signs improved.
6 weeks later the girl could walk and move her arm and head :Follow up MRI study showed regression (Fig 3.). During this time repeated cerebral fluid and blood cells analysis led to the correct diagnosis : Ebstein - Barr virus infection was proved.
4 months after the craniectiomy, the cranial bone replacement was undertaken succesfully.
Discussion
Intracranial inflammatory disease can be classified into : bacterial, viral and fungal infection, (table 1).
Viral encephalitis can produce haemorrhage, edema, necrosis, and vascular congestion.
Pathological features depends on the type of virus, patient age and the immun status. In prenatal age CMV, herpes simplex, and rubeola can cause encephalomalacia, multicystic changes, athrophy, microcrania, brain parenchymal calcification (2-4 ).
In neonates herpes simplex cause progressive diffuse, bihemispheric process and in adults a temporal or biotemporal process.
In our case, it was a very rapid process in the parietal lobe with haemorrhage. The MRI image was similar to a tumor with edema, midline dislocation, inhomogenous increase signal on T2 , inhomogenous decrease signal on T1 and contrast enchancement close to the meninx. The other symptoms of inflammatory disease (blood, cerebrospinal fluid parameter changes, fever..) did not present in the beginning of encephalitis.
Conclusion
What we learnt from this case in the following :
- EBV encephalitis has a wide range of both clinical and radiological findings.
- Certain cases of large space occupaying lesions of infectiosus origin leading to brain stem compression may require surgical management even if their costumary treatment is conservative (5,6).
References
1. Domachowski JB., Cunningham CK., Cummings DL., Crosley CJ., Hannon WP., Weiner LB. : Acute manifestations and neurologic sequele of EBV encephalitis in children.
Pediatr. Infect. Disease J. 10 : 15 (10) :871 - 5, 1996
2. Margolis Mt., Glickman MG., Hoff J. : Focal encephalitis simulating neoplasm Neuroradiology 4,3 - 5., 1972
3. Kasó G., Mészáros J., Hollósy K., Hertelendy Á. : Case of Herpes simplex encephalitis requering surgical treatment.
Orvosi Hetilap 137 - 6, 299 - 302, 1996 (in Hung)
4. Whitley RI., Morawetz RB : Herpes simplex virus infections.
Neurosurgery Mc. Graw-Hill Book Company,vol III. 1987 - 1993, 1985
5. Shain Wj., Chi - Ch : EBV virus encephalitis and encephalomyelitis : MR findings.
Pediatric Radiology 26 : 690 - 3, 1996
Krisztina Urbanek, M.D.
Department of Diagnostic Imaging
Huniko KDK, Semmelweis tér 1.
2143, Kistarcsa, Hungary
Figure 1. MR scans on the first day showed a tumor like lesionon the right parietal lobe. On T1 weighted coronal scan there was an inhomogenous, decreased signal intensity (a) with contrast enchancement wich was seen on T1 weighted axial scan (b).
T2 weighted axial scan showed a huge high signal intense oedematous region close to the surface (c).
Figure 2. MRI scans after the craniectomy showed increased contrast enchancement on T1 weighted coronal scan (a) and the size od oedema is extended (b).
Figure 3. MRI scans 6 weeks later showed a good regression of the lesion on T1 weighted coronal scan with contrast (a) on T2 weighted axial imaging (b).
Table 1.
Bacterial infection - cerebritis, brain abscess
- ventriculitis
- purulent meningitis
- epidural, subdural empyema
Granulomatous disease - tuberculosis
- sarcoidosis
Fungal infection
Parasitic infection - toxoplasmosis
- sarcoidosis
- other parasits
Viral infection - encephalitis
- encephalomeningitis
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Videourodynamic study of neurogenic bladder function
Review article
Szabó L1, Lombay B2, Borbás É2, Tóth V1, Bajusz I1, Losonczi K1, Peja M3, Szabó E3.
Borsod County Teaching Hospital, Child Health Center, Department of Nephrology1, Radiology2 and Rehabilitation3.
Abstract
The study of urodynamics is a method to analyze the function of the upper and lower urinary tract and its controlling central and peripheral nervous system. The greatest urologic risk to the patient with neurogenic disease is the decompensation of their bladder and deterioration of renal function. Videourodynamics is the "gold standard" investigation of these problem. Usefulness of videourodynamic study in different neuropathic conditions are discussed using current data of literature and our experience.
Key words : videourodynamics, neurogen bladder.
Study of urodynamics is still essential for diagnosis of the lower urinary tract dysfunction. The lower urinary tract has two primary functions concerning the transport of urine: adequate storage capacity and efficient emptying capability. These function are governed by a complex physiologic process that involves several centers within the brain, various levels along the spinal cord, the intrinsic properties of the smooth muscles of the bladder and posterior urethra, and the striated muscle component of the external urethral sphincter. These components must all work in a coordinated manner to maintain proper functioning of the lower urinary tract. They are under the control of the somatic and autonomic nervous systems (sympathetic and parasympathetic) [3, 13]. In the assessment of the majority of patients presenting with urinary frequency and urgency, simple cystometry provide all of the necessary information. The conventional procedure was described earlier [19].
Methods for investigation of patients with bladder dysfunction
All children with suspected neurogenic bladder dysfunction should have a urine culture; serum creatinine analysis; a urodynamic assessment and ultrasonogram to determine the status and drainage of the upper urinary tract. The studies should be done soon after an injury occurs or is diagnosed, or, in the case of congenital lesions, in the early postnatal period. The child has uncoordinated detrusor-external sphincter function or elevated pressure during voiding on the urodynamic study (therefore, this group is at high risk for bladder decompensation and/or upper urinary tract deterioration). Combined Voiding Cysto-Urethrography and Cystometry that is videocystometrogram is the gold standard investigation. Videourodynamics documents the presence of reflux, the characteristics of the bladder wall, the appearance of the bladder neck and posterior urethra during both filling and voiding, the presence of urethral obstruction, the emptying capability of the bladder, and evaluates the function of the lower urinary tract. Every attempt is made to reproduce the natural act of voiding [13]. Synchronous Voiding Cysto-Urethrography(VCUG) and Cystometry(CM) recordings are most important in the assessment of complex cases, particularly where different neurological lesions occur, since this investigation allows a combined anatomical and functional evaluation of the lower urinary tract function [10, 20]. In our practice with this procedure, the results of VCUG and CM are recorded on computer. Ultrasound is combined with a flow rate to provide more detailed information on bladder function than these investigations alone. In our practice these result also are recorded on computer and video. Methods, definitions and units conform to the standards recommended by the International Continence Society [13].
Usefulness of Videourodynamic study different neuropathic conditions.
The most common neuropathic conditions affecting vesicourethral function in children are congenital neurospinal dysraphisms. These include myelomeningocele (Fig 1.), lipomeningocele (Fig 2.), sacral agenesis (Fig 3.), and occult lesions. Other traumatic causes of spinal cord or cerebral dysfunction resulting in neurogenic bladder dysfunction are rare in children. [1]. Myelomeningocele is the most common etiology of neurogenic bladder dysfunction in children, with an incidence of one per 1000 live births in North America. Worldwide, however, the incidence is decreasing [18]. Prenatal detection using ultrasound, alpha-fetoprotein examination, and amniocentesis in high risk pregnancies may reduce further the number of children born with this condition [6]. More than 90% of patients with myelomeningocele have some degree of the lower urinary tract dysfunction depending on the level and completeness of the neurological injury [2]. The longitudinal evaluation of children with neurogenic bladder dysfunction has defined a subset at risk for urinary tract deterioration based on certain unfavorable urodynamic patterns [1, 4]. High pressure dyssynergic voiding may result in irreversible damage to the urinary tract. Ultrasound showed a small bladder, a dilated upper urinary tract, and the uroflow an intermittent voiding (Fig. 4a-c). Small bladder with wide bladder neck, many sacculus and small diverticulum and high grade vesico-ureteral reflux on the left side were seen on VCUG and cystometry showed high pressure, low capacity bladder with unstable detrusor contractions (Fig. 4d-e). When uncorrected, bladder outlet obstruction results in sacculation, increased connective tissue and muscle mass, and changes in the regulation of contractile proteins that may have functional significance [14, 17]. When exposed to an increased work load (emptying against increased resistance), bladder smooth muscle has a hyperplastic and a hypertrophic response [11, 14]. Upper urinary tract deterioration and incontinence are the most frequent urological problems in patients with myelodysplasia [17]. Decreased bladder storage and increased emptying pressure resulting from decreased bladder compliance and increased bladder outlet resistance put the upper urinary tract at significantly higher risk for deterioration [4]. Persistently elevated bladder pressure may result in alterations in the cellular and matrix components of the bladder wall, causing decreased compliance [15]. Increased bladder storage and increased sphincter mechanism may also result in upper urinary tract deterioration, but only later. In this situation ultrasound shows a large bladder, uroflow curves are also intermittent with large residual urine, VCUG shows an elongated bladder, and cystometry a better compliance, but many uninhibited detrusor contractions (Fig. 5a-c). The specific parameters that correlate most closely with renal deterioration include dyssynergia between the bladder and striated muscle component of the external urethral sphincter, high filling and/or voiding pressure, decreased bladder compliance and leak point pressure greater than 40 cm of water [4, 5]. Elevated leak point pressure develops in patients with an upper motor neuron lesion due to the failure of relaxation of the external striated sphincter during voiding (Fig.6). In contrast, high leak point pressure in cases of complete lower motor neuron lesions develops due to a fibrotic response of the skeletal muscle to denervation [12] (Fig. 7).
A valuable clinical point is that, because the innervation of the bladder arises, any form of spinal cord disease which causes bladder dysfunction is likely to produce clinical signs in the lower limbs (Fig. 8), unless the lesion is completely restricted to the conus.
The fact, that videourodynamics are sophisticated, time-consuming and require well trained personnel, it has stimulated studies to investigate whether urodynamics could be replaced by other less invasive investigative techniques. Clinical investigation, pinprick sensation and bulbocavernosus reflex are specific tests, but are not sensitive enough; therefore, urodynamics are essential for the complete evaluation of patients with incomplete lumbar-sacral spinal injuries. Clinical evaluation is unable to replace urodynamic studies as an essential component of initial urological evaluation after spinal cord injury [16]. Patients with thoracolumbar spinal injuries, even when neurologically intact, may have neurogenic lower urinary tract dysfunction on urodynamics [21]. In a series of 48 children with daytime wetting, only four showed spinal dysraphism on MRI; however, of the rest, after a urodynamic investigation, 12 out of 51 had clear bladder neuropathy [9].
Damage to the innervation of the lower urinary tract mostly affects the detrusor and the sphincter. These structures may become either hyperreflexic or areflexic, and both can be affected in the same way or differently by the damage. The differentiation of the dysfunctional pattern can only be assessed accurately using urodynamics.
Before the introduction of modern treatments, renal failure was a common cause of death following spinal cord injury. The bladder problems of those with spinal cord injury must therefore be managed in such a way as to lessen the possibility of upper urinary tract disease as well as provide the patient with adequate bladder control for a fully rehabilitated life. Therapy must be based on the underlying pathophysiology. The management of detrusor hyperreflexia with anticholinergic agents, mostly combined with bladder emptying by clean intermittent chateterization (CIC), has proved to be an effective therapeutic strategy, preventing damage to the upper urinary tract and providing continence [8].
Our policy is to begin clean intermittent chateterization and anticholinergic medication in the first few months of life in all patients at high risk for later bladder or upper urinary tract deterioration. Clean intermittent chateterization is a safe and effective way of emptying the bladder with a low incidence of complications, such as epididymitis, meatitis and mechanical trauma.
Recommendation of the method.
Video urodynamic evaluation shortly after birth has an integral part in the optimal treatment of children in whom congenital neurological lesions affect the urinary tract. Follow-up urodynamic examination after the initial evaluation is as equally important, since detrusor-sphincter coordination may change with time. Video urodynamic studies were performed yearly in most patients. Children who had radiographic evidence of upper urinary tract deterioration, such as new or worsening hydronephrosis, reflux, or worsening of clinical symptoms, such as incontinence, underwent video urodynamic testing at more frequent intervals.
Figures
Figure 1. On AP lumbosacral spine plain film spina bifida and scoliosis at the lumbo-sacral
region was seen.
Figure 2. A photograph of a 10 years old girl with lipoma at the lumbo-sacral region.
Figure 3. A 6 years old girl, AP lumbosacral plain film showed sacral agenesis.
Figure 4. A 6 years old boy with a sacral agenesis and neurogenic bladder function.
Ultrasonography showed a small, round bladder with thickened bladder wall (a) and a dilated upper urinary tract (b).
Uroflow: intermittent curves (thin line) as a sign of micturition by abdominal pressure. Thick line showed voided volume (c), Voiding Cystourethrography: small bladder with wide bladder neck, many sacculus and small diverticula, and high grade vesico ureteral reflux on the left side (d), Cystometry showed high pressure, low capacity of the bladder with unstable detrusor contractions. Thick black line shows the pressure in the bladder. Thin black line shows the pressure in the rectum (e).
Figure 5. A 10 years old girl with an operated myelomeningocele.
Ultrasound showed a large, atonic bladder (a).
VCUG showed an elongated bladder, without vesico ureteric reflux (b) cystometry showed a good compliance of the bladder, and many uninhibited detrusor contractions (arrows) (c).
Figure 6. VCUG showed a "tong" like wide bladder neck as a sign of upper motor
neuron lesions.
Figure 7. VCUG showed a"lance" like wide bladder neck as a sign of lower motor
neuron lesions.
Figure 8. Ankle plain radiograph showed bones deformities of the foot.
References
1. Bauer SB: Neurogenic Bladder Dysfunction in Pediatric Kidney Disease ed: Edelmann CM Jr. Little, Brown and Company, Boston/Toronto/London, pp:2085-2109 1992.
2. Bauer SB. Neurogenic dysfunction of the lower urinary tract in children. In: Campbeel`s Urology, 7th ed. Edited by P.C. Walsh, A.B. Retik, E.D. Vaughan and A.J. Wein. Philadelphia. W.B.Saunders Co. Chapt. 65, 1998.
3. Fowler CJ: Neurological disorders of micturition and their treatment. Brain 122:1213-1231, 1999.
4. Galloway NTM, Mekras JA, Helms M, and Webster GD: An objective score to predict upper urinary tract deterioration in myelodysplasia J. Urol. 145:535-7, 1991.
5. Ghoniem GM, roach MB, Lewis VH, Harmon EP: The value of leak pressure and bladder compliance in the urodynamic evaluation of myelomeningocele patients. J Urol 144:1440-4, 1990.
6. Hobbins JC, Venus I, Tortora M, et al: Stage II ultrasound examination for the diagnosis of fetal abnormalities with an elevated amniotic fluid alpha-fetoprotein concentration. Am J Obstet Gynecol 142:1026-1029, 1982.
7. Kaefer M, Keating M, Adams M, Rink R: Posterior urethral valves, pressure pop-1offs and bladder function. J Urol 148:708-710, 1995.
8. Kaefer M, Paddy A, Kelly M, Darbey M, Bauer S: Improved bladder function after prophylactic teratment of the high risk neurogenic bladder in newborns with myelomeningocele. J Urol 162:1068-1071, 1999.
9. Lewis MA, Shaw J, Sattar TM, Bannister CM. The spectrum of spinal cord dysraphism and bladder neuropathy in children. Eur J Pediatr Surg 7(suppl 1), 35-37, 1997.
10. Madersbacher H: Video urodynamics. World J Urol 6:14-17, 1988.
11. Malkovicz S, Wein AJ, Elbadawi A, Van Arsdalen K, Ruggieri M, Levin R: Acute biochemical and functional alterations in the partially obstructed rabbit urinary baldder. J Urol 136:1324-8, 1986.
12. Mandell J, Lebowitz RL, Hallet M, Khoshbin S, Bauer SB: Urethral narrowing in region of external sphincter: radiologic-urodynamic correlations in boys with myelodysplasia. AJR 134:731-4, 1980.
13. Norgaard JP, van Gool JD, Hjalmas K, Djurhuus JC, Hellstrom AL: Standardization and definitions in lower urinary tract dysfunction in children. Brit J Urol 81 Suppl 3:1-6, 1998.
14. Peters CA, Vasavada S, Dator D, Carr M, Shapiro E, Lepor H, McConnell J, Retik AB, Mandell J: The effect of obstruction on the developing bladder. J Urol 148:491-5, 1992.
15. Shapiro E, Becich MJ, Perlman E, Lepor H: Bladder wall abnormalities in myelodysplastic bladders: a computer assisted morphometric analysis. J Urol 145:1024-7, 1991.
16. Shenot PJ, Rivas DA, Watanabe T, Chancellor MB. Early predictors of bladder recovery and urodynamics after spinal cord injury. Neurol Urodynam 1998, 17:25-29.)
17. Smith ED. Urinary prognosis in spina bifida. J Urol 108: 815-7, 1972.
18. Stein SC, Feldman JG, Friedlander M et al: Is myelomeningocele a disappearing disease? Pediatrics 69, 511-513, 1982.
19. Szabó L. and Borbás E.: Advantages and limitations on unichannel urodynamics in childhood. Orv. Hetil. 133:3253-3256, 1992. (in Hungarian)
20. Szabó L: Urodynamics versus radiologic imaging. Year Book Ped Rad 7:55-57, 1995.
21. Watanabe T. Vaccaro AR, Kummon H, Weich WC, Rivas DA, Chancellor MB. High incidence of occult neurogenic bladder dysfunction in neurologically intact patients with thoracolumbar spinal injuries J. Urol, 159: 965-968 1998.
László Szabó MD PhD,
Borsod County Teaching Hospital, Child Health Center,
3501 Miskolc PO Box 188. Hungary
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Ultrasonography inflammatory process of lower urinary tract
Szabó L1, Tóth V1, Deák M2, Bajusz I1, Losonczi K1.
Pediatric Nephrology1, Pediatric Radiology2, Child Health Center, Borsod County Teaching Hospital, Miskolc
Abstract
44 children (36 girls and 8 boys, age range 3 months - 16 years) with lower urinary tract infection's symptoms (urgency, frequency, lower abdominal pain and infected urine) were examined for 3 years. All patients had urine microscopy and culture, blood examination and ultrasound. In 12 children uroflow was performed. Hemorrhagic cystitis was discovered in 12 patients out of whom 7 had bladder wall disturbances. (A total of 19/44 patients had bladder abnormality by ultrasound, such as thickened bladder wall, trabeculation and increased echogenicity of bladder mucosa(. Uroflow curves were small and spiky in all 12 patients, a sign of bladder instability.
Key words: ultrasound, lower urinary tract, infection, bladder
Lower urinary tract symptoms, that is urgency, frequency, nocturia, bedwetting in a previously dry child, discomfort and pain during micturition are common among children referred to the pediatrician for evaluation. Many of these patients have infection, malignancy or outflow obstruction, and those with neurological disorders can also present with these symptoms [1,6,7]. The use of ultrasound for a non-invasive assessment of bladder in different urinary symptoms is a very useful and complementary examination [4,8,11].
Patients and Methods
All 44 children (36 girls and 8 boys, age range 3 months - 16 years) with urgency, frequency, lower abdominal pain and urine infection were examined between 1997-99. All patients had urine microscopy and culture, blood examination and ultrasound. Scans were obtained with a real time ultrasound scanner using a 5 MHz convex probe and the images were recorded on thermal paper. Uroflow examination was performed in 12 children. All children were treated with antibiotics or chemotherapy for two weeks.
Results
Distribution of different complains, symptoms and results of urine are seen in the table. Hemorrhagic cystitis was found in 12 patients and 7 of them had bladder wall disturbances. A total of 19 out of 44 patients had bladder abnormality by ultrasound. Figure 1a and 1b document recurrent hemorrhagic cystitis in a girl at the age of 4 and 6 years respectively. The bladder wall was thickened (4.9mm) and within it increased echogenic structures were seen. The bladder was small and almost round, the bladder wall was irregular (Fig. 1 a-b). Smooth, circularly thickened bladder wall without irregularity was seen in a 10 years old girl with a Pseudomonas cystitis (Fig.2). A trabeculation (arrow) was seen in a 14 years old girl with cystitis without bacterial specimen (Fig. 3). A small round bladder with thickened bladder wall was seen in an 8 years old boy without haematuria (Fig. 4). A small wellcircumscribed wall thickening (arrow) was seen in a 7 years old girl with dysuria (Fig. 5). Uroflow curves were small and spiky in all 12 patients examined, a sign of bladder instability.
Discussion
Inflammatory disorders of the bladder are a common problem in children. The prevalence of urinary tract infections varies markedly with sex and age. Symptomatic urinary tract infections occur in about 1.4/1000 newborn infants, with a slight male preponderance. Older group infections are much more common in females [1]. In our study 81% of patients was girl. Urine provides a good medium in which aerobic Gram negative rods such as Escherichia coli and Proteus species can grow rapidly. Urinary tract infections are caused mainly by colonic bacteria. In females, 75-90% of all infections are caused by E. coli, followed by Klebsiella and Proteus [1,5]. In our series only 30% of children had positive urine culture. Viral infections may also occur, but proving the presence of virus is difficult and needs longer time to identify than the duration of cystitis. There are many situations where it is essential to assess urinary bladder by ultrasound. Bladder wall changes are demonstrated far better with ultrasound than with conventional radiographic techniques. The mucosa, wich is hyperechogenic, can often be distinguished from the more echolucent detrusor muscle [3,4,7,8,11]. Acute bacterial cystitis is characterized by mucosal congestion and edema, occasionally with petechiae and hemorrhage. Ultrasound showed this abnormality in 58% of our patients with hemorrhagic cystitis, and 37.5% of our patients with other cystitis. The ultrasound examination also can help in other lower urinary tract infection such as xantogranulomatous [9], eosinophilic cystitis [2] or special hemorrhagic cystitis [6, 10].
In a normal situation, the bladder wall is visualized as a thin, and homogenic contour by ultrasound. The uroflow curves were spiky in children with unstable bladder function [7]. The voided volumes were small, because of functionally decreased bladder capacity as a result of lower urinary tract infection.
The advantages of ultrasound for assessing bladder wall disturbances in different lower urinary tract symptoms are very informative, simple, non-invasive and acceptable for children.
References
1. Gonzalez R: Urinary tract infections. In: Pediatrics ed: Nelson WE. W.B. Saunders Company, Philadelphia pp: 1360-1363, 1998.
2. Goodman TR, Connolly B, Taylor G: Eosinophilic cystitis following an infected urachal remnant. Pediatr Radiol 29:487-489, 1999.
3. Jequier S, Rousseau O.: Sonographic measurement of the normal bladder wall in children. American J Radiology, 149:563-566, 1987.
4. Neuenschwander S, Montagne JP: Urinary tract abnormalities. In: Pediatric Ultrasound. ed: Kalifa G: Springer- Verlag Berlin Heidelberg New York Tokyo, pp:142-172, 1990.
5. Saint S, Scholes D, Fihn SD, Farrell RG, Stamm WE: The effectiveness of a clinical practice guideline for the management of presumed uncomplicated urinary tract infection in women. Am J Med 106:636-641, 1999.
6. Sangra MS, Ramadwar RH, Mackinlay GA: A case of coliform haemorrhagic cystitis presenting as a tumour of the bladder BJU Intern 83:871-872, 1999.
7. Szabó L, Borbás É: Unstable bladder and recurrent urinary tract infection. Year Book of Ped Rad, 5:59-64, 1993.
8. Szabó L, Borbás É: Bladder wall abnormalities in different voiding problems in children. Year Book of Ped Rad. 9:41-46, 1997.
9. Tai HL, Chen CC, Yeh KT: Xantogranulomatous cystitis associated with anaerobic bacterial infection. J Urol. 162:795-796, 1999.
10. Vögeli TA, Peinemann F, Burdach S, Ackermann R: Urological treatment and clinical course of BK Polyomavirus-associated hemorrhagic cystitis in children after bone marrow transplantation. Eur Urol 36:252-257, 1999.
11. Willi UV: Bladder wall changes. In: Atlas of Pediatric Ultrasound. ed: Schultz RD and Willi UV, Thieme, pp:140-147, 1990.
László Szabó MD PhD
Child Health Center, Borsod County Teaching Hospital,
Miskolc H-3501 PO Box. 188. Hungary
Table
Distribution of complains and symptoms in 44 children with lower urinary tract infection.
Complains and symptoms
Number of patients
Pain in lower abdomen
17
Dysuria
29
Urgency-frequency
39
Nocturnal enuresis
2
Leukocyte in urine
44
Haematuria
12
Bacterium in urine culture
13
Figures
Figure 1. A 4 years old girl with hemorrhagic cystitis. Ultrasonography showed small, and
almost round shape bladder with thickened echogenic wall (a), 2 years later she had a recurrent hemorrhagic cystitis. US showed an irregular bladder wall (b).
Figure 2. A 10 years old girl with Pseudomonas cystitis. Ultrasound showed smooth,
circularly thickened bladder wall without irregularity.
Figure 3. A 14 years old girl with cystitis without bacterial specimen. A local trabeculation
(arrow) was seen on US scan.
Figure 4. An 8 years old boy without haematuria. A small round bladder with thickened
bladder wall was seen on US scan.
Figure 5. A 7 years old girl with dominant dysuria. A small wellcircumscribed wall
thickening (arrow) was seen on US scan.
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The role of ultrasound in the management of ovarian masses
in childhood - 10 years experiences
Kis,É., Verebély,T., Várkonyi, I., Kövi,R., and Máttyus,I.
First Department of Paediatrics of Semmelweis University, Budapest, Hungary
Abstract
Between 1988 and 1998 we studied 140 girls who were diagnosed with ovarian cysts and tumors by ultrasound. All patients were under 18 years old, and 48 of them were newborns.
136 patients had benign disease, and 4 had malignant tumors. The ultrasound examination showed that seventy-one masses were cystic, out of which 37 cured spontaneously.
Ultrasound showed complex masses in 69 cases (teratomas, torsion with cystic formation, malignant tumors). Conservative treatment was done only in 9 cases.
In four children with malignant tumors ultrasound showed a large mass, mainly solid with cystic changes, well encapsulated, and a very huge ascites.
Sonography was correct in determining the origin of masses in all cases, in differentiating other, non-ovarial pelvic diseases. Cystic ovarian masses were specific , representing benign ovarian cysts, in one part of these cases conservative management was indicated. Complex masses were proved to be ovarian teratomas, hemorrhagic cyst with torsion, and malignant tumors. In some cases of complex masses conservative management were possible.
Keywords: ovarian, mass, children, ultrasound.
Lower abdominal pain, a common paediatric problem is often due to ovarian masses in girls. Prior to the era of the ultrasound their true incidence was unknown, however with the widespread use of sonography the detection rate of small (less then 5 cm) cysts has increased. In children without significant symptoms these cysts are usually managed conservatively and followed up by a series of ultrasonographic examinations. (4,8,12)
We present the results of a retrospective study of 140 girls who were diagnosed by ultrasound having ovarian cysts and tumours over a 10 year period.
Patients and methods
Between the years 1988-1998 all girls in whom an ovarian mass has been detected by ultrasound were included. All patients were less than 18 years old, 48 of the 140 patients were newborns or infants.
All children were examined by using a real time scanner (Hitachi EUB 515,555) with 3,5-5 MHz transducer, through a full urinary bladder.The longitudinal, transverse and anteroposterior dimension of the cysts were recorded. Cyst was considered large, if greater than 5 cm in any diameter, simple, if the wall was smooth and the internal fluid was anechoic, and complex, with internal echos, or fluid debris level, septated, or with solid and cystic parts. Urgent surgery was done in cases of a large mass, a solid, or mostly solid mass, or in cases with serious clinical symptoms. In cases of small
cysts, or large anechoic, or nearly anechoic cysts in asymptomatic girls, or patients with mild clinical symptoms conservative therapy was done. Follow up ultrasound examinations were done untill the cyst resolved. In 4 patients additional abdominal CT was done.
Results
Indication of pelvic ultrasound is shown in Table 1.The age distribution of 140 girls is presented in Figure 1. Ultrasound characteristics, the size and location of the masses are presented in Table 2. The ultrasound appearance and the histological diagnosis according to the therapy are presented in Table 3.
94 girls (67%) had surgery : 70 of them (74%) had a large cysts above 5 cm diameter, most of them with mixed echostructure. In 18 cases (19%) the mass was smaller than 5 cm, but mostly solid echostructure. In 6 patients with simple anechoic cysts, increase in pain made surgery necessary. In case of haemorrhagic cyst (9/94) and ovarian torsion (11/94) internal echoes or fluid-debris level were detected .
In 13 operated cases (14%) the histological diagnosis was teratoma. All masses were shown to have complex echostructure, with partly solid, partly hypoechoic or anechoid areas .
4 children (3%) had malignant tumour (Yolk sac tumour, dysgerminoma, granulosa cell tumour and androblastoma). All patients had a large, palpable mass, mainly solid with cystic changes, well encapsulated and a huge ascites. In three of them abdominal CT was done as well.
Forty six girls (33%) did not have surgery, but were treated conservatively, and followed with a series of ultrasound scans. 37 cysts were anechoic, and 16 of them were above 5 cm in diameter. 9 cysts showed mixed echostructure, 7 of them larger then 5 cm, with mild clinical symptoms. (Figure 2.)
One girl had bilateral, large ovaries, mostly solid structure with small cysts and pelvic ascites. Abdominal CT clarified the diagnosis of polycystic ovary disease.
Some cysts resolved within a few days, most of them took 2-8 weeks. Two girls were found to have recurrent cysts, over a period of a few months but not always on the same side.
Among the 140 patients 48 newborn were examined, of whom 40 (83%) had prenatally detected ovarian mass. Among them 30 surgeries were done, 19 girls had a large cyst with a diameter between 5-10 cm. Eleven babies had a small cyst, between 2-4 cm, but with complex echostructure, with thick wall, fluid-debris level, or internal septums. (Figure 3.)
18 babies with simple cysts were followed up with serial ultrasound examinations, and all cysts disappeared . The ultrasound finding and clinical details are presented in Table 4.
Discussion
Ovarian masses are the most frequent pelvic tumours in paediatric age. (7). Ultrasound is an accurate, rapid method to identify normal and pathological pelvic structure in children. Most frequently the indication of the examination is an acute or chronic right lower quadrant or paraumbilical pain. In one part of the cases ovarian cysts are incidental findings in asymptomatic girls . By ultrasound examination ovarian masses could be differentiated from other diseases with similar clinical symptoms (appendicitis, adnexitis, pregnancy, renal or gastrointestinal disease).
Sonographic features of ovarian masses are identical like those in adults, cystic, complex and solid. Cystic masses are generally benign simple cysts, with thin wall without internal echoes. Whether the mass has both cystic and solid components or whether it is septated , a hemorrhagic cyst, torsion or neoplasia should be suspected (Figure 4). In cases of solid masses malignancy could be considered, however, torsion or hemorrhage should be solid as well. (4,8,12) Ascites in malignant ovarian tumours is rarer in children than in adults. (7) In our 4 cases with malignant tumour huge ascites was detected. There were no solid masses among our patients, the malignant cases showed mixed echostructure as well.
Management depends on the seriousness of clinical symptoms, the size and echostructure of ovarian masses and on the age of the child. The simple cysts generally can resolve spontaneously, but in cases of very large cysts surgery is indicated. Complex masses may or may not be benign.The ultrasonographic appearance can not reliably distinguish between them. Therapy must therefore be directed in light of the clinical picture. In cases of mild clinical features conservative clinical observation with repeated sonography is possible. Hemorrhagic ovarian cyst normally will undergo change in its sonographic characteristic. However, if the mass does not change, neoplasia, either benign or malignant should be suspected, and surgery is recommended. (4,8,12)
Previously, neonatal ovarian cysts was considered rare. Nowdays most of this cysts are detected prenatally.
According to the literature most cysts in utero and early infancy are follicular and develop as a result of maternal and fetal hormone stimulation of Graffian follicle development (1). However not all cysts are benign, and the possibility of ovarian torsion is frequent, therefore surgery is recommended in this age group for any complex cysts regardless of size (1,3). Treatment options depend on the risk of complication. The most frequent complication is torsion, which was reported in 69 of 257 cases of antenatally diagnosed ovarian cysts. (1) It is more common in large cysts, therefore early surgery is indicated in cysts above 5 cm and in simple cysts as well. Other rare complications are gastrointestinal obstruction, urinary tract obstruction, vesicoureteral reflux. (5,9) Rupture of the cyst is extremely rare(11). Simple ovarian cysts smaller then 5 cm can be safely observed, and followed up their resolution by ultrasonography.(2,6,10)
In conclusion, one part of ovarian cysts in children may be safely followed with serial pelvic ultrasonography, and spontaneous resolution should be expected. Cyst size and character alone is not reliable in predicting outcome. Sonography could help to differentiate ovarian masses from other abdominal diseases, it can determine the correct size and structure of the ovarian mass, and the changes in them during the follow up period. In uncertain cases CT examination is indicated.
Surgery is indicated when the cyst has a complex structure and does not change on serial ultrasound scans, or serious clinical symptoms are present.
Neonatal ovarian cysts can resolve spontaneously, but above 5 cm surgery is indicated in simple cysts as well.
References
1. Brandt,ML., Luks,FI., Filiatrault,D. et al.: Surgical indications in Antenatally Diagnosed Ovarian Cysts. J Pediatr Surg. 26. 276-282. 1991
2. Calisti,A.., Pintus,C., Celli,S. et al.: Fetal ovarian cysts: postnatal evolution and indications for surgical treatment. Pediatr Surg int. 4.341-346. 1989.
3. Croitoru,DP., Aaron,LE., Laberge,JM. et al.: Management of complex ovarian cysts presenting in the first year of life. J Pediatr Surg 26.1366- 1368 1991
4. Haller,JO., Bass,IS., Friedman,AP.: Pelvic masses in girls: an 8-year retrospective analysis stressing ultrasound as the prime imaging modality. Pediatr Radiol. 14. 363-368.1984.
5. Herman,TE., Shackelford,BD.: Larged torsed ovarian cysts associated with massive vesicoureteral and intrarenal cyst. J Perinatol. 17. 75-78, 1997.
6. Nussbaum,,AR., Sanders,RC., Hartman,DS. et al.: Neonatal ovarian cysts: Sonographic-Pathologic Correlation. Radiology. 168. 817-821. 1988.
7. Sty,JR., Wells,RG.,: Other Abdominal and PelvicMasses in Children . Seminars in Roentgeeology. Vol XXIII.216-231. 1988.
8. Thind,CR., Carty,HML., Pilling,DW.: The Role of Ultrasound in the Management of Ovarian Masses in Children. Clinical Radiology. 40. 180-182. 1989.
9. Towne,BH., Mahour,GH., Wooley,MM. et al.: Ovarian cysts and tumors in infancy and childhood. J Pediatr Surg. 10.311-320. 1975.
10. von Schweinitz,D., Habenicht,R., Hoyer,PF.: Spontane Regression von neonatalen Ovarialzysten. Monatschr Kinderheilkd. 141.48-52. 1993.
11. Vyas,ID., Variend,S., Dickson,JA.: Ruptured ovarian cyst as a cause of ascites in a newborn infant. Z. Kinderchir. 39.143-144. 1984
12. Warner,BW., Kuhn,JC., Barr,LL.: Conservative management of large ovarian cysts in children: The value of serial pelvic ultrasonography. Surgery. 112. 749-755. 1992.
Éva Kis M.D.Ph.D
First Dept. of Pediatrics,
Semmelweis University
Budapest,1083,Bókay J.53,Hungary
No. of patients
prenatal diagnosis 40
abdominal pain
right lower quadrant 31
recurrent abdominal 43
palpable mass 8
precocious puberty 1
suspected renal disease 5
incidental finding 8
infection 3
Total 140
Table 1. - Pelvic ultrasound indication and number of patient
Number Percentage
of cases
Side
Right 104 74%
Left 36 26%
Type
Simple cyst 71 51%
Complex 69 49%
Diameter of cyst
2-5 cm 47 34%
over 5 cm 93 66%
Table 2. - Sonographic features of the masses
Ultrasound Histological No of Conservative Surgery
diagnosis patients
Cystic simple cyst 71 37 34
Mixed
nonneoplastic
simple cyst 32 9 23
hemorrhagic cyst 9 9
ovarian torsion 11 11
neoplastic
Teratoma 13 13
Yolk sac tumor 1 1
Dysgerminoma 1 1
Granulosa cell 1 1
Androblastoma 1 1
Total 140 46 94
Table 3. - Ultrasound and histological findings of patients
No. of patients 48
Prenatal diagnosis 40 (83%)
Therapy:
Surgery 30
large cyst (5-10 cm) 19
small complex cyst 11
Conservative 18 - all cysts disappeared
Table 4. - Results of surgical and conservative treatment
Figures
Figure 1.
The age distribution of patients with ovarian mass.
Figure 2.
The distribution of conservative and surgical therapy.
Figure 3. a-b
Lower abdominal ultrasonography showed anechoid ovarian cyst in a 12 years old girl (a), 15 days later the cyst resolved (b)
Figure 4.
Lower abdominal ultrasonography showed a large ovarian cyst with thick wall, fluid-debris level in a newborn baby.
Surgery: torquated ovarian cyst was found.
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Spontaneously resolved posttraumatic intracerebral cyst
Case report
Urbanek K., Gyarmati J., HUNIKO Diagnostic Imaging Center, Kistarcsa
Abstract
We present a case of a 4 years old boy, who developed seizures few days after suffering injury to the head. CT & MRI scans were performed which revealed a celebral cyst in the left parietal lobe. The cyst resolved spotaneously 3 years later.
Key words: Posttraumatic cyst, spontaneous resolution.
Cystic cerebral disease can be caused by inflammatory disease, tumor and trauma.
Direct mechanical injury and secondary pathophysiological changes affect the outcome of head trauma.
Direct mechanical injury is immediately produced by physical force (blood vessels, nerve). Cortical contusion is a type of brain contusion involving the cortex and relative sparing of the deep white matter. Cortical contusion can be 2 - 4 cm in size, associated with haemorrhagic component. Secondary pathophysiological changes can occur including edema and a raised intracranial pressure.
Secondary effect could be leukomalacia and cystic malformation (1,2). Spontaneous resolution of a secondary lesion is very rare.
Case report
A 4 years old boy was admitted because of a mild head trauma. Two days later he developed seizures. CT and MRI examination was performed. In both scans, a 4x4 cms cystic lesion was seen in the left parietal lobe (Fig 1.). In the differential diagnosis hydatoid cyst was suspected but it was not supported by serologic examination.
The child received antiepileptic therapy. Biopsy was not performed, since the parents refused to consent.
MRI examination was performed yearly, but no change in morphology was detected. After three years, the cyst resolved spontaneously and was replaced by fibrotic tissue and cortical enchancement (Fig2.). The antiepileptic therapy was followed. Every year MRI scans were performed and after 4 years there is no change, the cyst did not refill.
Discussion
Differential diagnosis of intracerebral cystic lesions is often difficult. Inflammatory disease, cerebritis may develop into a cerebral abscess. The cerebral area is surrounded by fibroblast and central liquefaction develops. Typically, edema surrounds the abscess and the fibroblast build a three layer abscess wall.
Heamatogeneous origin abscesses are usually solitary, and are most frequent in the frontal and temporal lobe.
Streptococci, pneumococci and staphylococci also often cause abscesses. In the immunsuppressed patient toxoplasma, cryptococcus, candida, nocardia, tuberculosis, aspergillosis are often responsible for the causes of brain abscess. Toxocariasis or echonococcosis can also cause cystic malformation in the brain (3,4).
It is essential to rule out cystic tumor.
Astrocytoma is the most common brain tumor in childhood. Characteristics of supratentorial low grade astrocytoma include hemispheric white matter location, slight hypodensity, lack of sharp margination, little or no contrast enchancement, undetectable or minor oedema.
In our case the image was similar but spontaneous resolution in low grade astrocytoma is not typical.
Glioma, ependymoma, primitive neuroectodermal tumor, haemangioblastoma, medulloblastoma and metastatic brain tumors can also be cystic (1,5).
We would like to pay attention of the fact that in some case, rarely, the posttraumatic cystic lesion can resolve spontaneously.
References
1. Grossmann CB. : Magnetic resonance imaging and CT of head and spine. 1990 Williams Wilkins
2. Hesselink JK. : MR imaging of brain contusion. A comporative study with CT.
AJNR 9 : 269 - 278, 1988
3. Mc. Corhell SJ., Lewall DB. : Computed Tomography of intracerebral echinococcal cyst in children.
J. Comput Tomographie 9 :514, 1985
4. Zee C., Segal HD., Apuzzo MLJ et al : Intraventricular cysticercal cyst. AJNR 5 : 474 - 475, 1984
5. Kjos Bo., Brant Zawadsk M., Kucharczyk W. : Cystic intracranial lesions, MRI.
Radiology 155 : 363 - 369, 1985
Urbanek Krisztina, MD
Department of Diagnostic Imaging
Huniko KDK, Semmelweis tér 1.
2143 Kistarcsa, Hungary
Figure 1. On CT scan, after 6 days of trauma. Well defined hypodense cystic lesion was seen (a) MRI coronal (b) and sagittal T1 weighted imaging (c) showed the hyopointense inhomogenous lesion in the right parietal lobe closly at the surface.
Figure 2. Three years later the cyst is nearly resolved, replaced with fibrotic tissue which was seen on T1 (a) and T2 weighted axial MRI scan (b).
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Nonfunctioning benign pancreatic tumor in a child.
Kiss Á.L1., Borbás É2., Tamási K3., Lombay B2.
1Department of Pediatric Surgery, 2Pediatric Radiology and 33rd Department of Pediatrics, Borsod - A.Z. County Teaching Hospital, Miskolc
Abstract
A case of a 12 year-old boy with pancreatic tumor is presented. He was investigated because of abdominal pain and a rutine ultrasonography revealed the pancreatic lesion. Ultrasonography and CT scans showed a well defined round tumor. At surgery the mass was removed and a benign endocrine tumor was proved by histology.
Pancreatic islet cell anomalies usually present with marked endocrine findings. The indentity of the involved cell type determines the clinical symtoms. Investigation of endocrine disorders can reveal the rare cases of hormon releasing pancreatic tumors. The real rarety is, however, when a tumor is diagnozed first and then it is followed by a workup for the possible endocrine problems.
Nonfunctional neoplasms of the pancreas are extremely rare and usually malignant (1,10).
Case report
A 12 year-old boy presented with an episode of colicky abdominal pain. Ultrasound and CT demonstrated a 1,5 x 2cm in diameter solid mass in the body of the pancreas.(Fig 1.)
Though after a few days the child became symptom-free a thorough workup was commenced on which did not reveal any exocrine or endocrine disorder of the pancreas. Corresponding to the lack of clinical symptoms the hormone levels were within the normal range and also there was no sign for disturbed metabolism of carbohydrate. Paraneoplastic symptoms were not found, either. Surgery was decided.
At laparatomy a dark, firm, solid mass of 2 x 2 cm in diameter was found at the border of the body and the head of the pancreas.
Though the tumor appeared encapsulated it was quite adherent to the pancreatic parenchyma, and it was only possible to remove it with cautious sharp dissection. The histological diagnosis was benign endocrine tumor of the pancreas.
Postoperatively the child made a straightforward recovery. He is still kept under review but 6 years after the surgery he is keeping well, and there is no sign for recurrence on ultrasound.
Discussion
Dearrangement of Langerhans islet cells usually entails severe endocrine disorders. Four types of islet cells are decribed and three of them have endocrine activity (11). The alpha-cells are the presumed site of synthesis of glucagon. The beta-cells produce insulin. Hyperfunction of the delta-cells (terms of gamma or nonbeta-cells are also used) is responsible for hypergastrinemia (Zollinger-Ellison syndrome).
A pancreatic lesion may be focal (adenoma) or diffuse. Adenoma may be solitary or multiple and occasionally its identification may be difficult.
Historically, pancreatic adenomas, owing to their high degree of vascularity, were demonstrated by celiac angiography (6). In children, however, the angiography has only limited success due to the normal rich vascularity of the pediatric pancreas (14). A lot but not all small lesions can be easily visualized by ultrasound (4,5). In the majority of cases the diagnosis and the localisation may be confirmed by CT scan particularly with contrast enhancement. It is still difficult to recognize and differentiate the tumors of small sizes, the multiple adenomas and the diffuse islet cell anomalies. Transhepatic portal catheterization with selective pancreatic venous sampling proved to be a valuable approach towards the anatomic site of endocrine lesions (3,14). If localisation of a hormonally active pancreatic tumor remains undetermined despite the use of different modalities surgical exploration should be carried out. Intraoperative ultrasound may be of great help to the surgeon in indentification of a nonpalpable lesion (8,13). Depending on the operative finding and the frozen section examination enucleation, distal pancreatectomy, subtotal resection, pancreatoduodenectomy or in presence of extensive hepatic metastases hepaticopancreaticogastroduodenectomy with liver transplant should be carried out (2,9,12,15).
Ten percent of insulin - releasing tumors, 70 percent of glucagonomas and majority of the nonfuctioning tumors are malignant (1,7).
The 12 year-old boy we report on had no endocrine symptoms, the tumor was not palpable. The pancreatic lesion was detected incidentally at an episode of abdominal pain.
Having been localised by ultrasound and CT the tumor was duely enucleated. Considering the high rate of malignancy among nonfunctioning pancreatic tumors the early detection and removal of the tumor is likely to prevent it from a malignant transformation.
Reference
1. Ashcraft K.W., Holder T.M : Paediatric Surgery. Second Edition. W.B. Saunders Co. Philadelphia, London, Toronto, Montreal, Sydney, Tokyo 1993. p532.
2. Aszodi A., et al : Giant nonfunctioning islet cell tumor requiring pancreaticoduodenectomy and complete liver revascularization. J. Suerg. Oncol 53 p273-276, 1993.
3. Brunelle F., et al : Pancreatic venous samplings in infants and children with primary hyperinsulinism. Pediatr Radiol 19 p100-103, 1989.
4. Bundscherer F., et al : Sonografische Daignostik abdomineller solider Raumforderungen in Kindesalter. Monatsschr Kinderheilkd 135 p30-35,1987.
5. Davies PF. Et al : Ultrasonography of the pancreas in patients with multiple endocrine neoplasia type I. J Ultrasonography Med 12 p67-72,1993.
6. Dodek AB., Sagedhi-Nejad A. : Value of selective pancreatic angiography in the evaluation of hyperinsulinemic hypoglycemia in infancy. Pediatrics 90 p636-638, 1992.
7. Friesen SR., Thomson N.W. : Surgical Endocrinology. J.B. Lippincott Co. Philadelphia, Grand Rapids, New York, St. Louis, San Francisco, London, Sydney, Tokyo. p213-248.
8. van Heerden JA., et al : Occult functioning insulinomas : which localizing studies are indicated? Surgery 112 p1010-1014, 1992.
9. Horisawa M., et al : Frantz's tumor (solid and cystic tumor of the pancreas) with liver metastasis : succesful treatment and long-term follow-up. J Pediatr Surg 30 p724-726, 1995.
10. Jaksic T., et al : A 20-year review of pediatric pancreatic tumors.
J Pediatr Surg 27 p1315-1317, 1992.
11. Kissane J.M. : Pathology of infancy and childhood. The C.V. Mosby Co.
St Louis 1975. p334-341
12. Lobe TE., et al : Hepaticopancreaticogastroduodenectomy with transplantation for metastatic islet cell carcinoma in childhood. J Pediatr Surg 27 p227-229, 1992.
13. Telander RL., et al : Intraoperative ultrasonography of the pancreas in children. J Pediatr Surg 21 p262-266, 1986.
14. Warden MJ., et al :The surgical management of hyperinsulinism in infancy due to nesidioblastosis. J Pediatr Surg 23 p462-465, 1988.
15. Zeller J., et al : Localisation d'un adenome langerhansien par catheterisme portal transhepatique. Arch Fr Pediatr 50 p675-680, 1993.
Dr. Ákos Kiss
Department pf Pediatric Surgery
Borsod A.Z. County Hospital,
Child Health Center, 3501 Miskolc
PoBox 188, Hungary
Legends
Fig. 1. Abdominal ultrasonography revealed a well circumscribed solide mass at the border of the head and the body of the pancreas (a), on CT the tumor was hypodense with a hyperdense rim (b).
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Epidemiology of cerebral palsy in newborns and infancy. Clinical and diagnostic characteristics and therapeutic results.
Fejes M.(, Szűts A.(, Cservenyák J.(, Lombay B.2
Department of Neonatal Pathology and Perinatal Intensive Care Centre( and Department of Pediatric Radiology2, Borsod A.Z. County Teaching Hospital, Miskolc
Abstract
The aim of the study was to determine the clinical, diagnostic and epidemiologic characteristics of cerebral palsy(CP).9218 newborns were delivered in the county of Borsod in 1995. Out of them 695 ( 7,39 % ) had risk for cerebral palsy (CP). About half of them had cerebral paretic signs below one year of age.CP incidence was 1,7 ‰ among term and 4,16 % among preterm newborns at the age of one year.The clinical sign was mainly tetraparesis (46,6%) in term and mainly diplegia (58,06%) in preterm infants. Type of CP was spastic in 66,6 % of term, and mixed (axial hypotony followed by limbs spasticity)in 64,51 % of preterm infants. Newborns with positive cerebral ultrasound showed larger movement disorders, probably they had macroscopic cerebral injury. The treated patients could walk at the age of about 13 months ( SD: ( 3,63 ).
Keywords: cerebral paresis,neurohabilitation, ultrasound.
In utero, during delivery, newborn and infant age foetus and the newborn are impaired by different injuries. The first visible sign of injuries to central nervous system is the occurence of CP symptoms. These symptoms could be originated from several causes.(i.e.CP risk factors could be praenatal, subnatal or postnatal.) The most frequent factors are shown in Table 1.
Several publications came out in recent years that a perinatal intensive care centre itself could be a source of stress (excess noise,light, painful examinational procedures), could play significant role in the development of various neurological symptoms to severely ill neonates at a later age.(1,4,5,6,9)
Patients / Methods
In 1995 in the hospitals of Borsod-Abaúj-Zemplén County 9304 newborns were delivered and 9218 of them survived.In our department,one of the biggest one in Hungary,75 term newborns were treated with acute fetal distress, 4 with postasphyxiated syndrome, out of these one baby died. Hypoxic ischaemic encephalopathia occurred in 19 patients, and we managed to keep alive 13 of them.There were 22 term neonates with hyperbilirubinaemia, who were in good condition at birth, but in their history obstetric complication was mentioned, e.g. umbilical cord around the neck, lengthy delivery or impaction etc. These infants have no neurologic signs in the first days,the moderately increased serum bilirubin level was treated,however, at the age of 4-6 weeks spasticity of muscles appeared in tetraparesis distribution.In table 2.the distribution of term and preterm babies and the rate of (risk newborns( are seen.(Table 2.)
For CP screening the following elementary movement patterns are used clinically.
1.verticalisation: pushing up until sitting, pulling until sitting, sitting in air. 2.locomotor patterns : stepping, assisted creeping, crawling down slope in prone state.
In our practice the mostly used primitive reflex examinations are: step-on, supporting reaction, symmetrical and asymmetrical tone neck reactions. If an abnormality is found as a result of the screening, then a complex neurologic examination is undertaken prior to the individual's neurohabilitation therapy.(6)
Cranial ultrasonography (US) was generally performed at the pediatric radiology department (Toshiba 140 equipment, 5 MHz sector and 7,5 MHz linear probes)during the first week of age. For seriously ill babies bedside US was done on the first or second day of life. Follow up US was tailored depending on the clinical status and/or US findings. Both clinical and US controll examinations at different intervals (2-3 months) were usually followed up to one year of age. Follow up studies were extended in some cases.
In our department the neurohabilitation therapy has several stages. After birth we aim to stabilise the state, and minimize stress from neonatal intensive environment. Careful tactile stimulation, which means not more than 5 stimulus per minute, was used after stabilisation. Active training adjusted to the individual from the age of 2-6 weeks being the earliest, in case of premature neonates it can only be started from 3-4 months old due to their physical status. Psychic treatment takes a few minutes at the beginning and later it is increased up to half an hour per day.(4,6)
Results
326 term and 369 preterm newborns had high risk for CP.Incidence of CP was higher in preterm and very low birth weight groups than in term neonates.(Table 3-4.) Types and distributions of CP were different in these groups.(Table 5-6.)There were no observed athetosis,ataxia,only a patient had transitional hand tremor.
Out of 695 CP risk newborn US morphological anomalies were shown in 141(54 term,87 preterm).We did not prove CP signs in 26,24% babies.It seemed to be that CP patients with US abnormality were slowly impaired in health,showing no statistical significance,their observeble movements developed later.(Table 7.)
Subependymal haemorrhage (Fig 1,2.) was found on one or both sides in 21 term and in 30 preterm neonates. Intraventricular hemorrhage (Fig. 3.) was seen in two preterm babies. Periventricular leucomalatia (PVL) (Fig. 4.) was shown in 10 term and 13 preterm patients. The signs were in accordance with anatomical localisation.2 preterm and 3 term babies had porencephalic cyst which enlarged temporaly then maintained a particular size between 6 and 8 months. Periventricular hyperechogenecity was found in 16 term and 35 preterm newborns usually on both side. In a premature baby periventricular leucomalatia occured. Cerebral edema was observed in 6 term and 3 preterm newborns.Their recovery was rapid with early habilitaton. 67 preterm and 11 term neonates had septum pellucidum cysts (Fig 5.). They were associated with cavum vergae in 10 preterm and one term neonates.There were no correllation with clinical signs. Their size decreased and all the cysts resolved during the follow up studies. With our early neurohabilitation therapy,the treated patients could walk at the age of about 13 months(SD:(3,63) making correcting it is to gestational age it is also means they could walk at the age of about 12 months (SD:(3,14).
Discussion
Cerebral palsy is a complex symptom,not a specific disease.It was defined by Club in 1959 as a persistent and not changing disorder of movement and posture appearing early in life and due to a nonprogressive lesion of the developing brain.(2)Now we can say,that CP signs and symptoms may change with age depending on severity and treatment.According to Taudorf and his colleages, motor symptoms could disappear,
but several patients might show neuropsychological difficulties or mild mental retardation.(17)
Which factors are responsible for this different processes and success of early neurohabilitation treatments? A possible explanation that the myelinisation and synaptisation not finished at birth.Impaired functions can partially be compensated in developing brain.Using functional magnetic resonance imaging(FMRI) signals of unaffected hand of a hemiplegic patients were primaly evident over the rolandic region of contralateral (intact) hemisphere.In contrast, the paretic hand resulted in activation of wider regions of the contralateral(injured) hemisphere as well as of ipsilateral intact hemisphere, again with a wider(perirolandic) distribution. (3,6)
Among our patients CP prevalence was 1,7 ‰ in term and 4,16 % in preterm newborns at one year of age,which correllated well with another publications.(1,9) More than a third of high risk newborn showed CP signs within the first year of life,but real CP was only seen in a sixth of high risk newborns and these patients were mostly treated for asymptomatic status.
Advances in medical technology during the last three decades are credited with dramatic reduction in mortality.More and more lower birth weight,lower maturity newborns survived so intra/periventricular haemorrhages increased with larger rate of hemiparesis among preterm and VLBW newborns.
Diagnostic imaging modalities : cerebral ultrasound (US), computer tomography(CT), magnetic resonance imaging(MR) examinations have prognostic importance for CP outcome.Third and fourth grade intra/periventricular haemorrhage were examinated with US and CT by Lin and colleaques. They found that cerebral haemorrhage tended to result asymmetrical syndromes.(8)The US diagnosis of germinal matrix haemorrhage is now accepted and provides very good correllation with pathological specimens.Non-caviting echogenicity seems to have a good prognosis and not to be associated with severe CP. Frontal or parietal periventricular leukomalacia(PVL) may be associated with severe forms of CP but lesions of occipital periventricular white matter and infants with both side lesions have a very high risk of severe motor impairment.Before the development of cysts, parenchymal echogenicity is often evident.Porencephaly is a later US finding, its prognosis depends on the localisation.US examination has a better prognostic value after two weeks of age.(7)PVL developed for 3-4 week of life at another follow up.(18)In our study cerebral US differences were associated with early severe CP symptoms, impaired movement developed and secondary signs were also shown in this group.
The anatomical distribution of hypoxic-ischaemic lesions in the neonatal brain depend on the gestational age at that time of the injury. Rademakers and his colleagues demonstrated these well known pathological facts on MRI studies that is active myelinisation as shown in cortical,subcortical region,in basal ganglias and hyppocampus among term babies.These were the mostly vulnerable places.(10)
Athetoid CP was proved after injuries of basal ganglias.After initial period of injury babies were in hypertonic state with opisthotonus, irritability ,tremor and then later become hypotonic.(12) There were 8 general hypotonic patients in our study, but none of them developed later athetoid signs.
Infants with hypoxic-ischaemic encephalopathy (HIE) were studied using serial MR imaging up to the age of 2 years.There was a strong positive correlation between MR findings and physical status.The patients with an abnormal outcome had extensive white matter abnormalities.Bilateral basal ganglia abnormalities were associated with severe developmental delay.(13)
Conclusion
Systematic,longterm neurodevelopmental follow up is recommended for high risk newborns. US screening should be performed for positive history of pregnancy and for infants with mild CP signs.Preventive neurohabilitation treatment for muscle tone abnormality and impaired movement developing should be initiated below the age of three months.Developing brain can mainly compensate this eliminated functions.(6,11)
Reference
1.Allan, M. A., Capute, A. J. : Neonatal neurodevelopmental examination as a predictor of neuromotor outcome in premature infants, Pediatrics, 83., 498- 508, 1989.
2.Club,. L. : Memorandum on terminology and classification of 'cerebral palsy '. Cereb. Palsy Bull. , 1, 27 -35, 1959.
3.Filloux, F. M.: Neuropathophysiology of movement disorders in cerebral palsy, J. Child, Neurol., 11, Suppl. 1. 3-10, 1996.
4.Glass, P.: The vulnerable neonate and the neonatal intensiv care environment. In: Avery, G.B., Fletcher, M.A., MacDonald, M.D.: Neonatology : Pathophysiology and management of the nexborn. Philadelphia, J. B. Lippincott co., 1992.
5.Grether, J. K., Nelson, K. B., Emery III, E. S.,, Cummins, S. K.: Prenatal and perinatal factors and cerebral palsy in very low bith weight infants, The J. of Pediatr., 128. , 407- 414, 1996.
6.Katona, F.: Klinikai fejlődésneurológia, Budapest, Medicina, 1999.(in Hung.)
pp. 229-277
7.Levene,M.I.:Cerebral ultrasound and neurological impairment:telling the future. Arch. of Dis.in Child.,65,469-471,1990.
8.Lin, J., Goh, W., Brown, K., Steers, A. J. W.: Heterogeneity of neurological syndromes in survivors of grade 3 and 4 periventricular haemorrhage, Child's Nerv Syst 9, 205 -214, 1993.
9. Msall, M. E., Buck, G. M., Rogers, B. T., Merke, D., Catanzaro, N. L., Zorn, W. A. : Risk factors for major neurodevelopmental impairments and need for special education resources in extremely premature infants, The J. of Pediatr, 119. 606- 614, 1991.
10.Rademakers, R. P., Knapp, M. S. van der, Verbeten, B.Jr., Barth, P. G., Valk, J. : Central cortico- subcortical involvement : a distinct pattern of brain damage caused by perinatal and postnatal asphyxia in term infants, J. of Comp. Assist. Tomogr., 19, 256- 263, 1995.
11.Rosenbloom, L.: Diagnosis and management of cerebral palsy. Arch. Dis. Child. 72, 350 - 354, 1985.
12.Rutherford, M. A., Pennock, J. M., Murdock - Eaton, D. M., Cowan, F. M., Dubowitz, L. M. S.: Athetoid cerebral palsy with cysts in the putamen after hypoxic ischaemic encephalopathy, Arch. Dis. In Child., 67, 846 - 850, 1992.
13.Rutherford, M., Pennock, J., Schwieso, J., Cowan, F., Dubowitz, L.: Hypoxic -ischaemic encephalopathy: early and late magnetic resonance imaging findings in relation to outcome, Arch. Dis. In Child., 75, F145 -F151, 1996.
17.Taudorf, k., Hansen, F. J., Melchior, J. C.: Spontaneous remission of cerebral palsy, Neuropediatrics, 19 -22, 1986.
18.Váradi, V., György, I., Karmazsin, L.: Neonatal periventricular leukomalacia: diagnosis and monitoring by real- time ultrasound., Akadémai Kiadó, Budapest, 43- 51, 1988.
Melinda Fejes M.D.
Department of Neonatal Pathology
Borsod A.Z. County Teaching Hospital 3501. Miskolc, POBox 188, Hungary
Table 1. The most frequent risk factors of cerebral paresis.
praenatal risk factors
* intrauterin asphyxia
* praenatal infection
* brain malformations
* praenatal syndromes
* familiar
subnatal risk factors
* narcotics
* umbilical cord problems
* caesarean section
* birth injury
postnatal risk factors
* low gestational age
* low birth weight
* intracerebral haemorrhage
* cerebral oedema
* hypoxic ischaemic encephalopathy
* 1 minute Apgar score 0- 3
* central nervous system malformations
* postnatal infection of central nervous system
* cardiac causes
* vaginal birth of prematures
* mechanic ventillation
Table 2. Distribution (%) of term and preterm babies
Term
Preterm
Total
survived newborns
8474 (91,9 %)
744 (8,07 %)
9218 (100 %)
risk newborns
326 (3,84 %)
369 (49,59 %)
695 (7,53 %)
Table 3.Cerebral paresis prevalence (Number of patients and %)
Term
(No 8474)
Preterm
(No 744)
Total
(No 9218)
below 1 year
120 (1,41)
141 (18,95 %)
261 ((2,83 %)
1 year
15 (0,17)
31 (4,16 %)
46 ( 0,49 %)
15 months
3 (00,3)
2 (0,26 %)
5 (0,05 %)
Total
138 (1,61)
174 (23,37)
312 (3,37)
Table 4.
Very low birth weigth newborns cerebral paresis prevalence ( Number of patients and %)
<15oo gram
<1250 gram
<1000 gram
survived premature
60 (100 %)
22 (100 %)
6 (100 %)
Cerebral paresis prevalence
below 1 year
25 (41,6 %)
11 (50 %)
3 (50 %)
1 year
5 (8,3 %)
2 (9,09 %)
0
15 months
1 (1,66 %)
0
0
Table 5.
Cerebral paresis distribution ( Number and %)
term newborns preterm newborns
below l year
1 year
Below 1 year
1 year
Hemiparesis
16 (13,33)
4 (26,66)
25 (17,73)
5 (16,12)
Tetra-paresis
44 (36,66)
7 (46,66)
43 (30,49)
8 (25,80)
Diplegia
27 (22,50)
4 (26,66)
51 (36,17)
18 (58,10)
Not classified
25 (20,83)
0 (0)
23 (16,71)
0 (0)
Table 6. Types of cerebral paresis (Number and % )
term newborns preterm newborns
below 1 year
1 year
below 1 year
1 year
Spastic
45 (37,50)
10 (66,60)
63 (44,60)
11 (35,40)
mixed
64 (53,30)
5 (33,40)
64 (45,30)
20 (64,51)
hypotony
11 (9,16)
0
10 (7,09)
0
not classified
0
0
4 (2,83)
0
Table 7. Finding of US in term and preterm babies with CPS
Term prematures babies
Subependymal
hemorrhage (SH)
21
30
SH+cysts
3
4
Intraventricular
hemorrhage
-
2
Periventricular
Echogenicity
16
35
Periventricular
leukomalatia
10
13
Porencephalic
Cyst
3
2
Cerebral edema
6
3
Non hemorrhagic
hydrocephalus
1
-
Total
60
89
Legends
1. Intracranial US : subependymal, periventricular, hyperechogenecity, mild hydrocephalus.
2. Intracranial US : subependymal hyperechogenecity with small cystic lesions on both sides and moderate hydrocephalus.
3. Intracranial US : huge intraventricular hemorrhage with severe hydrocephalus.
4. Intracranial US : left sided periventricular leukomalatia, right sided porencephalic cyst.
5. Intracranial US : subependymal hyperechogenecity and hyperechogenic septum pellucidum
cyst.
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Cleidocranial dysplasia
Deak M., Sagodi L., Lombay B.
County Teaching Hospital, Department of Pediatric Radiology and 3rd Department of Pediatrics Child Health Center, Miskolc
Abstract
A case of a 9 years old boy with cleidocranial dysplasia is presented. This skeletal disorder is characterized by generalized delay or even failure of ossification of portions of the skeleton results in clinical and radiological features that together establish the diagnosis.
From the radiologist's view, the most important findings are a triad of cranial, clavicular and pelvic abnormalities.
In our case mild shortness of stature, large head with prominent forehead, characteristic face, maldevelopment of clavicles, narrow pelvis, delayed of the pelvic bones and shortening of the distal phalanges was found.
Key words : cleidocranial dysplasia, clavicular defect.
Marie and Sainton first described the clinical and genetic features of this disease in 1898.[1]
This is an abnormality of mesenchymal tissue development that is manifest at birth and almost always has an autosomal dominant type with variable expressivity. Mutations occur in approximately one - third of the cases. [2]
Proportionate short stature is characteristic, but stature can also be normal.
Case report
9 years old boy with craniofacial dysmorphosis, somatic retardation and mild proportionate short stature was admitted to our unit. He had normal mental development. Familiar history was unknown.
He had large head with frontal and parietal bossing, a groove between the right and left halves, small face with a wide flat nasal bridge, which gives the impression of ocular hypertelorism, high - arched palate, failure of eruption of the teeth, narrow chest, dropping shoulders with easy approximation of the shoulders, narrow pelvis, joint hypermobility and abnormal gait. (Fig.1.)
Cranial plain film showed a brachycephalic skull with increased biparietal diameter, prominent frontal and parietal bones, platybasia, multiple wormian bones, dental anomalies. (Fig. 2,a.b.)
The lateral end of clavicles are hypoplatics, the scapulae are small and those are in a higher position than normal, the thorax is narrow, the ribs are short and oblique downwards(Fig 3.), dorso - lumbar scoliosis, delay in the closure of the posterior arch in the cervico - thoracic spine (spina bifida occulta).
The pubic bones are underdeveloped. A wide symphysis pubis is present with apparent widening of the scroiliac joints and Y cartilage of the acetubulum.
The iliac wings are hypoplastics, the pelvis is narrow. The ossification centers of the femoral heads are deformed with a rounded shape, the femoral necks are shortened. (Fig.4.)
Characteristic large metacarpals' and metatarsals' pseudoepiphyses are seen which produce a long second metacarpal, short terminal phalanges with massive epiphyses (marked in the thumb and in the toe), brachymesophalangia 5, and cone epiphyses. (Fig. 5,6.)
Discussion
Clinical features include large brachycephalic head with frontal and parietal bossing, large fontanels, small face with a broad nasal bridge, hypertelorism, high - arched palate, occasional cleft palate, failure of eruption of the teeth, supernumerary teeth and hearing loss.
In addition to the craniofacial changes, the affected children show dropping shoulders, narrow chest and scoliosis. They are capable of approximating their shoulders anteriorly across their chest. Genu valgum and short stubby fingers with rounded nails are the main limb manifestations. Abnormal gait, muscular hypotonia and joint hypermobility are common. These children have normal intelligence. [1,3,4]
Radiographic examination shows delayed ossification of the skull with delayed closure of sutures and fontanels. Early in life, ossification of cranial bones is markedly deficient, and there are multiple wormian bones. There may be platybasia. [5]
The facial bones are hypoplastic and the paranasal sinuses underdeveloped. The orbits are widely spaced and the mandible relatively large. [3]
The teeth may appear small, irregularly placed and crowded. Absence of some teeth or supernumerary teeth have been described. The mastoids are underpneumatized. [3] The clavicles present different degree of abnormality.
Complete absence is found in about 10% of patients. More often, small stumps are present medially, or the medial and lateral ends are present with a gap between them. Changes are usually bilateral and defects of the surrounding muscle are associated with it. Rarely, the patient may exhibit the other stigmata of the disease and have normal - appearing clavicles. [5]
The scapulae are small and winged. The acromial process may be wide and long.
The thorax is usually narrow, the ribs are short and oblique downwards. In the adult the sternum is sometimes incompletely ossified. [6]
There are vertebral abnormalities and scoliosis. Spina bifida occulta is observed in the cervical and upper thoracic levels, while the vertebral bodies retain their infantile shape with convex upper and lower plates into childhood. [1]
The pubic bones show failure of ossification. In the newborn, only ischial and iliac bone centers are present, the small bone centers of the ischiopubic and iliopubic rami absent.
With increasing age iliopubic and ishiopubic rami will appear, but the pubic bones ossify very late and may be defective even in adults, the symphysis pubis is greatly widened.
The Y cartigle of the acetabulum is wider and higher than normal.
The pubic bones are often underdeveloped resulting in a narrow pelvis. The iliac wings are frequently hypoplastic. Coxa valga or coxa vara can be found in a high percentage of cases.
The capital femoral center is commonly deformed with a rounded appearance. Genu valgum and obliquity of the ankle articular space is sometimes observed.
The long bones may appear broader and thicker than normal. Hypoplasia or even absence of the fibula and radius have been reported. [6]
Anomalies of the bones of the hands are most common. The 2nd and 5th metacarpals are longer than normal and supernumerary epiphyseal centers are commonly seen at their base. The 1st metacarpal is sometimes too short. The proximal phalanges are wide proximally and narrow and dense distally.
The middle phalanges are often short and this is almost constant for the 2nd and 5th fingers. The terminal phalanges are short and tapering, the ungual tuberosity is absent or smaller than usual.
During childhood cone - shaped epiphyses are commonly observed in the phalanges.
There may be retarded ossification of both carpal and tarsal bones. [6]
Irregular mineratization of the metaphyses simulate the lesions of metaphyseal chondrodysplasia. Pyknodysostosis and acroosteolysis must be considered in the diagnosis. [1,5]
In the newborn, diminished ossification of the calvarium is also seen in osteogenesis imperfecta and in congenital hypophosphatasia. The anomalies of the clavicle have been differentiated from congenital pseudarthrosis from resorption in hyperparathyroidism or after a trauma and from cleidofacial dysplasia.
Anomalies of the clavicle are also observed in trisomy 18, progeria, in focal dermal hypoplasia. [6]
In our patient a typical sceletal features with cranial, clavicular and pelvic abnormalities was found. Parents are unknown, so the genetic line (transmission) could not revealed.
Reference
1. Caffey's : Pediatric X-ray Diagnosis,
Ed. Silverman F.N. Year Book Medical Publisher, INC Chicago,
1985. pp. 565-568,
2. Kirks D.R. : Practical Pediatric Imaging,
Little Brown and Co., Boston, Toronto, London,
1991. pp. 303-305,
3. M.H. Reed : Pediatric Skeletal Radiology,
Williams and Wilkins, Baltimore, Hong Kong, London...
1992. pp. 50-52,
4. Swichuk : Imaging of the Newborn, Infant and Young Child
Williams and Wilkins, Baltimore, Hong Kong, London...
1989. pp. 832-834,
5. Taveras, Ferruci : Radiology
J.B. Lippincott Co., Philadelphia,
1990. Vol. 5, Ch 10., pp. 4-5,
6. Progress in Pediatric Radiology - Intrinsic Disease of Bones
Ed. H.J. Kaufmann, Philadelphia,
S. Karger, Basel, München...
1973. Vol 4, pp. 211-234,
Maria Deak M.D.
Department of Pediatric Radiology
Borsod County Teaching Hospital
3501. Miskolc PoBox 188, Hungary
Legends
Fig.1. This photograph shows the characteristic large head and "dropping" shoulders.
Fig.2. On cranial plain films lateral AP view (a) and view(b) brachycephalic skull
with platybasia and multiple wormian bones were seen.
Fig.3. Chest X-ray showed the hypoplastic clavicles, the small scapulae and the short
ribs.
Fig.4. On pelvic plain film hypoplastic iliac wings, narrowed pelvis, rounded femoral heads and shortened femoral necks were seen.
Fig.5. Comparative hands' plain film showed the long second metacarpals with
pseudoepiphyses and short terminal phalanges.
Fig.6. Comparative foots' plain films showed the marked epiphyseal ossification
centers in the toes.
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tartalomhoz
Calcification of posterior urethral valve
K.B. Jumah., A.A. Yeboah
Department of Radiology, University of Ghana Medical School
Korle Bu Teaching Hospital, Accra, Ghana
Abstract
A case of a 13 years old boy with posterior urethral valve calcification is presented. The diagnosis of this rare anomaly was made by plain film and micturiting cystourethrogram. Routine urine analyzis revealed Schistoma hematobium ova as the reason of urethral calcification.
Calcifications of the urinary tract may occur in bilharziasis, tuberculosis or tumor. Posterior urethral valves are the most common obstructive congenital lesions of the male urethra with an incidence of 1 in 5.000 to 8.000. The majority of patients are diagnosed before the age of one year, although presentations over the age of 20 years have been reported in as many as 20 percent of patients. Clinical spectrum varies from mild lower urinary tract obstruction to severe renal compromise (1,2,3). Urethral stones are not uncommon, usually having been passed down from upper urinary tract, but calcification of the urethra and more so of the posterior urethral valve has never been documented in the literature.
Keywords : Calcification, Posterior Urethral Valve, Bilharziasis
Case report
A 13 years old boy with nocturnal enuresis for 8 years was referred to the Korle Bu Teaching Hospital, Radiology Department for intravenous urography (IVU) examination. Abdominal plain film (Fig. 1.) demonstrated calcification in a dilated portion of the urethra. An IVU (Fig. 2.) showed normal pyelograms, ureters and bladder with no obstructive uropathy.
Micturiting Cystourethrogram (Fig. 3.) confirmed the calcification of posterior urethral valve and urethra. Routine urine examination revealed Schistisoma haematobium ova.
Discussion
Posterior urethral valves are the most common cause of lower urinary tarct obstruction during infancy and childhood. They occur almost exclusively in males owing to the embryologic organ (1,2).
The clinical picture in children with congenital posterior urethral valve present in a variety of ways depending primarily on the degree of obstruction and classically presenting symptoms are age dependant.
Neonates may present with ascites and palpable abdominal mass from distended bladder and hydronephrosis. In infants, it may present with failure to thrive, vomiting and easily palpable kidneys and bladder. In older children like this 13 years old boy, the presentation is more variable, frequently revolving around voiding problems, either nocturnal eneuresis or daytime urgency. The obstruction is correspondingly less severe and voiding may be normal in these children. Intravenous urograms are often normal or nearly so as in this case (3). Bilharziasis of the urethra may occur as the adult schistosoma haematubium worm may gain access to the urethra via connections between the vesico-prostatic venous plexus, the urethral veins and the dorsal veins of the penis. Lesions usually develop in three successive phases characterized by the formation of bilharzial granulations in the submucosa, followed by atrophic changes in the overlying mucosa and the development of superficial ulcers which heal with variable degree of scarring but bilharzial stricture of the urethra is a rare occurrence (4). Urethral calcification as a complication of urethral bilharziasis has never been reported in the literature and more so calcification involving the posterior urethral valve.
Reference
1. Aaronson IA : Posterior Urethral Valve : a review of 120 cases. S. Afri. Med J 65 : 418, 1984.
Cheever AW, Young SW, Shehata A.
2. Firlit C : Urethral abnormalities.
Urol Clin. North Am 5 : 31, 1978.
3. Eklof O., Olsson H : The Variability of Urographic findings in posterior urethral valves. Peditric Radiol 14 : 215, 1984.
4. Calcification of Schistosoma haematobium eggs : Relation of radiologically demonstrable calcification to eggs in tissue and passage of eggs in urine.
Transaction Royal Society Tropical Medicine Hygiene, 69 : 410 - 141, 1975.
Legends
Figure 1. Abdominal plain film showed urethral calcification.
Figure 2. Intravenous urogram showed normal filling of pyelocaliceal system, ureter, and bladder.
Figure 3. On micturiting cystourethrogram calcification of urethral valve and urethra was seen.
Jumah K.B. M.D.
Department of Radiology,
University of Ghana Medical School
Korle Bu Teaching Hospital, Accra, Ghana
Editorial comment
It will be interesting to know if there were any similar cases after the 1980s in the
literature.
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