Impaired differential renal function in a child with pheochromocytoma.

We report a girl with extra-adrenal pheochromocytoma complicated with impaired renal function of the ipsilateral kidney, discussing aetiologies. A 14-year-old girl presented with uncontrolled hypertension, high urinary vanillylmandelic acid level and a 5 x 6 cm mass at the right renal hilum. Her blood pressure was under control with propranolol, prazosin, and nifedipine before surgery, and with sodium nitroprusside during surgical intervention. The total tumour removal required reconstruction of the right renal vein. Histopathology confirmed pheochromocytoma. Dimercaptosuccinic acid (DMSA) after surgery showed low uptake of isotope by the right kidney; it was unclear if this was due to the surgery or the tumour causing renal artery stenosis, but fortunately her blood pressure returned to normal thereafter. We recommend obtaining a DMSA in the preoperative evaluation of pheochromocytoma.

The diagnostic value of anorectal manometry as a screening test for Hirschsprung's disease.

The diagnostic accuracy of anorectal manometry as a screening test for Hirschsprung's disease (HD) was assessed in 46 children suspected of having HD on clinical grounds. Among 33 children in whom a rectosphincteric reflex (RSR) was not demonstrated on manometric recordings, 31 were later proven to have HD by full thickness rectal biopsy: the remaining 2 children were shown not to have HD. None of the 12 children who had a normal RSR had HD. Thus, two false positive diagnoses were observed in this study, giving an overall diagnostic accuracy by anorectal manometry of 95 per cent and diagnostic sensitivity of 100 per cent. Anorectal manometry is an accurate, non-invasive test which has a place as the screening test of choice for HD.

Multiple endocrine neoplasia type IIb: a case report.

We reported a 14-year-old boy who had had multiple thyroid nodules for 4 yrs. Physical examination revealed marfanoid habitus, coarse facies with thick lips and prominent jaw. Mucosal neuromas were present on the buccal mucosa, tongue and upper eyelids. Thyroid scan demonstrated multiple, cold nodules and medullary thyroid carcinoma was confirmed by pathological examination. He was normotensive and his 24 hrs urinary vanillyl mandelic acid was in the normal range. Computerized tomography demonstrated normal adrenal glands. Multiple endocrine neoplasia type IIb (MEN IIb) was diagnosed by the appearance of typical phenotypic features, multiple mucosal neuromas and medullary thyroid carcinoma. The scarcity of such reports in children and the importance of early recognition of this disorder prompted us to describe this patient.

Posterior sagittal anorectoplasty for imperforate anus: a preliminary result.

Posterior Sagittal Anorectoplasty (PSARP) is a new technique which was pioneered by Pena and de Vries in 1982 for the repair of high anorectal malformations. It is based upon complete exposure of the anorectal region by means of a median sagittal incision that runs from the sacrum to the anal dimple cutting all muscle structures behind the rectum. The incision is kept in the median plane using a muscle or nerve stimulator as a guide. Between March 1988 and April 1989, we operated on 10 patients who had high and intermediate types of imperforate anus according to the "Wingspread Classification". All of the patients had PSARP as the definitive operation. The age of the patients were between 1.5 months and 15 years at the time of operation, four were under six months. Nine patients had this operation as the primary procedure, one patient had re-pullthrough after failure to gain continence by a previous conventional abdomino-perineal pullthrough operation. Six out of 8 patient had good results, two patients had fair results according to Kiesewetter's clinical criterion for fecal continence. Two patients are waiting for colostomy closure and are yet to be evaluated. Two of the good results included a 15-year-old girl who had high type with rectovaginal fistula, and a 2-month-old male infant with a recto-urethral fistula. In a re-pullthrough patient, the continence, as evaluated by loopogram and defecogram through the colostomy before closure of the colostomy, is good.(ABSTRACT TRUNCATED AT 250 WORDS)

Renal abscess in healthy children: report of three cases.

We report three cases of renal abscess in otherwise healthy boys. One had a history of blunt trauma and two of antecedent skin infection. All presented with flank pain and fever. Urine and blood cultures were sterile. The diagnosis was made using ultrasound and computerized axial tomography. The first patient had recurrent renal abscess, following surgery and a short course of antibiotics. Eventually all responded well to long-term antibiotics only.