Multicystic dysplastic kidney - A need for conservative and long term approach.

Objective. We retrospectively studied our cases of Multicystic Dysplastic Kidney (MCDK). The review was aimed at identifying the pattern of the disease in Indian Scenario and the required management thereof. Methods. We studied the clinical, radiological and nuclear scan findings of 22 patients with unilateral MCDK. They were diagnosed and/or treated in our unit from 1999 to 2007. The diagnosis was achieved by Ultrasound and further confirmed by DMSA scans. Other ancillary investigations like Micturating cystourethrogram were done if indicated. These patients were followed and followup investigations consisted of renal ultrasound, blood pressure measurement, and urinalysis and blood biochemistry. Results. A total of 22 patients (18 boys and 4 girls) with unilateral MCDK were investigated and followed for a mean period of 41 months. MCDK was detected on antenatal ultrasound only in 12(55%) and postnatally in 10(45%) babies. Mean age for postnatal diagnosis was 20 months. Follow up ultrasound revealed complete involution of MCDK in 3 patients and partial regression in 11 patients. The size of dysplastic kidney was unchanged in 4 patients and a further 4 patients underwent nephrectomy. Indications of nephrectomy were parental anxiety in 2, hypertension in 1 and palpable mass in 1. Conclusion. Large proportion (45%) of patients in presented series are diagnosed post natally contrary to western world where more than 80% are diagnosed antenatally. Uncomplicated isolated MCDK carry good prognosis with nephrectomy required in only a few patients. Association with other urological anomalies in ipsilateral/contralateral genitourinary tract is important to identify as they have worse outcome in terms of ultimate renal function. All patients with simple/complex unilateral MCDK should be advised long term follow up for the possible development of hypertension and/or hyper infiltration injury.

Risk factors for renal injury in patients with meningomyelocele.

PATIENTS AND METHODS: Thirty operated patients of myelodysplasia were clinically evaluated for the age at presentation, the extent of lesion and neurological deficit. Urological assessment was done with urine cultures, serum creatinine, radiological (ultrasound of kidney, ureters and bladder, voiding cystourethrogram) and urodynamic (water cystometry) parameters. An objective scoring for bladder (Galloway, et al.) was applied. Dimercapto-succinic acid (DMSA) scan was done in all the patients for evidence of renal scars. The results of above investigations were correlated with presence or absence of renal scars (renal injury) on DMSA scan. None of the patients had received any prior bladder care. RESULTS: Twenty one patients had no renal scars and 9 patients had evidence of renal scarring. Patients with renal scars were older at presentation, they had greater degree of hydroureteronephrosis (P < or = 0.001) and vesicoureteric reflux (P < or = 0.005). The incidence of high leak pressures (>25 cm of water, P < or = 0.05), unacceptable bladder volumes (maximum cystometric capacity < 60% for age, P < or = 0.005) and high risk Galloway's score (> 5, P < or = 0.05) was high in patients with associated renal scarring as compared to their nonscarred counterparts. Three of these patients had serum creatinine >1 mg/dl (P < or = 0.005). The incidence of urinary complaints and positive urine cultures was also higher in these patients (NS). CONCLUSION: Increasing age, evidence of hydroureteronephrosis and vesicoureteric reflux, high leak pressures, low bladder volume and high combined Galloway score (>5) define a high risk bladder in our population and predispose to renal injury in patients of myelodysplasia. Early referral for bladder risk assessment and management of all myelodysplasia patients is recommended.

Anterior urethral valves.

Posterior urethral valves (PUV) are the most frequent cause of obstructive uropathy in boys. Rarer causes of obstructive uropathy include bladder diverticulae, meatal stenosis and urethral or bladder stones. Anterior urethral valves are rare causes of urinary obstruction in boys and are ten times less frequent than PUV in the literature(1-6). This paper highlights our experience with 5 patients with anterior urethral valves.

Conservative management of giant hepatic mesenchymal hamartoma.

We report a six-month-old male infant with mesenchymal hamartoma of the liver which was left untreated. Repeat CT scan 1 year later showed regression and calcification of the tumor. The child is well 3 years later.

Non-syndromic paucity of interlobular bile ducts.

A 3 1/2-month-old male infant presented with cholestatic jaundice. Peroperative cholangiogram showed a gall bladder and small but patent extrahepatic bile ducts. Liver histology showed paucity of interlobular bile ducts. The child is being treated medically for his symptoms.