S. Dave

Gastric teratoma in children.

Abstract Gastric teratoma (GT) comprises less than 1% of all teratomas in children. Though GT in the presence of immature neuroepethelial elements is regarded as malignant, the prognosis is excellent after complete excision of the tumor. Because of its rarity the world literature lacks a large study. Clinical experience with ten cases of GT is presented and discussed. Only one patient was female; the mean age at presentation was 3.2 months. Two cases were immature grade III GT; one of these had infiltrated the left lobe of the liver and the transverse colon while the other had metastasized to the regional lymph nodes and omentum. All the patients underwent complete excision. There were no deaths, and after a mean follow-up period of 4.2 years, all the patients had no recurrence and were healthy. Both the mature and immature types of GT have an excellent prognosis after complete excision of the tumor. Even when the immature type infiltrates surrounding structures, complete excision offers recurrence-free survival without requiring chemo- or radiotherapy.

Factors affecting outcome in the management of posterior urethral valves

Abstract Children with posterior urethral valves (PUV) are at high risk for renal failure (RF). The outcome of renal function is significantly influenced by early diagnosis and the choice of primary therapy. We reviewed the outcome of renal function in 58 children with PUV. The choice of therapy in each case – primary valve fulguration, vesicostomy, or high ureterostomy – was individually decided on the basis of the response to initial catheter drainage of the bladder. Patient age at diagnosis varied from newborn to 5.5 years, and follow-up ranged from 1.6 to 6 years (mean 3.9 years). The most common procedure was primary endoscopic valve ablation, which was carried out in 56.8% of cases. The other procedures were vesicostomy in 32.75% and high ureterostomy in 10.45%. Most neonates (66.6%) had RF at presentation, but one-half of them had achieved normal serum creatinine values at last follow-up. The recovery of renal function was lowest (33%) in older children where the diagnosis was delayed. A comparison between two groups of neonates and infants who differed on the basis of creatinine concentrations at 1 year of age suggested a statistically significant trend: children with normal or near-normal serum creatinine (0.8 mg/dl or less) by 12 months of age maintained good renal function at the time of final evaluation (1.0 mg/dl or less). Children with higher creatinine values at 1 year of age continued to have progressive RF. Seventy-five percent of the patients who had undergone early high ureterostomy after failure to respond to initial catheter drainage had regained normal renal function. We conclude that: serum creatinine at presentation is not predictive of subsequent renal function, but the values after a period of urinary-tract decompression are prognostically more useful; delay in diagnosis results in a poor outcome of renal function; and for optimal recovery of renal function, the choice of the primary procedure varies from case to case and can be determined by a systematic, stepwise approach (stepladder protocol).

Augmentation colocystoplasty in bladder exstrophy

Abstract A good bladder capacity and adequate outlet resistance determine success after staged reconstruction of bladder exstrophy (BE). Augmentation cystoplasty (AC) is an established salvage procedure to treat the small, noncompliant bladders of some of these children. In a series of 89 patients with BE treated over the last 12 years, 19 underwent detubularized augmentation colocystoplasty (ACC) as an adjunctive procedure. Nine underwent ACC at the time of bladder-neck reconstruction (BNR) for small bladder capacity and poor compliance; 10 underwent ACC as a secondary procedure after BNR for persistent urinary incontinence or poor bladder compliance and upper-tract deterioration. The follow-up period ranged between 6 months and 12 years (mean 41 months). Complications included symptomatic urinary-tract infection in 4 patients, recurrent epididymo-orchitis in 2, calculi in 3, colonic anastomotic dehiscence in 1, bladder-patch fistula in 2, and secondary coloureteric-junction obstruction in 1. There was no postoperative reservoir perforation or mortality. The upper tract remained normal or stable in all patients. Nine of the 19 patients are dry both day and night; 4 others are dry during the day but have occasional nocturnal wetting. Three patients have nocturnal wetting with stress incontinence and 2 remain incontinent. Seven patients can void effectively using abdominal contractions and 12 require clean intermittent catherization to ensure complete voiding. The indications and results of AC in BE from other series are reviewed. AC is an important and safe adjunctive procedure in a subset of BE patients with small and poorly compliant bladders. Despite the known complications, more liberal use of AC in BE is warranted.

Betamethasone in plus Phenobarbitone prior to hepatobiliary scintigraphy increases diagnostic accuracy in infants with jaundice

Objective. In the diagnostic work up of the child with neonatal obstructive cholangiopathy (NOC), hepatobiliary scintigraphy (HBS) determines the need for peroperative cholangiography (POC). Traditionally, phenobarbitone is recommended to prime the liver to HBS. This retrospective study was designed to evaluate whether addition of the betamethasone (BM) alters the diagnostic accuracy of the HBS in distinguishing neonatal hepatitis (NH) from extra hepatic biliary atresia (EHBA).Methods : Between 1993 – 1999, 202 patients presented with NOC and this study was not designed as a prospective randomized clinical trial. Of these, 126 patients had received Phenobarbitone (Group I) and the remaining 76 (Group II) had received BM in addition to the PB in a dose of 5 mg/k/d and 2.2 mg/k/d respectively for 7 days prior to HBS.Results : Retrospective analysis revealed that, in the Group I, 41 showed excretion and 85 did not show any excretion of the radiopharmaceutical and the latter underwent POC which revealed that 31 patients (36%) of them showed patent biliary tract. In group II, 32 patients revealed excretion and 44 did not show any excretion of the radiopharmaceutical and the latter had undergone POC, which revealed that only 8 patients (18%) showed patent biliary tract. The percentages of false positives (36% vs 18%) was statistically significant (p<0.03).Conclusion : Addition of BM increases the diagnostic accuracy of the HBS and this would lead to decreased need for POC to distinguish NH from EHBA.

Cystometric evaluation of reconstructed classical bladder exstrophy

Objective To evaluate the association of urodynamic variables with continence and upper tract status after reconstruction in patients with classical bladder exstrophy.

The role of imipramine therapy in bladder exstrophy after bladder neck reconstruction

Objective To assess the role of imipramine therapy after bladder neck reconstruction in patients with bladder exstrophy.

Duodeno-renal fistula due to a nasogastric tube in a neonate

Abstract Major complications due to nasogastric tubes (NGT) are uncommon. Esophageal and duodenal perforations have been described. We describe a newborn in whom a NGT led to a duodenal perforation, causing duodeno-renal fistula.

Advanced biliary atresia: is portoenterostomy justified in all infants?

During 1989–98, of the 127 patients with biliary atresia, 23 were seen with advanced biliary atresia (ABA) presenting with (i) at more than 120 days of age (ii) established cirrhosis and (iii) features of portal hypertension. Sixteen of these underwent exploratory laparotomy, dissection at the porta hepatis and hepatico-portoenterostomy (HPE) (group I). The remaining 7 infants underwent portal dissection and sump drainage only (instead of HPE). The drainage if any was evaluated for any change in colour, volume and concentration in the post-operative period (group II). Age and presentation were the same in both the groups. In group-II, HPE was considered only if the bile flow was noted after portal dissection. Bile flow was seen in 3/16 in group I and 0/7 in group II. The fall in serum bilirubin during the first seven post operative days was noted in 2/16 in group I and 0/7 in group II. No drain output was recorded in any of the group II infants. The incidence of complications and the duration of hospital stay was significantly higher in group I patients. The sump drainage as an alternative procedure to HPE not only served the purpose of evaluating the patients with ABA for the possible bile flow in the post operative period but also avoided the need for a major operative procedure like HPE.