Widespread antenatal screening has resulted in increased detection of anomalies of the kidneys and urinary tract. The present guidelines update the recommendations published in 2000. Antenatal hydronephrosis (ANH) is transient and resolves by the third trimester in almost one-half cases. The presence of oligohydramnios and additional renal or extrarenal anomalies suggests significant pathology. All patients with ANH should undergo postnatal ultrasonography; the intensity of subsequent evaluation depends on anteroposterior diameter (APD) of the renal pelvis and/or Society for Fetal Urology (SFU) grading. Patients with postnatal APD exceeding 10 mm and/or SFU grade 3-4 should be screened for upper or lower urinary tract obstruction and vesicoureteric reflux. Infants with vesicoureteric reflux should receive antibiotic prophylaxis through the first year of life, and their parents counseled regarding the risk of urinary tract infections. The management of patients with pelviureteric junction or vesicoureteric junction obstruction depends on clinical features and results of sequential ultrasonography and radionuclide renography. Surgery is considered in patients with increasing renal pelvic APD and/or an obstructed renogram with differential renal function <35-40% or its subsequent decline. Further studies are necessary to clarify the role of prenatal intervention, frequency of follow up investigations and indications for surgery in these patients.
Gastric teratoma (GT) comprises less than 1% of all teratomas in children. Though GT in the presence of immature neuroepithelial 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.
V89L polymorphism of the SRD5A2 gene is a strong determinant of hypospadias risk among children of Indian origin. However, our results suggest that the presence of leucine allele, especially among agriculturalists, may increase the propensity of having a child with hypospadias.
Intestinal atresia accounts for about one third of all cases of neonatal intestinal obstruction. The survival rate has improved to 90% in most of the series with the operative mortality being < 1%. The survival rate improves with distal atresias. An increased mortality is observed in multiple atresias (57%), apple peel atresia (71%), and when atresia is associated with meconium ileus (65%), meconium peritonitis (50%) and gastroschisis (66%). Although appearance of echogenic bowel on prenatal ultrasonography is suggestive of GI, it is confirmed in only 27% cases. Prenatal ultrasonography is more reliable in detection of duodenal atresia than more distal lesions. Short bowel syndrome is the major impediment in the management of jejunoileal atresia. Although total parenteral nutrition (TPN) is the main adjunctive treatment, it delays intestinal adaptation and may cause cholestasia and subsequent liver damage. Graduated enteric feedings, use of growth hormone, glutamine and modified diets containing low fat, complex carbohydrates and protein supplements have been used in a adults with short bowel syndrome to successfully diminish TPN requirements and enhance nutrient absorption in nearly half of the patients. Utilization of growth factors to facilitate intestinal adaptation and advances in small bowel transplant may improve the long-term outcomes in future.
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).
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