Minu Bajpai

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.

Percutaneous nephrostomy in children with ureteropelvic junction obstruction and poor renal function

OBJECTIVES To evaluate the results of performing percutaneous nephrostomy (PCN) in all patients with kidneys with ureteropelvic junction obstruction (UPJO) and split renal function (SRF) of less than 10%, because the management of such cases is still under debate. If SRF improves, we perform pyeloplasty, otherwise nephrectomy is done. METHODS The records of all patients with UPJO (n = 20; 17 unilateral and 3 solitary kidney) with poor function who underwent PCN were analyzed. The PCN remained in situ for at least 4 weeks, during which patients received low-dose oral trimethoprim prophylaxis. Repeated renography was obtained after 4 weeks. If no improvement in the SRF had occurred, nephrectomy was performed, otherwise pyeloplasty was performed. The patients were followed up with renograms at 3 months, 1 year, 2 years, and 5 years. RESULTS Twelve of 17 kidneys with unilateral UPJO improved after PCN drainage and underwent pyeloplasty. The 5 kidneys that did not show improvement in the SRF underwent nephrectomy. In the patients with unilateral UPJO who improved after PCN drainage, the SRF increased to 29.2% +/- 12.6% and pyeloplasty was performed. At a mean follow-up of 2.3 years, none of these patients had developed hypertension, and the most recent SRF value was 31.4% +/- 12.8%. CONCLUSIONS Most of the poorly functioning UPJO kidneys show improvement in function and not all such kidneys should be removed without a trial of PCN.

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).

NONOPERATIVE MANAGEMENT OF NEONATAL MODERATE TO SEVERE BILATERAL HYDRONEPHROSIS

PURPOSE The natural history of prenatally diagnosed bilateral ureteropelvic junction hydronephrosis is unknown. No definite recommendations exist regarding management, and many authors recommend unilateral or bilateral pyeloplasty. We present the results of nonoperative management of bilateral hydronephrosis. MATERIALS AND METHODS A total of 16 patients (32 kidneys) with prenatally diagnosed moderate to severe bilateral hydronephrosis were followed nonoperatively. Pyeloplasty was performed only for evidence of deterioration of function and only unilateral surgery was performed when necessary. RESULTS Of the 32 kidneys 31 had moderate or severe hydronephrosis and 4 (12.5%) required pyeloplasty during a mean followup of 36 months. Hydronephrosis spontaneously resolved or improved in 78% of those observed nonoperatively. Of the 25 kidneys followed nonoperatively for at least 2 years 80% demonstrated improvement. While initial drainage half-time was more than 20 minutes in 55% of kidneys, 78% of unoperated kidneys had a final half-time of less than 20 minutes. Of the 10 kidneys with bilateral severe hydronephrosis 3 (30%) required pyeloplasty. All children had normal glomerular filtration rate for age at the latest followup. Only 1 child has persistent bilateral severe hydronephrosis at age 1 year. CONCLUSIONS Initial nonoperative observation appears to be safe in all cases of moderate to severe bilateral neonatal hydronephrosis. Spontaneous improvement can be expected in most kidneys by 2 years. Only a few children require unilateral pyeloplasty and bilateral operation can be avoided in most cases.

Hypospadias Risk and Polymorphism in SRD5A2 and CYP17 Genes: Case-Control Study Among Indian Children

PURPOSE Hypospadias is a common congenital error of genital development, the frequency of which is increasing. As androgens have a significant role in the development of the male urethra, we sought to investigate the association between 2 functional polymorphisms, CYP17-A1/A2 and SRD5A2-V89L, which are involved in the biosynthesis of testosterone and dihydrotestosterone, respectively, in relation to hypospadias. MATERIALS AND METHODS We examined DNA samples of 80 cases and 100 controls for SRD5A2-V89L and CYP17-A1/A2 gene polymorphisms. Information pertaining to family history, preoperative position of the urethral meatus and parental occupations along with maternal reproductive profile were collected for cases and controls. RESULTS Genotyping of 80 cases and 100 controls revealed a significant association between V89L polymorphism and hypospadias (OR 2.4, 95% CI 1.2-4.6, p <0.05). When analyzing the risk of hypospadias based on grade, genotypic distribution of SRD5A2-LL genotype differed significantly between severe forms and controls, with an odds ratio of 3.6 (95% CI 1.2-10.0, p = 0.02). Of affected children 71.25% had parents from a rural background, with agriculture as the primary occupation. A statistically significant association was observed for the LL genotype (OR 4.6, 95% CI 1.7-12.29, p <0.05) between children with parents having an agricultural background (likely exposed to pesticides) and controls with no such exposure. CYP17-A1/A2 genotypes did not show any significant results. CONCLUSIONS 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.

Polymorphism in CYP1A1, GSTMI, GSTT1 genes and organochlorine pesticides in the etiology of hypospadias

Exposure to endocrine-disrupting chemicals (EDCs) and maternal endogenous estrogen may cause hypospadias, common congenital anomaly. Several organochlorine pesticides (OCPs) have been reported to possess an endocrine-disrupting potential. Cytochrome P4501A1 (CYP1A1) and glutathione S-transferases (GSTM1 and GSTT1) of xenobiotic metabolizing enzyme family are involved in the metabolism of various environmental toxicants and steroidal hormones. Hence, the present study was designed to evaluate the role of CYP1A1, GSTM1, GSTT1 genes polymorphism, OCPs levels and risk of hypospadias. A total of 80 hypospadiac and 120 age-matched control boys were included. OCP levels in blood were determined using Gas Chromatograph equipped with electron capture detector (GC-ECD) and polymorphism in CYP1A1, GSTM1 and GSTT1 genes was evaluated by RFLP and multiplex PCR method. We observed significant high levels of β-hexachlorohexane (HCH), γ-HCH, and p,p′-dichlorodiphenyl-dichloroethylene (p,p’-DDE) in the cases. CYP1A1 polymorphisms were not significantly different among cases and controls, whereas concomitant deletion of GSTM1 and GSTT1 genotypes was significantly higher in cases as compared to controls. However, after adjusting for low birth weight and maternal occupational exposure, the results did not remain significant but odds of risk was higher (OR = 1.72, p = 0.14) among cases. In conclusion, our study suggests irrespective of genetic predisposition, higher level of some OCPs may be associated with increased risk of hypospadias.

Epigastric heteropagus twins--a report of four cases.

Abstract Asymmetrical conjoined twinning is an extremely rare congenital anomaly constituting only 1%–2% of all conjoined twins. In epigastric heteropagus twins (EHT), the dependent portion (parasite) is smaller than the host (autosite). The embryopathy is related to incomplete cleavage of the embryo at 2 weeks of gestation. However, some form of ischaemic insult in early gestation leads to selective atrophy of the cranial part of one of the monozygous twins. We report our experience with four cases of EHT and a review of the literature with a discussion of possible embryopathy.

Revised guidelines on management of antenatal hydronephrosis

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.

Prognostic Significance of Captopril Renography for Managing Congenital Unilateral Hydronephrosis

PURPOSE In this prospective study we evaluated the results of captopril enhanced diuretic renography in patients with congenital unilateral ureteropelvic junction type hydronephrosis. Captopril renography helps to distinguish patients who have renin-angiotensin system activation from those in whom it is not yet activated. Renin-angiotensin system activation heralds the beginning of compensatory vasoactive response in the kidney. Identifying grades of such activation may help in determine the end points of nonoperative management. MATERIALS AND METHODS We prospectively studied 25 patients with suspected unilateral ureteropelvic junction obstruction treated at our department. A prenatal diagnosis was made in 60% of cases. Patient age was 1 to 144 months (mean 40) and the male-to-female ratio was 4:1. Followup was 6 to 72 months (mean 30). Patient evaluation and analysis included sonography, standard diuretic and captopril renography, glomerular filtration rate, serum creatinine and blood pressure. The indications for surgery were symptoms, differential function below 35%, or a documented decrease of 10% or more during followup. Activation of the renin-angiotensin system was considered positive when split renal function decreased significantly by 5% or more in an obstructed system on a post-captopril study. The results of captopril renography were examined retrospectively in patients who required surgery according to preexisting criteria. RESULTS Society for Fetal Urology hydronephrosis grade was II to IV in 8, 10 and 7 renal units, respectively. The renin-angiotensin system was activated in 8 of the 25 cases (32%) of unilateral ureteropelvic junction obstruction. In 15 patients there was no change on pre-captopril and post-captopril studies and in 2 split renal function on the obstructed side marginally increased on the post-captopril study. Surgical correction was required in only 6 of the 8 patients with renin-angiotensin system activation. Of those who required surgery hydronephrosis was grades II to IV in 3, 1 and in 2 patients, respectively. CONCLUSIONS In the current study renin-angiotensin system activation correlated with the need for surgery in 75% of the cases of congenital unilateral ureteropelvic type hydronephrosis. Since captopril serves to identify patients who have renin-angiotensin system activation, it may also be possible to grade this activation. Our preliminary results show that there may be a role for captopril renography for identifying the risk group for surgical intervention.

Revised guidelines on management of antenatal hydronephrosis.

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 (VUR). Infants with VUR 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.