Consensus on the Management of Posterior Urethral Valves from Antenatal Period to Puberty.

The need for successful management of posterior urethral valves always captivates the minds of pediatric surgeons. Its success, however, depends on several factors ranging from prenatal preservation of upper tracts to postoperative pharmacological compliance. Regardless of measures available, some cases do not respond and progress to end stage. The management depends on several issues ranging from age and severity at presentation to long-term follow-up and prevention of secondary renal damage and managing valve bladder syndrome. This article is based on a consensus to the set of questionnaires, prepared by research section of Indian Association of Paediatric Surgeons and discussed by experienced pediatric surgeons based in different institutions in the country. Standard operating procedures for conducting a voiding cystourethrogram and cystoscopy were formulated. Age-wise contrast dosage was calculated for ready reference. Current evidence from literature was also reviewed and included to complete the topic.

Respiratory Distress Secondary to Rhabdomyosarcoma of the Tongue and Co-existent Choanal Atresia.

Whilst rhabdomyosarcoma (RMS) is the third most common solid tumour in children, congenital RMS of the tongue is extremely rare and usually present as painless progressive mass since birth (Dagher and Helman in Oncologist 4:34-44, 1999; Childs and Goudy in Int J Pediatr Otorhinolaryngol 5:126-128, 2010). In neonates, presentation with respiratory distress is unexpected as neonates are preferential nasal breathers and restricted oral breathing due to tumour usually poses no problem. We herein report a case of rhabdomyosarcoma of the tongue with co-existent unilateral choanal atresia, presenting with respiratory distress. The baby developed upper respiratory tract infection following which developed severe respiratory distress. Airway symptoms were precipitated as there was combined obstruction of both the nostrils due to infection or adenoid enlargement and unilateral chonal atresia. Treatment of respiratory distress in the presence of RMS and bilateral nasal pathology must first prioritise the security of the airway, before taking a multi-factorial approach to the therapy of the lingual mass (Childs and Goudy in Int J Pediatr Otorhinolaryngol 5:126-128, 2010). This case illustrates the importance of vigilance with respect to co-existent nasal pathology, in order to avoid the occurrence of complete airway obstruction. We therefore feel that any diagnosis of lingual RMS should warrant a formal examination of both nasal cavities.

In Vitro Evaluation of Carbachol and Endothelin on Contractility of Colonic Smooth Muscle in Hirschsprung's Disease.

Background: The hypomotility of colon observed in Hirschsprung's disease (HD) has been attributed to congenital aganglionosis only. So far, it is not clear whether the contractility of colonic smooth muscle in this condition is altered or not. Therefore, the present study attempted to understand the contractile status of colonic segments of HD patients by examining carbachol and endothelin (ET-1) evoked colonic smooth muscle contractions in vitro . Methods: Contractile responses were recorded from strips of colonic segments obtained from HD patients, using organ bath preparations. Cholinergic agonist carbachol and ET-1 along with their antagonists were used to evoke contractile responses. Thereafter, the samples were histopathologically confirmed for HD. Results: Colonic strips of HD did not show any spontaneous contractions but responded to carbachol and ET-1 to a lesser extent. In HD, response of carbachol was blocked by atropine and hexamethonium by nearly 73% and 50% respectively. ET-1 induced contractile responses were blocked by ET-A and ET-B antagonist up to 40%, signifying the possible role of ET-A and ET-B receptors in HD colon contractility. Conclusion: As evidenced by lack of spontaneous contractions and impaired carbachol and ET-1-induced contractile responses, it is concluded that, in addition to aganglionosis, decreased contractility of colonic smooth muscle may contribute to hypomotility observed in patients with HD.

Assessment and comparison of fecal continence in children following primary posterior sagittal anorectoplasty and abdominoperineal pull through for anorectal anomaly using clinical scoring and MRI.

INTRODUCTION: We aimed to compare and contrast these main surgical procedures for ARM in terms of structural outcome through pelvic MRI & functional outcome through Kelly's scoring. MATERIAL AND METHOD: A prospective study was conducted from August 2011 to July 2013 including all the cases of ARM managed in single stage (operated by one surgeon, first author) since 1995 that came for follow up at age of 3 years or more. Patients were divided in three groups: Group 8.A, PSARP (60 patients); Group B, Abdomino-PSARP (40 patients); and Group C, APPT (40 patients). The functional assessment of anal continence was carried out at the age of 3years or more using Kelly's method. Structural assessment was done by 1.5-T Magnetic Resonance Imaging (MRI). RESULTS: In 24 patients with rectobulbar fistula, 71.5% of Group A patients had good continence. In 50 patients with rectoprostatic fistula, 73.7% of Group A, 70% of group B and only 36% of Group C had good outcomes. Better development of each muscle was associated with better outcomes in terms of anal continence (P=0.001). CONCLUSION: There is better outcome with PSARP and Abdomino-PSARP in patients with rectobulbar and rectoprostatic fistula. MRI is a valuable modality for postoperative structural analysis of patients with ARM and is also useful for predicting the long term functional outcome of these cases.

Estimation of pro-renin as a prognostic marker for renal function in PUV patients.

OBJECTIVE: To estimate serum pro-renin, and its clinical significance, as a marker of chronic renal disease in posterior urethral valve (PUV) patients. PATIENTS AND METHODS: Forty patients with a PUV that were admitted to the hospital between 2010 and 2012 were reviewed. Twenty age-matched patients who were admitted for other non-urological diseases were selected for control. Clinical parameters, serum creatinine, urea, eGFR (estimated glomerular filtration rate) and serum pro-renin were analysed before and after valve ablation. RESULTS: Forty patients with PUV were included in the study. Three groups were formed according to age: <1 year, 1-3 years, >3 years. Pro-renin was measured using an ELISA (enzyme linked immunosorbent assay) kit and 'Graph Pad Prism' Software. The Spearman's rho test was used for correlation. Serum pro-renin had a negative correlation with the age group (correlation coefficient -0.395, P-value 0.012), eGFR (correlation coefficient -0.850, P-value<0.001) and follow-up eGFR (correlation coefficient -0.471, P-value 0.002). The pro-renin level correlated positively with serum creatinine at presentation (correlation coefficient 0.671, P-value<0.001), blood urea at initial presentation (correlation coefficient 0.684, P-value<0.001), serum creatinine at follow-up (correlation coefficient 0.546, P-value<0.001) and blood urea at follow-up (correlation 0.603, P-value<0.001). CONCLUSION: Pro-renin measured before PUV repair is associated with renal function three months after surgery.

Neonatal necrotising fasciitis managed conservatively: an experience from a tertiary centre.

OBJECTIVE: Conventional therapy for neonatal necrotising fasciitis (NF) involves resuscitation and aggressive surgical debridement of necrotic tissue. This approach adds surgical stress in septicaemic neonates with low reserves. The present study reports a more conservative approach to the management of neonatal NF. METHOD: A prospective study was conducted between July 2010 and June 2013 and included 11 cases of neonatal NF. Demographic characteristics of patients were noted. No debridement was performed. Dressings were applied after cleaning with normal saline every 24 hours. The necrotic slough was allowed to separate spontaneously. A record was made of type and duration of supportive modalities and complications. The primary outcome was recorded as final recovery, need of surgical intervention or need of grafting. RESULTS: Out of 11 neonates, 6 were males and 5 were females. Age ranged from 13 to 24 days (mean age 18.45±3.24 days), and weight varied from 1.8 to 2.6 kg (mean weight 2.12±0.24kg) at presentation. Duration of illness before admission to hospital ranged from 3 to 9 days (mean 4.84±2.13 days). The most common site of the initial involvement was the neck and upper thoracic region (n=4), scalp (n=3), lower back (n=2), face (n=1) and extremities (n=1). In all cases, the wound was cleaned with normal saline, dressed, necrotic slough was allowed to separate spontaneously and granulation tissue was allowed to develop; this took approximately 14 to 28 days (mean 20.45±5.78 days). CONCLUSION: As opposed to the conventional approach of aggressive debridement in neonatal NF, these cases can be managed conservatively without increase in morbidity or mortality. This approach also minimises the surgical risk and requirement of blood transfusion. Antifungal drugs have a definitive role in management of these immunologically deficient babies.

Non-operative management of giant omphalocele with topical povidone-iodine and powdered antibiotic combination: early experience from a tertiary centre.

PURPOSE: The aim of the study was to evaluate topical povidone-iodine and topical powdered antibiotic combination (Polymyxin, Bacitracin and Neomycin) in initial non-operative management with delayed closure of the defect of giant omphaloceles. METHODS: A prospective study was conducted between July 2010 and June 2013 including all neonates with giant omphalocele without signs of intestinal obstruction. All cases were managed by daily application of povidone-iodine (5% solution) followed by spraying topical powdered antibiotic combination to promote eschar formation and eventual epithelialisation. Record was made of sex, associated anomalies, length of stay, and thyroid function tests. RESULTS: Twenty-four neonates with giant omphaloceles were treated with topical povidone-iodine and topical powdered antibiotic combination. No sac ruptures were observed in our series. All patients had a normal thyroid function test at presentation and after 10 days of initiation of treatment. Six patients have undergone delayed repair. CONCLUSION: Topical povidone-iodine and powdered antibiotic combination promotes more rapid escharification and epithelialisation of the omphalocele than povidone-iodine alone. We also hypothesise that combination minimises the chances of hypothyroidism associated with use of povidone-iodine alone.

Intralesional bleomycin in lymphangioma: an effective and safe non-operative modality of treatment.

INTRODUCTION: Lymphangiomas are benign hamartomatous lymphatic tumors. The mainstay of the therapy is surgical excision, but due to its infiltration along the nerves and muscles, total excision is not always possible. In the present study, we have evaluated the clinical profile of all the cases of lymphagiomas coming to our department and evaluated the efficacy of intralesional Bleomycin as a sclerosing agent in its management. MATERIALS AND METHODS: In this prospective study, all patients were evaluated clinically and color Doppler ultrasonography (USG). The required dose was calculated as 0.5 mg/kg body weight, not exceeding 10 units at a time. The response was assessed clinically and on the basis of color Doppler USG. RESULTS: Thirty-five patients of lymphangioma were included in the study. The neck region was the most common site of involvement. The response was excellent in 7 (20%), good in 26 (74.29%), and poor in 2 (5.71%) patients. The complications included fever, transient increase in size of swelling, local infection, intraluminal bleed, and skin discoloration in 10 patients. CONCLUSION: This therapy may be used as primary modality instead of surgery in selected group of patients.

Rhabdomyosarcoma of biliary tract- a diagnostic dilemma.

Rhabdomyosarcoma is a soft tissue malignant musculoskeletal tumor. It is a rare tumor in children but the most common cause of malignant obstructive jaundice in them. A 4-year-old child presented to us with obstructive jaundice and palpable liver. He was misdiagnosed as choledochal cyst on imaging studies; however, intraoperative and histopathological features confirmed the diagnosis of rhabdomyosarcoma. Mass excision and Roux en Y portoenterostomy was performed. Post operatively; the patient was put on multimodal chemotherapy. This, being an uncommon entity, is being reported with a review of the available literature.

Unusual content of omphalocele: a congenital mature cystic teratoma of falciform ligament of the liver.

Omphalocele is a congenital midline abdominal wall defect that results in herniation of intraabdominal contents covered by a lining of peritoneum and amnion. The severity of the defect often ranges from a minor herniation of the umbilical cord to a significant protrusion that includes large proportions of intestine and liver. We report unusual content of omphalocele that is congenital mature cystic teratoma of falciform ligament of liver which was confirmed by histopathology. This is a very uncommon entity and not described in world literature.

Split notochord syndrome associated with dorsal neuroenteric fistula: A rare entity.

Split notochord syndrome (SNS) is an extremely rare congenital malformation associated with anomalies of the vertebral column, gastrointestinal tract and central nervous system. Twenty cases of SNS associated with dorsal enteric fistula have been reported in literature till date. The present report describes a unique case of SNS associated with lumbosacral meningomyelocele, dorsal neuroenteric fistula and dorsal herniation of right kidney along with vessels. The neonate was well managed by excision of enteric fistula, closure of duramater of meningomyelocele and repair of posterior wall hernia after placement of kidney in renal fossa. This kind of entity is uncommon and not been reported earlier.

A study of the functional aberration of the pouch in anorectal malformation associated with congenital pouch colon.

OBJECTIVE: There are no in vitro studies of congenital pouch colon (CPC) associated with anorectal malformation (ARM) to understand its mechanism of contractility. There is no consensus regarding the utilization of the CPC in repair of anorectal malformation. In view of the above, it was decided to carry out detailed in vitro physiological study of the excised CPC. METHOD: The excised specimens of CPC were taken and 15- to 20-mm long both longitudinal and transverse strips of muscle were obtained both from the proximal and distal part. Contraction was recorded using software chart-5 for windows. Drugs used were acetylcholine, histamine, adrenaline, atropine and pheniramine maleate. The amplitude of contractions was converted to tension gram and then the tension so developed was expressed as tension per unit mass (per gram of wet tissue). RESULTS: There were total of 21 specimens of high ARM with CPC. Five specimens were of the complete pouch, which did not show a response to any drug and the remaining 16 had an incomplete pouch. The mean longitudinal and circular muscle contractions of these 16 samples were statistically higher for the proximal segment than the distal segment both for acetylcholine and histamine. The effect of atropine was not significant but that of adrenaline and pheniramine maleate were significant on the distal segment muscle. CONCLUSION: Congenital pouch colon being deficient or having poorly developed receptors in their wall can not function properly as a reservoir for faeces and, hence, it should be excised to allow adequate function.

Chronic bilious vomiting in children in developing countries due to high bowel obstruction: not always malrotation or tuberculosis.

BACKGROUND: Bilious vomiting, in conjunction with abdominal pain is considered to be a surgical problem, unless proved otherwise. In children, besides tuberculosis (TB), we have found jejunal stricture (JS) due to non-specific jejunoileitis (NSJI) to be an important cause of chronic high small bowel obstruction and bilious vomiting. MATERIALS AND METHODS: In this retrospective study, the records of all children with complaint of intermittent bilious vomiting and failure to thrive were evaluated. Investigations included oral contrast study, ultrasound abdomen, chest X-ray and Mantoux test. Final confirmation was made at laparotomy. Treatment included jejuno-jejunal resection and anastomosis. Histopathology of the specimen was done to look for caseation, granuloma formation and other details. RESULTS: Out of total 100 patients with the complaint of bilious vomiting, 25 were having JS. Radiologic confirmation was possible in 19 (76%) patients of JS. No patient had evidence of TB as per our protocol. Histopathology revealed non-specific ischemic changes in all specimens. CONCLUSION: Jejunal stricture due to NSJI is a common entity in our setup leading to bilious vomiting. Contrast study can provide high index of suspicion in most of the patients. The diagnosis must be confirmed after proper histopathological examination. The results of the surgery are excellent.

Placement of the peritoneal end of a ventriculoperitoneal shunt in the suprahepatic space: does it improve prognosis?

INTRODUCTION: Hydrocephalus is a common pediatric problem. Ventriculoperitoneal shunts (VPS) are the most frequent operative procedures used to treat hydrocephalic children. The peritoneal end is usually placed in the general peritoneal cavity. We present an alternative site of peritoneal end placement in the suprahepatic space in an attempt to reduce the abdominal complications. MATERIAL AND METHODS: All patients with a diagnosis of congenital hydrocephalus were included in the study. In group 1, the lower end of the VPS was placed in the suprahepatic space. Patients were evaluated for abdominal complications like pseudocyst formation, intestinal obstruction and blockage of the lower end of the VPS. The data were compared with those patients in whom the peritoneal end was placed in the general peritoneal cavity (group 2). RESULTS: The total number of patients in groups 1 and 2 was 133 and 175, respectively. Complications in group 1 were dislodgement of the shunt in the general peritoneal cavity in 28 (21.05%), suprahepatic pseudocyst formation in 2 (1.5%) and blocked lower end in 2 patients (1.5%). In group 2, complications noted were pseudocyst formation in 5 (2.8%), blocked lower end in 25 (14.2%), intestinal obstruction in 9 (5.1%), inguinoscrotal migration in 10 (5.7%) and perforation of viscera in 6 patients (3.4%). The overall follow-up period ranged from 1 to 7 years. CONCLUSIONS: Placement of the lower end of the shunt in the suprahepatic space can be advantageous to placing it in the general peritoneal cavity. The procedure is simple and results can be rewarding.

Atresia of ileocecal junction, ileocecal valve: Rare variants of bowel atresia.

Atresia of ileocecal junction and isolated atresia of ileocecal valve are rare types of intestinal atresia with very few reports in literature. We report two such cases. Radiology showed dilated ileal segment and distal micro colon in both the cases. At laparotomy there was atresia of ileocecal junction in the first case and isolated ileocaecal valve atresia with normal ileocecal junction in the other case. Both the babies were managed by ileocolic resection with an end to end anastomosis. The prognosis of ileocecal atresias is satisfactory.

Concavo-convex oblique anastomosis technique for jejuno ileal atresia.

AIM: To evaluate the role of end-to-end oblique bowel anastomosis in bowel atresia. METHODS: End-to-end oblique anastomosis was done in 25 neonates of bowel atresia and the results were compared with traditional method of end to back anastomosis in 25 cases. RESULTS: We found less mortality and morbidity (5%) in our technique as compared to end to back technique (20%). CONCLUSIONS: We recommend this technique, as anastomosis is wide with less angulations, flow of effluent is linear, and there is less force exerted over post-anastomotic side wall.