Assessing the relationship between hypospadias risk and parental occupational exposure to potential endocrine-disrupting chemicals.

Objective: The association between periconceptional parental exposure to endocrine-disrupting chemicals (EDCs) and hypospadias remains inconclusive and controversial. Therefore, we conducted a hospital-based retrospective study to assess the relationship between hypospadias risk and parental occupational exposure to potential EDCs.

Methods: Incident cases (n=73) were boys between 0 and 14 years diagnosed with hypospadias with no micropenis or cryptorchidism.

Normative magnetic resonance imaging measurements of orbital structures in pediatric population of North-Eastern India: A retrospective cross-sectional study.

Purpose: The people of Northeast India comprise mostly of Mongoloid descent with characteristic craniofacial features. The purpose of this study was to evaluate the normal orbital structures with MRI and determine normative data in the Northeast Indian pediatric population.

Methods: MRI images of the 302 orbits of 151 pediatric patients below 16 years of age were retrospectively evaluated to measure the various orbital structures.

Long telomeres cooperate with p53, MDM2, and p21 polymorphisms to raise pediatric solid tumor risk.

Background: While leukocyte telomere length has been linked with altered risk in adult cancer, limited information is available on its association with risk in pediatric solid tumors. We investigated the association of telomeric alterations with risk of pediatric solid tumors. We also investigated whether altered telomeres cooperated with the TP53 rs1042522, MDM2 rs2279744 and CDKN1A (p21 ) rs1059234 single-nucleotide polymorphisms to modify cancer risk.

Indocyanine green clearance test to evaluate liver function in rat model of extrahepatic biliary atresia.

Background: Indocyanine green clearance test (ICG-K) has been shown as a sensitive marker of liver function in patients with cirrhosis. However, its role in the assessment of liver function in children with biliary atresia is not well established. The present study was undertaken to evaluate the ICG-K in an experimental model of cholangitis and partial biliary obstruction.

Extrahepatic Portal Vein Obstruction in Children: Role of Preoperative Imaging.

Aim: Extrahepatic portal vein obstruction (EHPVO) is characterized by features of recent thrombosis or portal hypertension with portal cavernoma as a sequel of portal vein obstruction. Imaging of spleno-portal axis is the mainstay for the diagnosis of EHPVO. The aim of this study is to analyze the role of imaging in the preoperative assessment of the portal venous system in children with EHPVO.

Growth and Psychological Development in Postoperative Patients With Anterior Encephaloceles.

Purpose: Anterior encephaloceles are rare malformations that are frequently associated with other brain anomalies. This study evaluates the growth and psychological development of children following encephalocele repair.

Materials & Methods: Growth and psychological assessment was done in 24 children with only encephalocele (group I); nine children with encephalocele and hydrocephalus (group II); seven children with encephalocele, hydrocephalus, and secondary malformations (group III); and 40 apparently healthy control subjects.

Evidence of gene-gene interactions between MTHFD1 and MTHFR in relation to anterior encephalocele susceptibility in Northeast India.

Background: Anterior encephalocele (AE) is a rare congenital anomaly of the central nervous system which is thought to be associated with genetic defects in folate metabolism.

Methods: This case-control study investigated the interactions of methylenetetrahydrofolate dehydrogenase 1 (MTHFD1)-1958G>A (rs2236225) and the methylenetetrahydrofolate reductase (MTHFR) - 677C>T (rs1801133) and 1298A>C (rs1801131) polymorphisms with the risk of AE in the Northeast Indian population. A total of 40 AE cases and 80 controls were investigated using polymerase chain reaction-restriction fragment length polymorphism technique.

Ultrasonography and magnetic resonance imaging evaluation of pediatric spinal anomalies.

Context: Spinal dysraphisms are congenital abnormalities of the spine due to imperfect fusion of midline mesenchymal, bony and neural structures. Imaging plays a vital role in their evaluation as significant portion of patients may present with concurrent anomalies that need to be corrected simultaneously to avoid repeat surgeries.

Aims: The aims of the study were to evaluate Spinal dysraphisms using USG and MRI and to correlate imaging findings with operative findings in patients undergoing surgery.

Gluteal teratoma: A rare site of extragonadal teratoma.

Extragonadal teratomas are rarely encountered in infants and children. These tumors are reported in retroperitoneum, floor of the mouth, mediastinum, craniofacial region or even in the solid organs of the body. Most of the gluteal teratomas reported in the literature are in fact, lateralized sacrococcygeal teratomas.

Sincipital Encephaloceles: A Study of Associated Brain Malformations.

Objective: The aim of this study was to evaluate the associated intracranial malformations in patients with sincipital encephaloceles.

Materials And Methods: A hospital-based cross-sectional study was conducted over 8 years from June 2007 to May 2015 on 28 patients. The patients were evaluated by either computed tomography or magnetic resonance imaging whichever was feasible.

Segmental dilatation of ureter: Report of two cases.

Segmental dilatation of ureter is a giant, focal segmental ureteral dilatation producing an elongated and distorted ureter. Two children presented with this condition, one had ipsilateral megacalycosis and contralateral vesicoureteric reflux. The other had duplication of the kidney.

Cloacal exstrophy: a single center experience.

Objective: Cloacal exstrophy is an exceedingly rare and complex anomaly. The records of 23 patients treated in a tertiary care center with limited infrastructure were analyzed for anatomic types, associated anomalies, surgical procedures adopted, and the outcome.

Materials And Methods: There were 14 males.

The prune belly syndrome in a female foetus with urorectal septum malformation sequence: a case report on a rare entity with an unusual association.

The prune belly syndrome is a rare congenital anomaly which is characterized by the triad of an absent or a deficient development of the abdominal muscle, bilateral cryptorchidism and an anomalous urinary tract. In its full form, this condition occurs only in males. However, a similar condition occurs in females in the absence of cryptorchidism.

Meatal and corpus spongiosum advancement: a better technique for distal hypospadias repair.

Objective: Several procedures have been described for repair of distal hypospadias. However, these are not without any complications. Corpus spongiosum advancement is a new technique employed in our patients for distal hypospadias repair with excellent clinical outcome and no post-operative complications.

Hepatic lobectomy and mucosectomy of intrahepatic cyst for type IV-A choledochal cyst.

Objective: Excision of extrahepatic cyst with wide biliary-enteric anastomosis is the treatment of choice in choledochal cyst. The diseased mucosa of the residual cyst may be the cause for postoperative complications. Mucosectomy of the cyst wall may prevent such complications in type IV-A choledochal cyst.

Congenital malformations in Assam.

Aim: To determine the annual incidence of congenital malformations in Assam and to analyze the data.

Materials And Methods: Data regarding babies born with congenital malformations in the state of Assam during the year 2006 were obtained through questionnaires and analyzed. The results were compared with similar Indian data.

Clinical experience with a new modified transanal endorectal pull-through for Hirschsprung's disease.

Single-stage transanal endorectal pull-through (TEPT) procedure is now widely used for rectosigmoid Hirschsprung's disease (HD). However, this procedure is associated with some common complications like stricture, enterocolitis, constipation, anastomotic dehiscence and perianal excoriation. The aim of this study was to evaluate the clinical outcomes of a modified TEPT operation in the management of rectosigmoid HD.

Type IVa choledochal cyst: Results following haemihepatectomy and mucosectomy of intrahepatic cyst.

Choledochal cyst is a rare condition characterised by congenital dilatation of the biliary tree. Commonly seen in the oriental countries, patients usually present with a varying combination of abdominal pain, jaundice, lump or cholangitis. Untreated patients or incomplete removal of the cysts usually leads to portal hypertension and cholangiocarcinoma.

Anterior encephaloceles in children of Assamese tea workers.

Object: Anterior encephaloceles are rare congenital malformations. Most of the cases in the literature are reported from Southeast Asia. In India it is seen more frequently among manual laborers in the tea gardens of Assam.

A novel, easy, non-operative method of treating prolapsed colostomy.

Colostomy prolapse is a major cause of morbidity in paediatric patients with Hirschsprung's disease and anorectal malformations. Although it is commonly associated with the distal loop of a transverse colostomy, a sigmoid stoma can also be affected. We report six babies with anorectal malformations between day 10 and 6 months presenting with incessant crying and irritability following prolapsed colostomy stomas.

Embryogenesis of esophageal atresia: Is localized vascular accident a factor?

Several theories on embryogenesis of esophageal atresia have been proposed, none could explain the whole spectrum of this anomaly. We report a new variant of esophageal atresia in which the two blind pouches were joined by an atretic band. Histology of the atretic part showed groups of striated muscle arranged haphazardly without any lumen.