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. Controls (n=146) were an age-matched group of boys without any congenital malformations, inguinal hernia, nephrological, urological and genital disorders. Their selection was independent of exposures to EDCs. Data on parental occupation and sociodemographic variables were collected using a structured questionnaire. We evaluated parental occupational exposures using a previously validated job-exposure matrix (JEM) for EDCs. RESULTS: In our case-control study, 30.1% of all pregnancies had likely exposure to potential EDCs. The most prevalent occupations conferring possible exposure were related to activities on farms. Maternal and paternal occupational exposure to potential EDCs significantly increased the risk of mild hypospadias than moderate-to-severe hypospadias (OR=6.55 vs OR=4.63). Among various categories, parental occupational exposure to pesticides was associated with at least a twofold increased risk of hypospadias. Maternal EDC exposure during the first trimester significantly increased the risk of bearing a hypospadiac child (OR=4.72 (95% CI 2.10 to 10.60)). CONCLUSION: This study suggests that EDCs are a risk factor for hypospadias through occupational exposure during fetal life.

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. MATERIALS AND METHODS: A hospital-based cross-sectional study was conducted on twenty children with EHPVO aged between 1 and 18 years over a period of 1 year. The children were evaluated clinically, followed by upper gastrointestinal endoscopy. Radiological assessment included imaging of the main portal vein, its right and left branches, splenic vein, and superior mesenteric vein using color Doppler ultrasonography (CDUSG) and magnetic resonance portovenogram (MRP). Evidence of portal biliopathy, status of collaterals, and possible sites for portosystemic shunt surgery were also examined. RESULTS: All the patients presented in chronic stage with portal cavernoma and only one patient (5%) had bland thrombus associated with cavernoma. The CDUSG and MRPs had a sensitivity of 66.6-90% and 96.7% and specificity of 91.5% and 98.3% respectively with regard to the assessment of the extent of thrombus formation and flow in the portal venous system. Both the modalities were found to be complementary to each other in preoperative assessment of EHPVO. However, the sensitivity of MRP was slightly superior to CDUSG in detecting occlusion and identifying portosystemic collaterals and dilated intrahepatic biliary radicals. CONCLUSION: Results of the present study indicate that MRP is well suited and superior to CDUSG in the preoperative imaging of patients 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. Psychological assessment was done by evaluating intelligence and temperament. RESULTS: Single-stage repair was performed in 38 children, and two underwent multistage repair. Major postoperative complications were noted in three individuals. The follow-up period ranged from 12 to 168 months, and during this time the growth velocity declined significantly among group II and group III patients when compared with control subjects. After adjusting the body mass index for age, our data revealed that group III participants had a significantly (P = 0.02) lower body mass index than the control group. Group III also had poor indices for intelligence quotient (P ≤ 0.01) and temperament (P ≤ 0.01). Female patients had lower temperament indices when compared with unaffected females with regard to approach withdrawal (P ≤ 0.01), mood (P = 0.026), and intensity (P = 0.03). Overall, increased disease severity adversely affected the psychological indices. CONCLUSION: Individuals with anterior encephalocele without associated intracranial defects had excellent postoperative outcomes in terms of growth and psychological developments. Hydrocephalus and agenesis of corpus callosum had the least impact on psychological development. However, the presence of secondary brain defects led to developmental delays. Gender differences in temperament may suggest a need for distinct treatment regimens to assess psychosocial well-being for males and females.

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. SETTINGS AND DESIGN: Hospital based observational study conducted over a period of year. MATERIALS AND METHODS: 38 cases of both sexes and below 12 years of age with spinal dysraphism were studied. USG was performed in 29 cases where acoustic window was available for proper evaluation. MRI was performed in all cases. USG findings were compared with MRI findings and operative follow up was taken in 23 cases who underwent operative management. STATISTICAL ANALYSIS USED: Results were analysed using percentage and arithmetic mean. RESULTS: 39.47 % cases were male and 60.53 % cases were female. Neonatal period was the most common presenting age group. Closed spinal dysraphism (63.16%) was more common than open (36.84%). 79.31% cases showed full agreement between spinal USG and MRI examinations and 6 out of 20.69% showed partial agreement. On operative correlation, USG findings were confirmatory in 91.30% cases and MRI findings were confirmatory in 100% cases. CONCLUSIONS: USG can be used as the initial modality for evaluation of spinal dysraphism as well as for screening of suspected cases. MRI is indicated to confirm abnormal USG findings, which shows all concurrent abnormalities and also provides additional anatomical details relevant to surgical planning.

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. Encephaloceles were described with respect to their types, contents, and extensions. A note was made on the associated malformations with sincipital encephaloceles. RESULTS: Fifty percent of the patients presented before the age of 3 years and both the sexes were affected equally. Nasofrontal encephalocele was the most common type seen in 13 patients (46.4%), and corpus callosal agenesis (12 patients) was the most common associated malformation. Other malformations noted were arachnoid cyst (10 patients), hydrocephalus (7 patients), and agyria-pachygyria complex (2 patients). CONCLUSION: Capital Brain malformations are frequently encountered in children with sincipital encephaloceles. Detail radiological evaluation is necessary to plan treatment and also to prognosticate such rare malformations.

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. On the other hand, the urorectal septum malformation sequence is a lethal congenital malformation which is characterized by the development of a phallus like structure, a smooth perineum and the absence of urethral, vaginal and anal openings. We are reporting a case of a female foetus with the prune belly syndrome, which was associated with a urorectal septum malformation sequence. A dead foetus with a protruded abdomen and ambiguous genitalia, was born at 32 weeks of pregnancy. On autopsy, it was found to have female internal genital organs. The left kidney, the urinary bladder and the rectum were absent. The sigmoid colon, the ureters and the fallopian tubes opened into a common cloacal sac. The histopathological examination of the ovary showed the presence of Leydig's cells. The occurrence of the female counterpart of the prune belly syndrome is extremely rare and only few of such cases were found to be discussed in the details in the indexed English literature so far. Hence, we hope that this case report will contribute to the existing knowledge on the prune belly syndrome.

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. Almost one-third of the cases have intrahepatic cyst, making complete cyst excision often impossible. We are reporting a 9-year-old girl with type IVa choledochal cyst presented to us with recurrent pain abdomen and intermittent jaundice for 1 year. Excision of extrahepatic cyst, left hepatectomy, mucosectomy of the residual cyst wall of right lobe of the liver and a wide bilioenteric anastomosis was done. Patients followed up with an magnetic resonance cholangiopancreatography (MRCP) 2 months later showed shrinkage of the residual cyst and good bile drainage. Mucosectomy of intrahepatic cyst may prevent recurrent cholangitis, calculus formation and cholangiocarcinoma in the long run.