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. MATERIALS AND METHODS: Thirty albino rats were divided into 3 groups of 10 each. After exploration under anesthesia, a vial of OK-432 diluted in 0.2 ml of normal saline was injected into the common bile duct (CBD) in rats of Groups B and C. In the control Group A, only saline was injected. Re-exploration was done at 3 weeks in Groups A and B and at 6 weeks in Group C, and freshly prepared ICG was injected into the inferior vena cava. Blood samples were collected at periodic intervals, optical density of the serum was measured, and half-life of ICG and fractional clearance (K) were calculated. Blood and tissue samples were obtained for biochemical tests and histological examination. RESULTS: The histological changes in CBD and liver were maximum in Group B; this correlated well with the K-value in this group, which was significantly delayed. In Group C, clearance was delayed than the control group with histological changes ranged from mild to moderate inflammation. The control group had normal histology of liver and CBD, and only four rats showed mild portal inflammation. CONCLUSION: ICG clearance rate is a reliable marker of liver function and can be utilized for evaluation of liver function in postoperative extrahepatic biliary atresia patients.

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. RESULTS: MTHFR 1298CC was significantly associated with AE risk (odds ratio [OR] 4.21; p = 0.01). The MTHFR haplotypes 677C-1298C/677T-1298A (OR, 2.50) and 677T-1298C (OR, 2.86) conferred risk in a progressive manner (χ2 = 9.82; p < 0.01). MTHFD1 1958G>A was not associated with disease susceptibility. Children with the rs2236225 GA and the rs1801131 CC genotypes were at an increased risk as compared to the reference genotype of rs2236225 GG and rs1801131 AA (OR, 14.4; p = 0.02). Children with the rs2236225 GG and rs1801133 CT genotypes were also at an elevated risk (OR, 4.76; p = 0.01). The MTHFD1 polymorphism together with the MTHFR haplotypes elevated risk in a progressive manner (χ2 = 6.29; p = 0.01). CONCLUSION: The data support our hypothesis of gene-gene interaction between MTHFD1 and MTHFR and the risk of AE. Together with the MTHFR haplotypes, MTHFD1 elevates risk in a progressive manner. The minor allelic frequencies of the MTHFD1 1958G>A and MTHFR 1298A>C in our populations were similar to those reported from Southeast Asian population, suggesting a possible explanation for the prevalence of this malformation in these regions. Birth Defects Research 109:432-444, 2017. © 2017 Wiley Periodicals, Inc.

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. We are reporting a case of unilateral big gluteal teratoma in a 6-year-old girl. The tumor did not have any connection with the sacrum or coccyx and was successfully removed. Histology showed well-differentiated bowel as well as fetiform structures.

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. The non-functioning lower moiety showed structure of xanthogranulomatous pyelonephritis.

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. Seventeen babies were preterm with an average weight of 1.92 kg. The time of presentation, gestational age, birth weight, position of the hemibladders and associated malformations were noted. Reconstruction procedures involved dismantling of the hemibladders and primary turn in, tubularization of the bowel with an end colostomy, and reconstruction of the abdominal wall. Results of the primary surgical repair, bowel function, and outcome of secondary procedures were analyzed. RESULTS: The position of hemibladders was lateral in 11, upper confluent in 4 and lower confluent in 8. Associated anomalies were noted in 19 patients. Four patients presented late (>5 days). Five died preoperatively, all had major associated anomalies. Four of them were preterm with average weight of 1.4 kg. Two patients refused surgery. Single-stage surgical reconstruction was done in 15 patients. Five patients died postoperatively because of associated anomalies, prematurity, and sepsis. One patient is waiting for surgery. Six patients had follow-up at 3-42 months and are awaiting further reconstruction. Four patients were lost to follow-up. CONCLUSIONS: Prematurity, late presentation, and sepsis are the major causes of high mortality noted in this series. In our experience, single-stage reconstruction without osteotomy gives satisfactory results.

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. PATIENTS AND METHODS: A total of 56 patients aged from 24 to 126 months were operated between January 2009 and August 2011. 32 patients had coronal, 9 had glanular and 15 had distal penile hypospadias. The meatus was located within 1 cm of the corona in all the patients. There was minimal or no chordae. All the patients underwent advancement of corpus spongiosum. RESULT: The mean operating time was 36 min and mean operating blood loss was 15 ml. In three patients, the corpus spongiosum was opened inadvertently during dissection. It was repaired with 5-0 vicryl and stitched to the cavernosal bed. An indwelling catheter was kept for 5 days. None of these three patients had any problem after removal of the catheter. Mean hospital stay was 0.5 days (0-6 days). 42 patients were discharged from the hospital on the day of surgery. Eight patients complained of dysuria on the first day. There were no other complications. The functional and cosmetic results have been excellent. The mean follow-up period was 16 months (8-38 months). CONCLUSION: Corpus spongiosum advancement is an easy technique, consumes less time and has excellent cosmetic and functional outcome. The procedure is most suitable for children with distal hypospadias without chordae.

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. PATIENTS AND METHODS: Five cases (male:female, 3:2) of type IV-A choledochal cyst, aged between 15 and 120 months, are presented. The intrahepatic cyst was confined to only the left lobe in 1 patient and the left and part of the right lobe in 4 patients. Excision of the extrahepatic cyst, left hemihepatectomy, mucosectomy of the residual intrahepatic cyst wall, and wide hepaticojejunostomy were done. RESULTS: Median hospital stay was 20 days. Postoperative biliary leak in 1 patient stopped spontaneously after 3 weeks. Postoperative MRCP (magnetic resonance cholangiopancreatography) scan in 1 patient showed shrinkage of intrahepatic residual cyst and good biliary drainage. Liver function tests at 3 months, 6 months, and at yearly intervals were within reference range in all patients. Cholangitis, hepatolithiasis, or malignancy was not noted in any of the patients. Median follow-up period was 36 months. CONCLUSION: Excision of the diseased mucosa from residual intrahepatic cyst wall may prevent long-term complications in patients with 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. RESULTS: The overall incidence of congenital malformation was 0.08%. This was considerably lower than similar published data from other states. Five hundred and eleven babies were born with congenital malformations, with 421 (82.4%) having major malformations. Males were affected more than females, 334 (65.4%) vs. 177 (34.6%). The gastrointestinal and genitourinary systems accounted for 26% and 25.8%, respectively. Malformation involving the central nervous system was more common in certain ethnic groups. CONCLUSIONS: The incidence of malformations in certain systems was at variance with the data from other states.

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. 45 patients with rectosigmoid HD admitted between August, 2004 and July, 2008 were included in this study. Pre-operative barium enema was done in all of them. Patients in whom the transition zone was well delineated were included in the study. Frozen section biopsy was done to confirm the presence of ganglion cells in the pull-through bowel. All odd number patients were included in group A, where conventional TEPT procedure was performed. All even number patients and the last five patients of the series were in group B. In this group, a modified transanal endorectal procedure was done. The initial part of submucosal dissection and mobilization of aganglionic rectum was the same as in the conventional procedure. The anorectal mucosa was anastomosed end-to-side to the seromuscular coat of the ganglionic bowel. Mobilized bowel was divided at about 2 cm distal to the anastomosis. Six hitching stitches were applied from the free edge of the bowel to the perianal skin so that the anastomosis remains outside the anal verge. The distal redundant bowel was excised on eighth post-operative day. Pre-operative bowel preparation was done in all patients in group A. The bowel preparation was not mandatory in group B patients. Post-operative results of both these groups were compared and analyzed using the Mann-Whitney U test. Mean operative time in group A was 86 min (range 75-95 min) versus 90 min (range 70-100 min) in group B. Average length of the bowel resected was 20 cm (range 10-26 cm) in group A and 22 cm (12-40 cm) in group B. Post-operative feeding was started after 48 h in group A and after 6 h in group B. Average hospital stay was about 8 days in both the groups. Median follow-up was 36 months in group A (range 14-54 months) and 32 months (range 12-52 months) in group B. Anastomotic stricture or stenosis was noted in two patients in group A and none in group B (p < 0.01). Partial anastomotic dehiscence was noted in two patients in group A and none in group B (p < 0.01). Average bowel movement in group A was 6-8 per day at 1 month and 2-6 per day at 6 months. In group B, it was 8-10 at 1 month and 2-8 at 6 months. Cuff narrowing causing constipation was noted in two patients in group A and none in group B (p < 0.01). Enterocolitis following pull-through was noted in two patients in group A and three patients in group B. Four patients in group A and one patient in group B had post-operative constipation. All of them responded to bowel management program: only one of them (group A) required enema; soiling was noted in one patient each in both the groups. Modified TEPT procedure is associated with excellent clinical outcome with fewer complications, and permits early post-operative feeding. Operating time, hospital stay and post-operative bowel movement were comparable with the conventional technique.