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.

Anterior intratumoural chemotherapy: a newer modality of treatment in advanced solid tumours in children.

OBJECTIVE: Advanced and inoperable solid tumours in children have high mortality despite aggressive multimodal treatment. Intravenous chemotherapy is abandoned at times because of systemic toxicity. This study investigated intratumoural chemotherapy and compared it with intravenous chemotherapy. METHODS: Forty children with advanced inoperable solid tumours (Wilms' tumour and neuroblastoma) were randomly allocated into two groups of 20. Group A was given intratumoural chemotherapy and group B was given intravenous chemotherapy. Both groups were compared for reduction in tumour size and volume, tumour resectability, histopathological changes and drug side effects. RESULTS: Intratumoural chemotherapy was superior to intravenous chemotherapy in terms of reducing tumour size and volume (63% in group A vs. 22% in group B). The resectability was 70% in the intratumoural group compared with 40% in the intravenous group. The overall good histopathological response was 71% in group A as opposed to 0% in group B. Moreover, the incidence and severity of drug side effects and morbidity were less with intratumoural chemotherapy. Mortality was also low in group A (5%) compared to group B (20%). CONCLUSION: Intratumoural chemotherapy can be offered as an effective and safe alternative treatment modality for advanced and inoperable Wilms' tumour and neuroblastoma.

Is ligation of azygos vein necessary in primary repair of tracheoesophageal fistula with esophageal atresia?

INTRODUCTION: Congenital tracheoesophageal fistula with esophageal atresia (TEF with EA) is not an uncommon disease of newborns. Classical approach for primary repair of TEF with EA is right thoracotomy with extrapleural approach, ligation of the azygos vein, identification and ligation of tracheoesophageal fistula, identification of upper esophageal pouch and end-to-end anastomosis. This study was conducted to evaluate if the ligation of the azygos vein is a must during primary repair of TEF with EA. METHOD: We studied 50 randomly selected cases in the last two years (Jan 2003 - Jan 2005). In 25 babies (group A), primary repair was done with preservation of the azygous vein while 25 babies (group B) were operated with a classical approach with ligation and division of the azygos vein. The postoperative result was comparative in both cases. RESULT: Postoperative pneumonitis was higher in the babies operated with the classical approach (56 % in group B and 12 % in group A) and was found to be statistically significant. In postoperative complication, anastomotic leak was slightly higher in group B but was not statistically significant. Mortality rate was similar in both groups. Babies in group A were hemodynamically more stable in comparison to group B. CONCLUSION: Preservation of the azygos vein maintains the normal venous drainage of mediastinum and hence decreases the postoperative chest congestion and pneumonitis in the postoperative period in cases of congenital esophageal atresia with tracheoesophageal fistula, so it should be preserved whenever possible.

Novel therapy for insulin-dependent diabetes mellitus: infusion of in vitro-generated insulin-secreting cells.

Insulin-dependent diabetes mellitus (IDDM) is a metabolic disease usually resulting from autoimmune-mediated ß-cell destruction requiring lifetime exogenous insulin replacement. Mesenchymal stem cells (MSC) hold promising therapy. We present our experience of treating IDDM with co-infusion of in vitro autologous adipose tissue-derived MSC-differentiated insulin-secreting cells (ISC) with hematopoietic stem cells (HSC). This was an Institutional Review Board approved prospective non-randomized open-labeled clinical trial after informed consent from ten patients. ISC were differentiated from autologous adipose tissue-derived MSC and were infused with bone marrow-derived HSC in portal, thymic circulation by mini-laparotomy and in subcutaneous circulation. Patients were monitored for blood sugar levels, serum C-peptide levels, glycosylated hemoglobin (Hb1Ac) and glutamic acid decarboxylase (GAD) antibodies. Insulin administration was made on sliding scale with an objective of maintaining FBS < 150 mg/dL and PPBS around 200 mg/dL. Mean 3.34 mL cell inoculums with 5.25 × 10(4) cells/µL were infused. No untoward effects were observed. Over a mean follow-up of 31.71 months, mean serum C-peptide of 0.22 ng/mL before infusion had sustained rise of 0.92 ng/mL with decreased exogenous insulin requirement from 63.9 international units (IU)/day to 38.6 IU/day. Improvement in mean Hb1Ac was observed from 10.99 to 6.72%. Mean GAD antibodies were positive in all patients with mean of 331.10 IU/mL, which decreased to mean of 123 IU/mL. Co-infusion of autologous ISC with HSC represents a viable novel therapeutic option for IDDM.

Urinary mucoprotein in pediatric urolithiasis.

Primary bladder stone is a common pediatric surgical problem in developing countries. Many theories are prevalent. The stone matrix theory is based on increased excretion of its precursor, the uromucoid (the urinary mucoprotein). Uromucoid, studied in urine and stones by the electroimmunodiffusion technique in 49 cases with controls, showed significantly increased excretion in stone cases. Family income and serum protein were the only important influencing factors (negative). Causal relationship between uromucoid excretion, stone matrix, and pediatric bladder stones is discussed. Methylene blue decreased uromucoid excretion but not magnesium oxide, vitamin C or B6 (four common therapeutic drugs for prevention/dissolution of urolithiasis).

A unique presentation of atypical complete duplication of terminal ileum, colon, rectum, and urinary bladder.

A unique case of complete duplication of urinary bladder, distal ileum, cecum, appendix, colon, and rectum with two mesocolons and separate vascular arcades is being reported.

Abdominal cocoon in children: a report of four cases.

Abdominal cocoon is a rare cause of intestinal obstruction. The authors report four cases (3 boys, 1 girl; age range, 6 to 8 years) that presented with features of intestinal obstruction. There was no history of previous surgery, peritonitis, or prolonged drug intake in any of these cases. One patient presented with acute intestinal obstruction and gangrene of bowel. The etiology, preoperative diagnosis, and management of this condition are discussed.

Role of steroid in childhood haemangioma: a 10 years review.

1195 cases of various type of haemangioma were treated with various steroid modalities. The response to intralesional steroids was excellent in strawberry and mixed haemangiomas. The response to oral steroids was also good in these two types. The response of cavernous variety was poor with either modality used alone but with combined modality, a moderate response can be obtained.

Estimation of tissue DNA content in Wilm's tumour.

DNA genome, is affected by malignant processes. Estimation of DNA content was carried out biochemically using Gile's and Myer's technique to assess its prognostic value in 21 cases of Wilm's tumour. The DNA content of tumour tissue and normal adjoining renal tissue was estimated in each case. The DNA content was significantly higher in Wilm's tumour tissue as compared to normal renal tissue (p < 0.05). As the disease advanced, the DNA content of the tumour further increased. The tumours having unfavourable histology had significantly higher DNA content as compared to favourable histology cases (p < 0.001). Similarly, the patients who died because of the disease, had a very high DNA content as compared to those who survived. Further, it was seen that two fold increase in DNA content indicated advanced stage (III & IV), whereas a three fold or more increase indicated grave prognosis as it was usually seen in advanced stage with unfavourable histology and such cases died early. Thus, the DNA content estimation has a prognostic significance in Wilm's tumour.

Local steroid therapy in cutaneous hemangiomas.

OBJECTIVES: To evaluate the efficacy of intralesional triamcinolone in enhancing regression in various type of hemangiomas. SETTING: Outpatients department of a teaching hospital Subjects: One hundred and five patients with rapidly growing surface hemangioma. The age ranged from 1 month to 15 months (mean 7 months). INTERVENTION: Intralesional triamcinolone administered at monthly intervals, ranging from single injection to 7 injections (mean 3.6 injections). MAIN OUTCOME MEASURE: Regression of the hemangioma. RESULTS: The overall response rate was 88.6% (excellent 51.4%, and good 37.2%). Maximum response was observed in children below 1 year of age (> 90%), lesions over the face (92.8%) and strawberry hemangiomas (99.9%). CONCLUSION: Intralesional administration of triamcinolone devoid of systemic side effects and an effective initial modality for rapidly growing hermangiomas.

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.

Simultaneous occurrence of jejuno-jejunal and ileo-ileal intussusception in a child: a rare occurrence.

Intussusception is the most common cause of intestinal obstruction in infants and children. This condition is frequent in children and presents with the classic triad of cramping abdominal pain, bloody diarrhoea and a palpable tender mass. Small bowel intussusceptions are much less common, with jejuno-ileal and duodeno-jejunal intussusceptions being the rarest types of all. Multiple simultaneous intussusception is a peculiar variety of intussusception. The authors report the simultaneous occurrence of jejuno-jejunal and ileo-ileal intussusception in a patient. As this is an extremely uncommon entity, it is being reported with a brief review of the relevant literature.