Single-stage treatment of spina bifida with hydrocephalus based on a prediction rule derived from preoperative cranial ultrasound.

AIMS: It was the aim of this study to report a prospective study of 110 spina bifida patients with hydrocephalus treated by simultaneous ventriculoperitoneal (VP) shunt and repair of the meningomyelocele. METHODS: Between January 2005 and February 2008, 264 patients with spina bifida were admitted to the author's department. Those patients in whom the preoperative cranial ultrasonography measurement of the bifrontal diameter was >26 mm, the bicaudate diameter >20 mm and the diameter of the body of the lateral ventricle >26 mm were predicted to develop postoperative hydrocephalus (n = 245). Of these, 110 patients underwent simultaneous (group 1) VP shunt with repair of the meningomyelocele, while 135 (group 2) underwent sequential surgery due to the preference of the attending consultant. RESULTS: Twenty-two of the 110 patients in group 1 developed complications (wound problems, cerebrospinal fluid leak, shunt malfunction, death) compared with 38 of 135 patients in group 2. The remaining patients had an uneventful postoperative and follow-up course. CONCLUSIONS: Simultaneous VP shunt with surgery for hydrocephalus was feasible in our setup. The rate of complications was not higher than in staged surgery. Single-stage repair of spina bifida with hydrocephalus offers considerable advantage in the form of a reduction in hospital burden, costs and patient morbidity.

Modified method of primary esophageal anastomosis with improved outcome in cases of esophageal atresia with tracheoesophageal fistula.

Survival rates for infants who have esophageal atresia (EA) with or without fistula (TEF) have improved dramatically in the past 50 years. Despite excellent long-term survival for patients with esophageal atresia with tracheoesophageal fistula (EA-TEF), many significant complications can occur. Anastomotic leak at the esophagoesophagostomy site is one such problem resulting in considerable morbidity and mortality in these patients. The methods of esophageal anastomosis for long period has remained the simple end to end anastomosis of esophageal ends with various modifications described from time to time. The present study aims to study the effect on the early postoperative complications, following horizontal mattress suture technique on the primary esophageal anastomosis in cases of EA-TEF. A total of 32 patients with EA-TEF, were operated by our technique during a period of 1 year (2007-2008). The results were compared with the patients (n = 66), who were operated by the traditional simple technique during the same period. Among those patients in whom the esophageal anastomosis was done by horizontal mattress suture, only one had major anastomotic leak, while two had minor anastomotic leaks, as compared to six and nine cases correspondingly in other patients in whom anastomosis was done by simple technique. There was single mortality. We propose that, the utilization of our technique of horizontal mattress suture in primary anastomosis of esophagus in cases of EA-TEF significantly reduces the risk of anastomotic leaks and subsequent morbidity and mortality.

Anorectal junction stenosis: diagnosis and management.

PURPOSE: The aim of this paper is to describe the management of 9 patients with anorectal junction stenosis and present the diagnostic features together with a single-stage surgical technique with reproducible results. MATERIALS AND METHODS: Nine patients with anorectal junction stenosis were seen over a period of 12 years. The children (aged from 2 months to 15 years) presented with constipation . Anorectal junction stenosis was diagnosed by rectal examination during which the tip of the finger was unable to pass beyond the stenosis in all cases. Contrast study showed the dilated rectosigmoid proximal to the stenosis. RESULTS: Six patients (who did not have significant rectal dilation) underwent single-stage surgery by posterior Y-V plasty which was curative. Two patients were operated through the posterior sagittal route with a covering colostomy done during the same session; the oldest child required resection of the megasigmoid with abdominoperineal pull-through. None of the six patients operated with Y-V plasty experienced any complications. One of the patients operated via the posterior sagittal route had a leak from the anorectal anastomosis requiring revision. The follow-up ranged from 6 months to 12 years. All patients are alive and well and there was no recurrence of stenosis in any case. CONCLUSION: Anorectal junction stenosis is a rare anorectal anomaly easily diagnosed by digital rectal examination. Single-stage surgery by posterior Y-V plasty is effective in curing the majority of these patients if significant rectosigmoid dilation is not present.

Delayed presentation of anorectal malformations.

AIMS AND OBJECTIVES: Delayed management of anorectal malformation (ARM) increases the surgical and functional complications for the patient. We defined "delayed presentation of ARM" and reviewed our patients with ARM to find out the incidence and causes of delayed presentation. MATERIALS AND METHODS: Patients satisfying the criteria of "delayed presentation of ARM" were involved. Detailed information of each patient including the mode of presentation, associated anomalies, plan of management and follow-up was obtained from the hospital records. RESULTS: Between 2003 and 2006, 43 patients satisfied our criteria of "delayed presentation of ARM". There were 21 males and 22 females. Seventeen of these males presented with low-type ARM. Eleven of them were managed by a single-stage procedure. These "delayed presenters" had to live with constipation, inadequate weight gain and parental anxiety for a greater time. Analysis of the outcomes showed more functional complications in patients who had undergone failed perineal surgery previously. In females with low ARM, the procedure of choice was anterior sagittal anorectoplasty (ASARP). Single stage surgery provides good outcomes for most of low type of ARMs. High-type ARMs in males and females were managed by a staged procedure. CONCLUSION: "Delayed presentation of ARM" is a major group of ARM in our setup. The management and results of their treatment are not different from those of the early presenters. The most common cause of delayed ARM is wrong advice given by the health care providers followed by inadequate treatment elsewhere. Corrective surgeries taking second attempt in perineum always produces poor outcomes.

Congenital duodenal obstruction with situs inversus totalis: Report of a rare association and discussion.

This report is to present and discuss an extremely rare association of situs inversus with duodenal atresia in an 11-day-old male neonate born full term and weighing 1.9 kg. The baby presented with recurrent bilious vomiting. Babygram revealed situs inversus and duodenal obstruction. Echocardiography showed dextrocardia with a small ASD. Exploration confirmed a duodenal diaphragm with a central perforation between the third and fourth part of the duodenum and situs inversus. The literature search revealed 20 cases reported so far.

Esophageal atresia: Factors influencing survival - Experience at an Indian tertiary centre.

OBJECTIVE: To study the clinical profile of the cases of esophageal atresia (EA) and/or tracheoesophageal fistula (TEF) and various factors affecting the surgical and early postoperative management and their outcome. MATERIALS AND METHODS: A prospective analysis of 127 cases of EA from February 2004 to May 2006 was performed. Waterston prognostic criteria were used for grading. RESULTS: EA with TEF was the commonest type in 117 cases (92%). Associated congenital anomalies were present in 52 (41%) patients, the commonest being the cardiac anomalies, which was followed by the gastrointestinal anomalies. VACTERL was found in 6 (5%) cases. Prematurity, associated congenital anomalies, gap between esophageal ends and preoperative respiratory status were the significant factors affecting the survival (P = < 0.001). Primary extrapleural repair was the surgical approach in most of the patients. Azygos vein was preserved in 46 cases and no retropleural drainage was used in 27 cases. Staged procedures were performed in 19 cases, including 6 cases of isolated esophageal atresia. Pneumonitis and sepsis were the most common early postoperative complications (42%). Hypoxia and cardiorespiratory arrest were the most common causes of mortality (11 cases). Anastomotic leak complicated 13 cases, including 9 major and 4 minor leaks. Major leak followed by sepsis caused 7 deaths. Survival as per Waterston criteria was 100% in group A, 83% in group B and 22% in group C. CONCLUSION: Factors affecting the survival are major or life-threatening associated anomalies, long gap, pneumonia and sepsis at presentation or that acquired during hospitalization and major leaks. The high incidence of low birth weight, delayed diagnosis, poor referral, low-socio economic status and lack of advanced neonatological back up are important contributory factors to poor outcome.

Evaluation of congenital diaphragmatic hernia in a tertiary health center of a developing country: management and survival.

AIM: The purpose was to evaluate the diagnosis and efficacy of management of congenital diaphragmatic hernia (CDH) in a tertiary health center of a developing country. METHODS: Forty-six children aged from 1 day to 7 years were studied. Parameters studied were age, sex, clinical features, and management. RESULTS: Fifty-six percent of patients presented in the neonatal period; however, none of them presented on the first day of life. The majority (91.3%) of patients had left-sided CDH. Respiratory distress was the most common clinical feature observed (91.3%). Chest X-ray confirmed the diagnosis in 82.6% of patients, and contrast study was needed in the remaining 17.4%. The survival rate was 87%. It was better in patients presenting late than those presenting in the early neonatal period. Stabilization in the preoperative period improved survival. Not using a chest tube had no adverse effect on survival. CONCLUSION: The relatively increased survival rate of CDH in a tertiary health center of a developing country is attributed to delayed arrival to the center. Respiratory infections compound the survival. More studies are needed before it can be safely said that not using a chest tube has no adverse outcome. Late presentation has been associated with varied manifestations, hence proper clinical evaluation, a high index of suspicion and adequate management, which includes imaging and surgery after stabilization, gives excellent results.

Hepatic calcification following dengue virus-induced fulminant hepatic failure.

Hepatic calcification can be seen with various infectious and neoplastic conditions. We report a 32-year- old man who developed massive calcification in the right lobe of liver following recovery from dengue virus-associated fulminant liver failure.

Comparison of three solutions for total gut irrigation in pediatric patients.

Total gut irrigation (TGI) is a safe method of bowel preparation in children. Many solutions are used but none is ideal. This study has been done to compare household common salt solution prepared by dissolving 10 g of pure NaCl salt (Active Reagent Quality) in 1 l of tap water, polyethylene glycol with electrolytes (PEG) and ringers lactate for TGI in children. We prospectively evaluated the three solutions in terms of efficacy, safety, rapidity, tolerability and cost effectiveness in patients undergoing a variety of colorectal procedures. Patients (126) were randomly assigned into one of the three groups; Group I, household common salt solution, 40; Group II, Peglec, 55; Group III, Ringer lactate, 31. TGI with PEG is the most rapid method of bowel preparation but is least tolerable. Household common salt solution is inexpensive and most tolerable of the three preparations. All three are similar in safety and effectiveness in bowel preparation. Household common salt solution is effective, safe, cost effective and the most tolerable method of bowel preparation.

Retroperitoneal teratomas in children.

OBJECTIVE: This paper aims to highlight the clinical features, investigations and treatment of retroperitoneal teratomas condition. METHODS: 12 patients (8 females and 4 males, age range-2 months to 14 yrs) of retroperitoneal teratoma admitted to the department of Pediatric Surgery, King George Medical University, Lucknow between 1980 and 2004 were studied. Investigations included hematology, plain X-ray of the abdomen, intravenous urography, ultrasound, computerised tomography (CT) of the abdomen (after 1990, 8 patients) and serum alpha-fetoprotein assay (after 1991, 6 patients, preoperatively). All patients underwent surgery. Serum alpha-fetoprotein assay was used during follow-up to detect recurrence. RESULTS: Majority of the tumors were left pararenal in location. In two patients there was bilateral involvement. In all except one, the tumor could be excised easily preserving the kidneys. In one child with a massive cystic tumor with bilateral involvement, the tumor was marsupialised in the first stage and excised subsequently. One child died postoperatively, the other 11 children are well and there has been no tumor recurrence on follow-up. CONCLUSIONS: Retroperitoneal teratomas are uncommon lesions in children mostly arising in close relation to the kidneys. The majority are benign but complete excision is necessary for cure. Even large tumors with bilateral involvement of the retroperitoneum can be excised while preserving adjacent organs. Serum alpha-fetoprotein assay is a reliable method of detecting recurrence.

Non surgical management of cystic lymphangioma.

UNLABELLED: Aim/Purpuse: To evaluate our experience of 19 patients of lymphangioma who were treated by intralesional Bleomycin. MATERIALS AND METHODS: Nineteen patients of lymphangioma aged between 16 days to 11 years were managed in the department. The male-female ratio was of 2:1. Commonest sites were in the neck (58%) followed by axilla (21%). The patients were treated by intralesional bleomycin injection. Bleomycin was given at a dose not exceeding 0.5 unit/kg/dose at interval of 2 weeks. Reduction in size of the mass was noted in between 2 weeks to 16 weeks and number of injections required for each patient varied from 1 to 6. Follow up ranged from 1- 7 Year. RESULT: In injection group, significant reduction of mass was noted in 84% (n=l6) and 57% (n=11) of them showed complete disappearance. No serious complications were noted in any patient. CONCLUSION: Our experience showed that Bleomycin in aqueous solution is a good sclcrosing agent in the management of lymphangioma.

Patients with gallstones develop gallbladder cancer at an earlier age.

Significant proportions of Indian patients with gallbladder cancer are young. Multiple risk factors for gallbladder cancer are recognized among Indian patients. The effect of these risk factors on the age of development of gallbladder cancer is not known. We conducted a study to determine the influence of risk factors on the age at diagnosis of patients with gallbladder cancer and to assess the interactions between these risk factors. Patients with gallbladder cancer from two tertiary care institutions during the period 1994-2001 were prospectively studied. An ultrasound examination was done to look for the presence of gallstones. The influence of gender, gallstones, socio-economic status, smoking, residence in rural areas and in the Gangetic belt on the age at presentation was analysed using univariate analysis, logistic and linear regression analyses. The mean age of the 121 patients studied was 55+/-11.7 (SD) years. There were 51 (42%) patients aged less than 50 years. The younger patients (age < or =50 years) were more likely to have gallstones (88 versus 66%; P=0.008) and to have come from a lower socio-economic background (88 versus 71%; P=0.02) in comparison with older patients. However, there was no effect of the other risk factors. The independent determinants for younger age of patients with gallbladder cancer on logistic regression analysis were gallstones [odds ratio (OR) 4, 95% confidence interval (CI) 1.5-11; P=0.006] and lower socio-economic status (OR 3.1, 95% CI 1.1-8.6; P=0.03). On linear regression analysis, age at presentation was lowered by 5.6 years if there were associated gallstones. The mean age of patients with these two risk factors was significantly lower than that of those with one risk factor or no risk factor (52+/-12 years versus 57+/-11 years versus 61+/-9 years; P=0.007). In conclusion, the presence of gallstones and lower socio-economic status were both independently associated with a younger age at diagnosis of gallbladder cancer and their effect was additive.

Do the progesterone receptors have a role to play in gallbladder cancer?

BACKGROUND: Gallstone disease as well as gallbladder cancer are more common in women and female sex hormones may be involved in their etiology. AIM AND METHODS: To determine whether female sex hormones have a role in the pathogenesis, of gallbladder carcinoma and in its prognosis, we estimated, by enzyme immunoassay, the estrogen and progesterone receptors (ER and PgR) in the gallbladders of 21 patients with gallbladder cancer, 19 patients with cholelithiasis, and 6 patients who underwent incidental removal of essentially normal gallbladder as a component of wider resection. RESULTS: ER were present in the gallbladder mucosa in all the three groups in proportions which were not significantly different (9/21 in carcinoma, 4/19 in gallstones, and 1/6 normal), whereas the expression of PgR was greater in carcinomas (13/18), less in cholelithiasis (4/12), and absent in normal gallbladders. PgR expression was higher in tumors of lower stage (7/7) and lower in advanced disease stage IV tumors (6/11). PgR expression was associated with better disease stage (p=0.05) and significantly longer overall survival (median survival of 301 d vs 54 d) as well as better survival within the same stage (269 d vs 54 d for stage IV disease, p=0.011). Cox's regression analysis showed that PgR was an independent risk factor (R=0.2283, p=0.0035). CONCLUSIONS: Our findings suggest that the female sex hormones may have a role in the pathogenesis of gallbladder cancer and that PgR expression has a prognostic significance. We believe that when this relationship is reaffirmed by larger studies, gallbladder cancer may be treated with appropriate sex hormonal manipulation.

A pancreaticographic study of malnutrition-related diabetes mellitus.

Pancreatic involvement is considered to be the hallmark of malnutrition-related diabetes mellitus (MRDM). Of the 2 subgroups of the disease, fibrocalculous pancreatic diabetes (FCPD) is characterized by pancreatic calcification. The nature of pancreatic abnormalities in MRDM have not been studied extensively in Indian patients. The present study was designed to compare pancreatic abnormalities (exocrine and endocrine) including endoscopic retrograde pancreaticography in patients with FCPD and protein deficient pancreatic diabetes (PDPD), in relation to controls. Ten patients each of FCPD and PDPD were studied with regard to clinical features, biochemical exocrine and endocrine pancreatic responses, C-peptide response, islet cell antibody, and pancreatographic changes. Five normal pancreatograms were taken as control. Clinical and biochemical features in patient with FCPD and PDPD were as follows: pain in 8 and 2 patients, respectively; the mean duration of diabetes was similar in both groups (62.28 +/- 71.92 months V. 72 +/- 50.9 months); and faecal fat excretion and insulin requirements were comparable in both groups. The main pancreatic duct was dilated in 6 of 10 patient with FCPD and only 1 of 10 with PDPD on ultrasonography. On pancreatography the duct was dilated in 9 of 10 patients with FCPD and only 1 of 10 patients with PDPD. The number of side branches was reduced in all cases with MRDM; in those with FCPD, these were stunted and dilated while in PDPD side branches are thin and spastic. We conclude that pancreatic ductal changes involving the main duct and side branches are more frequent in patients with FCPD as compared to those with PDPD.

Colonoscopic barotrauma treated by conservative management: role of high-flow oxygen inhalation.

Most patients with colonoscopy-induced colonic perforation require urgent surgical intervention. Certain patients may, however, recover with conservative management. If the colonic perforation has been induced by barotrauma, there may be an extravasation of air which may lead to collection and tracking of air along the tissue planes, involving the retroperitoneum, peritoneal cavity, mediastinum, pleura, pericardium, or even the scrotum. The conservative treatment of colonic perforation includes bowel rest and antibiotics. Absorption of air collected in different body cavities may take some time, and inhalation of high-concentration oxygen may enhance the rate of gaseous absorption. We describe here a patient with colonic perforation induced by barotrauma, who recovered fully on conservative management with inhalation of high-concentration oxygen. We recommend that this approach should be used more frequently in such a situation.

Sacrococcygeal teratoma.

This retrospective study details our experience regarding 72 patients with sacrococcygeal teratoma treated over a period of 17 years. The sex incidence was nearly equal, but there was a high proportion of Altmann type IV tumors. A preliminary colostomy before combined abdominosacral excision of large type III and IV lesions reduced morbidity. Sixty-six percent of the patients presented beyond the neonatal period; 14 had been treated elsewhere for bowel/urinary obstruction. Imaging studies included radiography, abdominal ultrasound, computed tomography, and magnetic resonance imaging (after 1995). In 60 patients the tumor was excised via the sacral route, 11 had a preliminary colostomy, and 1 had a vesicostomy. Eight children (5 with malignant lesions) required abdominosacral excision. After 1990, serial estimation of serum alpha-fetoprotein (AFP) was used to monitor tumor recurrence. There were 34 male and 38 female patients (age range 3 days-12 years); 47 had benign tumors, of which 42 were excised through the sacral route. Three patients underwent a preliminary colostomy and abdominosacral excision of the tumor with subsequent colostomy closure. There were 4 deaths in this group; no recurrence was seen in the surviving children with benign tumors. Twenty-five patients had malignant teratomas. In 18 of these the tumor was excised via the sacral route and 5 underwent abdominal-sacral excision. Eight had a preliminary colostomy and chemotherapy followed by excision of the residual tumor and colostomy closure. None of the initial 14 patients with malignant lesions survived beyond 2 years. Of the latter 11 (who received cisplatinum-based chemotherapy), 10 were alive 1 year after surgery. One patient is currently on preoperative chemotherapy and another developed recurrence of the tumor. The overall follow-up ranged from 3 months to 8 years; there has been no complaint of functional neurological deficit in any of the patients. As intrapelvic tumors tend to have a delayed diagnosis, this can be avoided by performing a rectal examination. There should be no recurrence after excision of a benign teratoma. Cisplatinum-based chemotherapy has improved the survival of patients with malignant tumors.

Fatigue in cholestatic liver disease--a perplexing symptom.

Fatigue is an important symptom and a quality of life determinant in patients with cholestatic liver disease. The pathogenesis of fatigue is obscure, although alterations in central neurotransmission and peripheral muscle dysfunction have been incriminated. No effective treatment is available at present. The available literature on fatigue in cholestatic liver disease is reviewed.

A pancreatography study of chronic calcific pancreatitis of the tropics.

Chronic Calcific Pancreatitis of Tropics is a disease of unknown aetiology and is characterised by chronic pancreatitis with calcification in young persons who present with pain, diabetes, and/or steatorrhoea. ERCP performed on 42 patients with this condition revealed changes compatible with chronic pancreatitis. These changes were however, more marked and somewhat different from those seen in the alcoholic chronic pancreatitis. Cystic dilatation, tortuosity, and obstruction of the main pancreatic duct were similar to that in alcoholic pancreatitis. The features of CCPT that were different from those of latter, were large pancreatic calculi, absence of strictures/stenosis and absence of irregularity of the ductal wall. The calculi were predominantly in the head region of the pancreas causing maximal dilatation of the main pancreatic duct in the head of pancreas. The secondary branches were stunted, short and scanty but revealed a lower grade of changes, than the changes documented in the main pancreatic duct. The pancreatic ductal changes in CCPT seems to be different from that seen in chronic alcoholic pancreatitis and may be due to the difference in the pathophysiology of the underlying disease.

Incidence, spectrum and antibiotic sensitivity pattern of bacterial infections among patients with acute pancreatitis.

BACKGROUND AND AIM: Secondary infection of pancreatic necrotic tissue and peripancreatic fluid is a serious complication of acute pancreatitis resulting in significant morbidity and mortality. The aim of this study was to find out the spectrum of bacterial infections, and their antibiotic sensitivity pattern in patients with acute pancreatitis. METHODS: All consecutive patients with acute pancreatitis were studied prospectively. Detailed investigations were carried out to identify bacterial infections and their antibiotic sensitivities in patients with suspected infection. These investigations included cultures of various body fluids, throat swabs, indwelling cannula and catheter tips. Pancreatic tissue was obtained by using needle aspiration or at surgery for Gram's stain, culture and sensitivity. All cultures were repeated until the presence of infection was confirmed or excluded. RESULTS: A total of 169 patients with acute pancreatitis were studied during the period between January 1997 and June 2000 (mean age 41.3 years; 116 males and 53 females). Of the 169 patients, 63 had infections at various sites. A total of 80 cultures were positive, and 12 different bacterial isolates were cultured from samples taken from these 63 patients. Polymicrobial infection was seen in 32% of patients. Twenty-four patients had a confirmed pancreatic infection. Blood cultures had a growth of organisms in 19 patients, with evidence of ongoing or worsening pancreatitis, thus raising a strong suspicion of infected necrosis in them. The commonest organisms were Escherichia coli from 20 cultures and Pseudomonas aeruginosa from 18 cultures. The antibiotic sensitivity pattern showed that most bacteria were sensitive to third generation cephalosporins and quinolones; notably among them were cefotaxime, ceftazidime, and ciprofloxacin. CONCLUSION: Bacterial infections were seen in 37% of patients with acute pancreatitis. The commonest organisms were Pseudomonas aeruginosa and Escherichia coli. Most bacterial isolates were sensitive to third generation cephalosporins and quinolones.

Clinical and biochemical comparative study of different types of common bile duct stones.

BACKGROUND: The chemical composition of common bile duct (CBD) stones may have significance with regard to the origin, clinical presentation and treatment. OBJECTIVE: To study the chemical composition of CBD stones and compare different types of CBD stones. METHODS: Consecutive patients with CBD stones seen over a 1-year period were studied prospectively. Their stones were analyzed for cholesterol, bilirubin and calcium contents. RESULTS: Of 74 patients with CBD stones, 42 had associated gall bladder (GB) stones (Group 1), 14 had post-cholecystectomy CBD stones (Group 2) and 18 had CBD stones with a stoneless GB in situ (Group 3). Of the 40 patients whose CBD stones were analyzed, 34 (85%; 18/19 in Group 1, 5/6 in Group 2 and 11/15 in Group 3) had cholesterol stones; the remaining 6 patients had pigment stones. CONCLUSION: Most CBD stones in northern Indian patients are cholesterol stones. Even in patients with isolated CBD stones (with stoneless GB in situ), three-quarters are cholesterol stones.