Conventional and unconventional surgical modalities for choledochal cyst: long-term follow-up

Background This study presents our experience of various modes of presentation, surgical methods applied (both conventional and unconventional), and their outcome on patients with choledochal cyst. Method This is a retrospective study in which files of all patients with choledochal cyst over a period of 10 years (1997–2007) were studied. Data collected from files included age, sex, modes of presentation, various surgical techniques, outcome, and follow-up. Results The total number of patients was 35. The most common mode of presentation was recurrent abdominal pain in 20 (57%) patients. It was followed by jaundice in 16 (45%) patients, fever, nausea, and vomiting in 10 (28.5%) patients, and other symptoms. Roux-en-Y hepaticojejunostomy was performed in 26 (74%) patients, hepaticoduodenostomy in five (14%) patients, external T-tube drainage in three (8%) patients, and cystoduodenostomy in two (5%) patients. Two patients expired in this series. The follow-up loss was 20%. Three patients who suffered from cholangitis were managed conservatively. No patient has shown cirrhosis and cholangiocarcinoma till now. Conclusion Choledochal cyst is an important entity in an Indian setup. The presentation has a wide spectrum; therefore, different types of surgical intervention would play a significant role in various situations.Keywords: choledochal cyst, follow-up of choledochal cyst, surgical technique

Congenital anterior urethral valves and diverticula: Diagnosis and management in six cases

Background: Anterior urethral valves (AUVs) are rare congenital anomalies causing lower urinary tract obstruction in children. Although they are referred to as valves, these obstructive structures often occur in the form of a diverticulum. The urethra in these cases shows saccular or bulbar dilatation known as anterior urethral diverticulum (AUD). They typically occur where there is a defect in the corpus spongiosum, leaving a thin-walled urethra. This segment of the urethra balloons out during voiding, simulating a mass that is sometimes visible along the ventral wall of the penis. The swelling is fluctuant and urine dribbles from the meatus on compression. The present study highlights the clinical approach in identifying the condition and its treatment options, especially for those, presenting with urethral diverticula. Materials and Methods: We have studied children with congenital anterior urethral valves and diverticula. Six patients of AUVs with diverticula were admitted during the period of 2000-2007 and were prospectively evaluated. The mean age of presentation was 16 months (15 days to 4 years). Straining at micturition and a palpable penile swelling were the most common presenting features. The diagnosis was established by voiding cystourethrogram (VCUG) and supported by ultrasonography (USG). All patients were treated with single-stage open surgical excision except one who died preoperatively due to urosepsis. Initial lay opening of the penoscrotal urethra and delayed repair were done in one patient. Results: The surgical outcome was successful in all but one patient, who died of delayed presentation with severe back pressure changes, urinary ascitis and urosepsis. On long-term follow-up, all patients demonstrated good stream of urine. The renal functions were normal and the patients had no evidence of urinary infections. Conclusion: We propose that, the patients of AUVs, if not excessively delayed for treatment are otherwise well in terms of general condition as opposed to patients of posterior urethral valves. The diagnosis is easily established by VCUG and the severity is revealed by a sonogram. Open surgical excision is the method of choice for patients with a urethral diverticulum; however, cystoscopic fulguration is also feasible in selected patients. The outcome is excellent with minimal morbidity and mortality

Retrospective analysis of paediatric achalasia in India: Single centre experience

Background: Developing countries at tertiary referral centre. The aim of this study was to share our experience of paediatric achalasia in Indian scenario.Materials and Methods: This was a retrospective analysis of children <16 years, operated for achalasia at our centre, from December 1998 to December 2011. Results: Total 40 patients (mean age 39 ± 4.29 months), including 1 patient of megaesophagus were operated over 13 years of period; 17 patients (associated congenital H-type tracheoesophageal fistula in one patient, non- responders/ lost follow-up for minimum of 3 years in 16 patients) were excluded from the study. The response rate of parents in followup was 60.0%. Mean symptoms duration was 27.88 ± 2 months. Most common symptoms were regurgitation and failure to thrive (78.2%). Mean symptom scoring in follow-up after 3 year was 1 ± 0.7 compared to 5 ± 0.51 at the time of admission (P < 0.012). One infant expired (mediastenitis), one developed adhesive intestinal obstruction and one needed posterior re-myotomy (for megaesophagus). There were no treatment failures in mean follow-up of 40.2 ± 5.07 months. Conclusions: Cardiomyotomy with partial fundoplication is the best modality of treatment for paediatric achalasia cardia, even from parents’ perspective.Key words: Cardiomyotomy, fundoplication, pediatric achalasia cardi

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

<b>Objective:</b> 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. <b> Materials and Methods:</b> A prospective analysis of 127 cases of EA from February 2004 to May 2006 was performed. Waterston prognostic criteria were used for grading. <b> Results:</b> EA with TEF was the commonest type in 117 cases (92&#x0025;). Associated congenital anomalies were present in 52 (41&#x0025;) patients, the commonest being the cardiac anomalies, which was followed by the gastrointestinal anomalies. VACTERL was found in 6 (5&#x0025;) cases. Prematurity, associated congenital anomalies, gap between esophageal ends and preoperative respiratory status were the significant factors affecting the survival (<i> P</i> = &lt; 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&#x0025;). 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&#x0025; in group A, 83&#x0025; in group B and 22&#x0025; in group C. <b> Conclusion </b> 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

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

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