Type V Congenital Pouch Colon: An Extremely Rare Variant of Anorectal Malformations.

Type V is the rarest form of congenital pouch colon with only four cases reported till date. We report this anomaly in a 6-month-old boy. He was managed successfully with excision of distal pouch and coloplasty of proximal pouch along with abdominoperineal posterior sagittal anorectoplasty. We recommend preservation of proximal pouch in such cases.

Undescended testicle: An update on fertility in cryptorchid men.

Cryptorchidism or undescended testis is one of the most common anomalies encountered in paediatric urology and is estimated to affect 1 to 4 per cent of full term and upto 30 per cent of preterm male neonates. The associated problems of sub-fertility or infertility and malignant transformation have been recognized for long. Fertility is impaired after both unilateral and bilateral cryptorchidism. The reported paternity rates in adults are about two-third for unilateral undescended testis and less than one-third for bilateral disease. Over the last five decades, the concepts related to cryptorchidism have changed dramatically as knowledge about its effects has accrued from research conducted worldwide. The recommended age of orchidopexy has fallen progressively from adolescence to less than one year. The realization that the infantile testes are not in a state of 'suspended animation' and the recognition of the defect in the androgen dependent transformation of gonocytes into adult dark spermatogonia in cryptorchidism have been recognized as the primary cause of sub-fertility in these patients. This has paved the way for hormone therapy in an attempt to simulate the 'post-natal gonadotropin surge' or 'mini-puberty'. This review summarizes the current knowledge about the various factors affecting the fertility status in cryptorchidism with a particular focus on the derangements in the development and maturation of the germ cells and the role of surgery, hormone therapy and antioxidants in reversing these changes.

Percutaneous trans-hepatic gelfoam-cyanoacrylate glue embolization for ruptured post-traumatic hepatic artery pseudo-aneurysm in a limited-resource scenario.

INTRODUCTION: We present our experience with an indigenously designed percutaneous trans-hepatic ultrasound-guided Gelfoam sponge cum cyanoacrylate glue-based embolization technique for the treatment of a ruptured post-traumatic aneurysm of a branch of the right hepatic artery (RHA) as a 'life-saving emergent' procedure in a patient unfit for surgery or endovascular intervention and in a 'limited-resource' scenario (non-availability of Digital Subtraction Angiography Suite). CASE DETAILS: An 8-year-old boy sustained crush-injury to the right lobe of the liver in a road-traffic accident and presented in shock. After resuscitation, a laparotomy and repair of the right lobe of liver were undertaken. Bleeding restarted 1 week after the surgery; the patient bled from drain site and went into shock. Exploration was not advisable in view of poor general condition, and sepsis, deranged coagulation and parental reluctance in view of guarded prognosis. Multi-detector Computed Tomography Angiography was performed after resuscitation which revealed active bleed from a ruptured pseudo-aneurysm of a branch of RHA. TECHNIQUE: The bleeding artery was identified with duplex sonography and was embolized by the percutaneous trans-hepatic route proximal to the site of pseudo-aneurysm and rupture by a two-step process. Initially, a thin paste/'slurry' made of powdered gelfoam dissolved in sterile saline was injected into the bleeding vessel. Subsequently, the area was sealed by injecting 1.0 ml of N-butyl-2-cyanoacrylate glue. Hemostasis was confirmed by Color and Power Doppler Ultrasonography both post-procedure and after 48 h. With supportive management, the patient showed a rapid recovery and was discharged after 2 weeks. He continues to be well at 3-month follow-up. CONCLUSION: The technique was effective in controlling hemostasis and life-saving in our set-up.

Evaluation of nebulized N-acetyl cysteine in outcome of esophageal atresia with tracheoesophegeal fistula.

AIM: To evaluate the role of nebulized N-acetyl cysteine (NAC) in liquefying the airway secretions and improving the outcome of patients of esophageal atresia with tracheoesophageal fistula (EA + TEF). METHODS: It was a non-randomized interventional study. Two milliliters of 10% NAC was given in a nebulized form (2:5 dilution, every six hourly) to patients of ET + TEF, along with regular suction of upper esophageal pouch. The group was compared with control, which comprised patients of EA + TEF receiving only saline nebulization. The consistency of the secretions was compared by hand held consistometer in unit of time (seconds) required to cross a predetermined distance along with gravity. RESULTS: Sixty patients were assessed. Of these, 30 patients were present in both groups. The study group showed significant (p = 0.01-0.0001) decrease in consistency of secretions from the control group after day 2 of NAC nebulization. Patients' discharge was significantly (p = 0.01) earlier in cases. There was no significant (p = 0.41) difference in mortality between the groups. No specific adverse effects were observed in the study group. CONCLUSION: It appears that nebulized NAC decreases the consistency of secretions in EA + TEF patients. It is interesting to note that the group of patients that received NAC was discharged earlier than the control group and had a higher survival rate than the control group. Whether this is directly attributable to the use of NAC is unknown. A prospective double-blinded randomized clinical trial is warranted to confirm these results. LEVEL OF EVIDENCE: Level II, prospective comparative study (non-randomized).

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.

Congenital pouch colon: Our experience with coloplasty.

BACKGROUND: Congenital pouch colon (CPC) or congenital short colon is an entity found mainly in Indian subcontinent. In CPC, colon is replaced with partially or completely abnormal pouch connected to the genitourinary tract by a fistula (colovesical). Management protocol is different in different institute. In this article, we are sharing our three stage standard management approach; principle and technique of coloplasty in cases in which colon length is not adequate for pull through and their follow-up. This study aims to show the result of coloplasty in complete CPC. MATERIALS AND METHODS: This is retrospective observational study, of 5 years duration. The medical record of these patients was reviewed for demographic information, clinical features, investigations performed, operative notes, post-operative events and the outcome of surgery. RESULTS: Total of 626 ARM cases were managed in 5 years duration in which 64 were of pouch colon. The age of presentation was 1-15 days. In fifty patients who completed their, all stage in that 34 patients were in which coloplasty were done in rest of 16 cases excision of CPC and colonic pull through done in view of adequate colonic length (type III and IV) for pull through. In 34 patient in which coloplasty were done showed satisfactory cosmetic and functional out came after stoma closer in follow-up. CONCLUSIONS: Properly created coloplasty and three stage procedure for complete pouch colon give better result and less complications. Excision of pouch is not requiring in all cases of CPC.

Two Cases of Massively Prolapsed Patent Vitellointestinal Duct.

Patent vitellointestinal duct (PVID) is a benign congrnital anomaly ususally presenting with fecal discharge from the umbilicus. In this report, we describe two cases of PVID presented with massive bowel prolapse through the PVID and signs of intestinal obstruction. Surgery revealed prolapse of the ileal intussusceptum through the PVID. Both of the babies were sucssesfully managed with surgery.

Double gastric outlet obstruction with epidermolysis bullosa in a neonate.

We report a 2-day-old baby who had double gastric outlet obstruction, rectovaginal fistula and associated epidermolysis bullosa. She recovered after excision of diaphragms, pyloroplasty and pelvic colostomy.

Delayed presentation of strangulated congenital diaphragmatic hernia: learning from our experience.

BACKGROUND: Strangulation is very rare in congenital diaphragmatic hernia (CDH) of the Bochdaleck variety. Here, we share our experience with six cases of delayed presentation of strangulated CDH. The aim of this article is to provide information on how to diagnose and manage this situation using a systematic approach. MATERIALS AND METHODS: A retrospective review identified six cases of strangulated/obstructed CDH from 1998 to 2011. Demographic data, clinico-radiological findings, management and complications, along with final outcome were recorded. RESULTS: Small bowel gangrene was found in one patient, gastric perforation in three, transverse colon perforation in one and colonic obstruction in one patient. Video-assisted thoracoscopic surgery (VATS) was used in all but one patients for definitive diagnosis, diaphragmatic repair, pleural lavage and management of empyema. Laparotomy was needed for management of strangulated or perforated bowel. Three patients in this study survived. CONCLUSION: Clinicians should always consider a diagnosis of obstructed Bochdaleck hernia in children, because X-ray findings are not always typical or even normal in complicated CDH. VATS may be considered as both diagnostic and therapeutic. Preventive measures for empyema or early intervention in the evolving stage can significantly reduce morbidity.

Congenital large maxillary teratoma.

Teratoma of the maxilla is a rare entity. Congenital intraoral teratoma occurs in 1:4000 births. It is a benign tumor, although malignancy has been described in adults. A 10-year-old male child with this condition is described in this report. This case illustrates a huge mass on the right side of the maxilla. The mass was excised under general anesthesia. Histopathologically, it consisted of all three layers of embryonic elements with predominantly fibrous tissue. Postoperative result was uneventful and no recurrence was detected after 2 years.

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.

Prolapse of bowel via patent vitello intestinal duct--a rare occurrence.

This report describes the presentation of prolapse of small bowel through the patent omphalomesenteric or vitello intestinal duct in a child. In spite of diagnosing the anomaly earlier, there was delay in surgical intervention that led to prolapse of the small bowel through patent remnants, which was life threatening. The patient presented to us with questionable viability of prolapsed bowel. Early surgery is recommended for this entity. As this is a very rare occurrence, it is being reported with a brief review of the relevant literature.

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.

Azygos vein preservation in primary repair of esophageal atresia with tracheoesophageal fistula.

The aim of this study is to report a series of patients with the Azygos vein preserved during the surgery for esophageal atresia with tracheoesophageal fistula (EA&TEF), highlighting the advantages in terms of survival and prevention of anastomotic leak. Ninety-six neonates with EA&TEF, admitted to the Department of Pediatric Surgery, King George Medical University between 2004 and 2006, were reviewed prospectively; the babies were randomly allocated to two groups: Group A (n = 46) in which the Azygos vein was preserved and Group B (n = 50), wherein it was ligated. The two groups were comparable in respect to sex, weight, prematurity, associated anomalies, Waterston classification, Spitz classification and distance between the pouches after mobilization. Anastomotic leak occurred in three cases (6%) in Group A and ten cases (20%) in Group B and was responsible for mortality in one (2%) case in Group A and six cases (12%) in Group B. Preservation of Azygos vein resulted in significant reduction in the number of anastomotic leaks. We propose that preservation of the Azygos vein prevents early postoperative edema of the esophageal anastomosis by maintaining the venous drainage and thus may form an additional protective factor against anastomotic leaks.