Rapunzel Syndrome in a 3-Year-Old Boy: A Menace too Early to Present.

Rapunzel syndrome is a rare type of trichobezoar with an extension of the hair into the small bowel. Clinical presentation is deceptive and vague, ranging from asymptomatic abdominal mass to gastrointestinal perforation. There are only few cases reported in literature, with the youngest age being 3 years. We present the case of a 3-year-old male child presenting with Rapunzel syndrome and features of subacute intestinal obstruction.

Hirschsprung's disease in a child with posterior urethral valve: An unwanted association.

Urinary bladder and the rectum share a common embryological origin, and the anatomical proximity of these two organs suggest that a dysfunction in either may influence the function of the other. Although, the coexistence of bladder and bowel dysfunction has been previously reported in the literature, there are hardly any reports on coexistence of posterior urethral valve (PUV) with Hirschsprung's disease. Here, we report a case of a 20-month-old male child who was initially treated for PUV and was later found to have coexisting Hirschsprung's disease.

Esophageal anastomosis medial to preserved azygos vein in esophageal atresia with tracheoesophageal fistula: restoration of normal mediastinal anatomy.

OBJECTIVE: We intended to prospectively study the technical feasibility and advantages of esophageal anastomosis medial to the preserved azygos vein in neonates diagnosed with esophageal atresia with tracheoesophageal fistula (EA/TEF). The results were compared to the cases where azygos vein was either not preserved, or the anastomosis was done lateral to the arch of preserved azygos vein. MATERIAL AND METHODS: A total of 134 patients with EA/TEF were admitted between January 2007 and July 2008 of which 116 underwent primary repair. Eleven patients with long gap esophageal atresia with or without tracheoesophageal fistula and 7 patients who expired before surgery were excluded. Patients were randomly divided in three groups comparable with respect to the gestational age, age at presentation, sex, birth weight, associated anomalies and the gap between the pouches after mobilization: Group A (azygos vein ligated and divided), Group B (azygos vein preserved with esophageal anastomosis lateral to the vein), and Group C azygos vein preserved with esophageal anastomosis medial to the vein). All the patients were operated by extra-pleural approach. The three groups were compared with respect to operative time and early postoperative complications like pneumonitis, anastomotic leaks and mortality. Odds ratio and Chi square test were used for the statistical analysis. RESULTS: Group A, B and C had 35, 43 and 38 patients respectively. No significant difference was observed in average operative time in the 3 groups. Though incidence of postoperative pneumonitis was higher in group A (28%) as compared to group B (13.95%) and group C (11.62%), it was not statistically significant (p > 0.005). Anastomotic leak occurred in 7 patients in group A (20%), 6 patients in group B (13.95%) and 4 patients (10.52%) in group C (p > 0.005). Group A had 3 major and 4 minor anastomotic leaks; group B had 2 major and 4 minor leaks and group C had 1 major and 3 minor leaks. There were10 deaths in the series- 5 in group A, 3 in group B and 2 in group C (p > 0.005). Patients with major anastomotic leaks in all 3 groups expired after re-exploration. The minor leaks were managed conservatively and all of them healed spontaneously. Severe pneumonitis and septicemia in patients having major associated anomalies also contributed to the mortality. CONCLUSIONS: Although esophageal anastomosis medial to the preserved azygos vein restores the normal mediastinal anatomy without technical difficulty or increased operative time, the study could not prove a statistically significant advantage in terms of mortality and postoperative complications.

Emphysematous epididymo-orchitis: A rare entity.

Emphysematous epididymo-orchitis is a rare cause of an acute scrotum and is a surgical emergency. Diagnosis is clinically difficult, and sonography with a high-frequency probe is useful to pick up gas shadows in the scrotal wall or testicular substance. A diabetic patient presented with fever, urinary tract infection, and an acute scrotal swelling. The patient needed orchidectomy and scrotal debridement. As in emphysematous pyelonephritis, this condition occurs in diabetics, and patients may need surgery. There is a need to perform sonography in all diabetic patients with an acutely inflamed scrotum, because detection of gas shadows makes surgical intervention more likely.

Study of pelvic floor and sphincter muscles in congenital pouch colon with the help of three-dimensional CT scan.

INTRODUCTION: Congenital pouch colon (CPC) is a pouch-like dilatation of shortened colon associated with anorectal malformation (ARM). The disease is prevalent in northern India. Postoperatively, the continence results are not as good as in other ARMs and there is higher incidence of incontinence and perineal soiling in these patients. The present study aimed to evaluate the pelvic floor and sphincter muscle characteristics in patients of CPC with the help of 64-slice computerized tomography with three-dimensional (3D) volumetric reconstructions of images, thus, to know the overall quality of these muscles in the patients. MATERIALS AND METHODS: The study was conducted in patients admitted over a period of July 2007 to November 2008 in our department. Totally, eight patients of CPC were subjected to 64-slice CT with three-dimensional reconstructions of images and different parameters such as quality of pelvic floor muscles, configuration of vertical and parasagittal fibres, shape and thickness of sphincter muscle complex, attenuation values of sphincters were studied. RESULTS: The 3D reconstructed images of pelvis in patients of CPC showed a well-developed pelvic floor and sphincter muscle complex. The length of the parasagittal fibres, transverse width of the vertical fibres and CT attenuation values of these structures with overall muscle quality were found to be good in these patients. CONCLUSION: In cases of CPC, the pelvic floor muscles including striated muscle complex (vertical and parasagittal fibres) are well developed. Higher rates of incontinence and soiling in CPC are not because of poorly developed pelvic floor and sphincter muscles. Three-dimensional CT can also provide important anatomical information that can help the operating surgeon while performing surgery.

Neonatal pneumoperitoneum: a critical appraisal of its causes and subsequent management from a developing country.

BACKGROUND: The diagnosis and management of neonatal pneumoperitoneum revolves around necrotizing enterocolitis (NEC) in most of the published literature. Although NEC remains the major cause of pneumoperitoneum in a neonate, there are several other causes leading to free air in the peritoneal cavity. A number of case reports have appeared describing pneumoperitoneum in a newborn due to rupture of one particular organ, but there have been only few collective reviews on the subject. The present study shares the experience with neonates admitted with a diagnosis of pneumoperitoneum in a pediatric surgical center of a developing country. The various causes of pneumoperitoneum in a newborn, their management and subsequent outcome are described. MATERIALS AND METHODS: The study was conducted in the Department of Pediatric Surgery, CSMMU (upgraded King Georges Medical College), Lucknow, India. All the neonates admitted with a diagnosis of pneumoperitoneum during the period of last 3 years (2005-2008) were retrospectively analyzed. Other neonatal admissions were also retrieved for the same period. Free air was confirmed by erect abdominal X-ray or lateral decubitus films in certain cases. The data sheets were analyzed regarding age of presentation, cause of bowel perforation, management offered and subsequent outcome achieved. All patients of NEC without evidence of perforation were not included in the study (n = 21). RESULTS: Out of total 537 neonatal admissions, 89 (16.5%) neonates were admitted with a diagnosis of pneumoperitoneum. There were 79 (88.7%) males and only 10 (11.6%) female neonates admitted during the study period. All of them had evidence of pneumoperitoneum at the time of admission. The age at presentation ranged from 4 to 32 days. NEC remained the single major cause of pneumoperitoneum in the newborn; however, in 44 (49.4%) patients the cause was not related to NEC. Perforated pouch colon, isolated colonic perforations, caecal perforations, gastric and duodenal perforations were the main causes of pneumoperitoneum not related to NEC. There were seven patients in whom no cause of pneumoperitoneum could be ascertained. The treatment was individualized according to the presentation. Most of the NEC-related perforations were managed by peritoneal drains. Laparotomy was done in rest of the patients. Three patients were managed conservatively. Overall, 19 (21.6%) patients expired. Most of those expired were of low birth weight with NEC and congenital pouch colon with perforation. CONCLUSION: Neonatal pneumoperitoneum remains a surgical emergency and outcome can be lethal if the problem is not addressed early. NEC remains the major cause; however, there are several other important causes of isolated gastrointestinal perforations leading to neonatal pneumoperitoneum. The management should be individualized in these patients and the outcome largely depends on the early recognition of the condition.

Neonatal incarcerated inguinal hernia with spontaneous scroto-fecal fistula.

This report describes the presentation of an incarcerated inguinal hernia in a neonate with the formation of a spontaneous scroto-fecal fistula. Delay in seeking medical attention in the present case was the cause of incarceration and formation of the spontaneous fecal fistula. We advocate that development of an inguinal hernia in an infant should be managed as an urgent problem even if it is easily reducible at presentation. There should be an early repair policy for all pediatric hernias, especially the neonatal ones.

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.

Traumatic diaphragmatic injuries in children: do they really mark the severity of injury? Our experience.

PURPOSE: Diaphragmatic injuries have been reported to be a predictor of serious associated injuries in trauma and a marker of severity. Because of its rarity in children, the diagnosis is often delayed for months and years, due to overshadowing injuries. Perhaps due to the elasticity of their tissues, traumatic diaphragmatic rupture is uncommon in children. The problem remains a challenging clinical entity and the description of such type of injuries in children remains scarce in the literature. Most of the cases are described along with associated injuries; presence of isolated diaphragmatic injuries in children is unusual. The present study highlights the presentation, diagnosis and management of all of the cases admitted with traumatic diaphragmatic injuries in a single pediatric surgical center. METHODS: We retrospectively studied eight children admitted to our center with a diagnosis of diaphragmatic injury following trauma during a period of 5 years (2003-2008). Relevant information regarding the mode and pattern of injuries were noted in all cases. Type of injury and surgical intervention and outcome of patients were evaluated. RESULTS: Mean age of presentation was 6.8 years (range 2-12 years). Seven patients were males, while one patient was female. Seven patients had a history of blunt trauma abdomen some time back. Only one patient had acute presentation with respiratory distress following road traffic accident, rest of the patients had no associated grievous injury at the time of presentation. One patient presented with features of acute obstruction. All patients could be diagnosed preoperatively and surgical intervention was performed in all cases. The patients recovered well and there was no mortality. CONCLUSION: Diaphragmatic injuries in children are rare. They are usually associated with other severe injuries; however, isolated diaphragmatic injuries occur more frequently in children than adults. A high index of clinical suspicion supported by prompt radiological tests is needed to diagnose these injuries in patients who otherwise have no associated grievous injuries.

Presentation of high ano-rectal malformation beyond neonatal period.

Anorectal malformation is one among the common congenital malformations usually encountered. The babies usually present early after birth with complaints of missing anal opening, or abnormally placed anal opening, but some patients have presented beyond the early newborn period without recognition of their anorectal malformation. We are presenting a case of a male child with high ARM, who unusually presented first at the age of 45 days, without gross abdominal distension or septicaemia.

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.