Congenital Colonic Stenosis: A Rare Gastrointestinal Malformation in Children.

AIMS: Congenital colonic stenosis (CCS) is an extremely rare cause of low-intestinal obstruction in neonates/child. We report our experience with seven cases of CCS presenting with low-intestinal obstruction and diagnosed intraoperatively and also propose an algorithm for its appropriate treatment for the adequate outcome. MATERIALS AND METHODS: It was a retrospective study of seven patients of CCS including two neonates (5-days and 15-days old), four infants (age range - 2-11 months), and one 24-month-old child admitted from 2014 to 2019. Information regarding the age of presentation, clinical presentation, physical findings, radiological and laboratory findings, details of surgery, and outcome was retrieved and analyzed. RESULTS: The male-to-female ratio was 5:2. Patients were initially diagnosed as cases of Hirschsprung's disease in five and ileal atresia in two. A final diagnosis of CCS was made during surgery and histopathological examination of resected stenotic segment. The segment involved was ascending colon in three, transverse colon in two, and sigmoid colon and junction of descending and sigmoid colon each in one patient. Resection of stenotic colonic segment and primary end-to-end anastomosis was performed in two, divided stoma after resection of the stenotic segment and secondary anastomosis in three, and proximal loop terminal ileostomy followed by resection of the stenotic colonic segment and ileocolic anastomosis after 10-12 weeks in two. CONCLUSIONS: CCS is a rare but possible cause of large-bowel obstruction, in neonatal, infant, and children particularly when associated with a history of chronic constipation since birth. It should be kept in mind as a differential diagnosis while managing a case of neonatal and pediatric intestinal obstruction, particularly low-bowel obstruction along with a history of chronic constipation. Treatment should be individualized for each patient based on clinical status and associated anomalies to give the best results with less morbidity.

Angiotensin-converting Enzyme Inhibitors: Can it be a Potential Treatment of Infantile Hemangioma.

AIMS: The aim of the sudy was to evaluate potential role of oral captopril, an angiotensin-converting enzyme (ACE) inhibitor, and in treatment of infantile hemagioma (IH) and report our preliminary results. METHODS: This prospective study included 18 children with IH admitted in the department of pediatric surgery with no history of prior treatment of any type. Baseline blood pressure (BP), electrocardiogram, two-dimensional echocardiography, serum electrolytes, and renal function test (RFT) were noted. Oral captopril was started as first-line drug at a dose of 0.1 mg/kg orally 12 h with gradually increase of dosage up to 2.0 mg/kg 12 h over the period of 10 days with monitoring of BP, serum electrolytes, RFT, and occurrence of any side effect. If no side effects were noted and patients were stable, they were discharged and followed up until 6 months after stopping treatment. During follow-up, response to treatment was documented clinically and photographically. Development of any side effect was also noted. RESULTS: Excellent response to captopril was noticed in nine patients over 16-18 months. Four patients showed good response. Oral propranolol had to be administered alternatively in one patient showing fair response during the initial 4 months but no response afterward and in four patients showing no response at all. One patient developed an allergic reaction to propranolol and was started oral corticosteroid. These five patients had near complete resolution of lesion for the next 8-10 months. CONCLUSIONS: ACE inhibitors might have a role, though slow, in the involution of IHs. Therefore, these may have the potential to emerge as an alternative treatment for IH in future after confirmation with randomized studies with propranolol.

Bracka Urethroplasty with Buccal Mucosa Graft: Ergonomic Management of Penile Skin Dartos in the First Stage to Facilitate Second-stage Neourethral Coverage.

AIMS: The aim of the study was to report a new technique of ergonomic penile skin-dartos management during buccal mucosa graft (BMG) to provide adequate penile skin-dartos for neourethral coverage at the time of second-stage tubularization. MATERIALS AND METHODS: Ten proximal hypospadias with severe chordee underwent first-stage surgery with a new technique. An incision along the urethral plate margin and preputial edge was used to split inner prepuce off preputial dartos and penile degloving leaving inner prepuce attached to corona. Urethral plate was divided into the subfascial plane. Penile dartos was bisected in the dorsal midline. Distal half of penile skin-dartos bifurcated and joined to inner preputial edges. Mobilized and lateralized penile skin-dartos was sutured flanking edges of BMG. The second-stage tubularization after 6 months provided neourethral double dartos coverage with eccentric suture lines. RESULTS: Adequate dartos for neourethral coverage during second-stage tubularization was available in all. Subcoronal urethrocutaneous fistula occurred in one that was repaired. CONCLUSIONS: Ergonomic management of inner-preputial skin and ventral transfer of penile skin-dartos helps in providing neourethral coverage during subsequent second-stage tubularization to minimize the occurrence of complications.

Supernumerary Kidney Associated With Horseshoe Malformation and Ureteric Stricture With Hydroureteronephrosis - A Rare Case.

Supernumerary kidney is an accessory kidney with its own vasculature, collecting-system and encapsulated parenchyma with about 100 cases reported in the literature. However, there is no report of supernumerary kidney associated with horseshoe malformation with ureteric stricture. We report a rare case of 20 months old female admitted with left-sided abdomen lump and mild abdominal pain. During surgery, supernumerary kidney with horseshoe component with grossly-dilated left-sided pelvicalyceal system and proximal 1-cm of left-ureter, distal to which whole of left-ureter was noncanalized, was seen. Right-ureter was normal. Distal dilated part of left-ureter was anastomosed to bladder-dome after excision of strictured segment of left-ureter with placement of nephrostomy.

Evaluation of Effect of Propranolol on Serum Vascular Endothelial Growth Factor and Tissue Inhibitor of Metalloproteinase-2 Levels in Infantile Hemangioma.

BACKGROUND: Infantile hemangioma is the most common tumor of infancy. Currently, propranolol is a preferred drug for treating hemangioma. The exact mechanism of action of propranolol is not known. In this study, we attempted to assess whether propranolol has any effect on vascular endothelial growth factor (VEGF) and tissue inhibitor of metalloproteinase-2 (TIMP-2) over a period of time, and if it is there, how long it affects it. MATERIALS AND METHODS: Propranolol was administered in the dosage of 2-3 mg/kg. The first serum sample was collected before starting the propranolol treatment. Thereafter, samples were collected at monthly intervals up to a total of six samples. The samples were assessed for TIMP-2 and VEGF using enzyme-linked immunosorbent assay kit. RESULTS: The duration of this study was from June 2016 to November 2017. The total number of patients in this study was 15. Thirteen patients responded to treatment. The mean age of patients was 7.1 months. The mean value of baseline VEGF was 0.234 ± 0.059 and that of TIMP-2 was 1.338 ± 0.679. As compared to baseline value, the P value was statistically not significant in any of sequential values. In category-wise analysis, apart from statistically significant value in the 6th month in excellent category and good response category in the 1st month, all other values did not reveal any significant change in VEGF analysis. The analysis of TIMP-2 revealed a significant change in the levels from Sample 2 to Sample 6 in the excellent response group; however, the levels did not show a specific trend either increasing or decreasing. CONCLUSION: Despite its beneficial action in regression of hemangioma, the exact mechanism is yet to be identified. The exact duration of treatment needs further evaluation.

A Novel Technique for Prevention of Gastroesophageal Reflux in Staged Repair of Long Gap Esophageal Atresia with Tracheoesophageal Fistula.

AIM: The objective of the study is to report a novel technique of preventing gastroesophageal reflux and air leak from fistula to stomach in patients of tracheoesophageal fistula with long gap atresia, to buy time for the staged procedure. METHODS: Seven patients of tracheoesophageal fistula with upper pouch of esophagus at 2nd thoracic vertebra were selected for the staged procedure. Weight ranged from 1.7 to 1.8 kg. During the 1st stage surgery for gastrostomy, midline strip of linea alba attached to xiphoid process was harvested and slinged around the gastroesophageal junction, along with right cervical esophagostomy. After radio-nuclear scan, the demonstration of abolition of gastroesophageal reflux, gastrostomy feed was started. The 2nd stage surgery performed after 6 weeks, included mobilization of esophagostomy, release of sling, thoracotomy, and tension-free esophageal anastomosis. Outcome measurement includes (1) prevention of air leak from esophagus into the stomach, (2) abolition of gastroesophageal reflux, (3) ability to start gastrostomy feeds, and (4) reversal of occlusion after release of the sling. RESULTS: The placement of linea alba sling and elevation of gastroesophageal junction, abolished air leak from fistula to stomach in all. Radio nuclear scan demonstrated abolition of gastroesophageal reflux in 6 with weight gain after gastrostomy feeding. One patient expired due to sepsis. One patient underwent final repair with reversal of occlusion with release of the sling. CONCLUSION: Using a sling of the linea alba around the cardioesophageal junction, prevents gastroesophageal reflux and escape of air from esophagus into the stomach, gives time to improve the respiratory and nutritional status of the patient, for a subsequent safer delayed primary anastomosis.

Giant Occipital Encephalocele - Challenges in Management.

INTRODUCTION: Giant occipital encephalocele (GOE) is a term used when the size of the OE is greater than or equal to the size of the head. It has been limited to case reports, with only sporadic exclusive series. This is a series of GOE managed at our center over time with emphasis on practical problems faced in management. MATERIALS AND METHODS: This was a retrospective observational study. The patients were evaluated for the age of presentation, sex, and head size. Any associated neural tube defect was also looked for. Imaging was used for associated brain anomalies and to plan the surgical procedure. The requirement of ventriculoperitoneal (VP) shunt was also assessed. RESULTS: During the study period of 7 years, 11 patients of GOE were admitted. Apart from one, all other patients were <1 year of age. Nine patients underwent surgical intervention, which included excision and repair of swelling with or without VP shunt placement. The content of the sac was only cerebrospinal fluid (CSF) in six patients and CSF and gliotic brain tissue in remaining patients. The attendants of two patients did not give consent for surgery and left against medical advice. CONCLUSION: GOE is an uncommon entity with limited information about management. Careful evaluation, proper imaging of patient, and care during intraoperative and postoperative periods with emphasis of factors determining the prognosis may provide satisfactory results.

A Pilot Study on Histopathology of the Jejunoileal Atresia-Can it Be Used as a Guide to Determine the Length of Adequate Resection?

INTRODUCTION: Some studies reported that there is abnormality in the histopathology of atretic bowel in jejunoileal atresia (JIA). We have made an attempt to assess sequential histopathologic changes in the resected atretic segment. MATERIAL AND METHODS: The histopathology of the resected segment was evaluated at 1, 3, 5, 7, 9, and 11 cm from atretic end (Sections A to F, respectively). The ratio of inner and outer muscle layer (measured by NIS-Element D software) was calculated at every section. Immunohistochemistry for α-smooth muscle actin (α-SMA) was also done. The findings were compared with control. RESULTS: In control set (n = 5), the ratio of inner and outer muscle layer was 1.03. In patients with JIA, the ratio was 0.68 to 0.9 at section A. This ratio varied at various sections in all specimens. In section F, this ratio was 0.95 to 1.09, which is close to control ratio. There were no specific findings related to α-SMA staining. CONCLUSIONS: It appears that the bowel proximal to the atresia is abnormal for a varied length. It may be a possibility that this abnormality is present at least up to about 10 cm proximal to atresia. Adequate resection is important for optimal outcome.

Management Strategy of Meconium Ileus-Outcome Analysis.

BACKGROUND: Meconium ileus (MI) is defined as an intestinal obstruction caused by the impaction of inspissated meconium in the terminal ileum. In this study, we have evaluated the nonoperative management of patients of simple MI without fluoroscopic support -an important requisite of the Noblett's criteria. Besides this, surgical management in cases of failed conservative management and complicated MI was also assessed. MATERIALS AND METHODS: This was a retrospective observational study. Various clinical and radiological parameters were evaluated. Conservative management included the use of water-soluble contrast diatrizoate meglumine and diatrizoate sodium. In case of nonpassage of meconium in 24 h from first intervention, exploratory laparotomy with ileostomy was performed. All complicated MI underwent exploratory laparotomy with creation of stoma as and when needed. RESULTS: The duration of this study was 6½ years. Twenty-five neonates of MI were admitted. Of these, 22 had simple MI and remaining three had complicated MI. Eighteen neonates responded to the conservative management. In four neonates, who did not respond, exploratory laparotomy was performed. All three neonates having complicated MI underwent exploratory laparotomy. One patient expired in follow-up. CONCLUSION: MI is an important neonatal emergency, which needed immediate attention of a pediatric surgeon. Proper evaluation of the patient, careful application of principals of conservative management, and timely surgical intervention may fetch satisfactory results.

Needle Insertion in a Child: A Rare Form of Child Abuse.

Needle insertion is a rare form of child abuse that, though prevalent in the society as an attempted infanticide/homicide, remains undiagnosed and underreported. One should have strong suspicion for occurrence of child abuse in a child with history of needle injury. Here, we report an unusual case of 3-month-old boy with history of hypodermic needle extrusion from multiple sites of body and suspicion of possible child abuse.

Technique of Antireflux Procedure without Creating Submucosal Tunnel for Surgical Correction of Vesicoureteric Reflux during Bladder Closure in Exstrophy.

AIM: To report the clinical application of the new surgical technique of antireflux procedure without creating submucosal tunnel for surgical correction of vesicoureteric reflux during bladder closure in exstrophy. MATERIALS AND METHODS: Based on the report of published experimental technique, the procedure was clinically executed in seven patients of classic exstrophy bladder with small bladder plate with polyps, where the creation of submucosal tunnel was not possible, in last 18 months. Ureters were mobilized. A rectangular patch of bladder mucosa at trigone was removed exposing the detrusor. Mobilized urteres were advanced, crossed and anchored to exposed detrusor parallel to each other. Reconstruction included bladder and epispadias repair with abdominal wall closure. The outcome was measured with the assessment of complications, abolition of reflux on cystogram and upper tract status. RESULTS: At 3-month follow-up cystogram, reflux was absent in all. Follow-up ultrasound revealed mild dilatation of pelvis and ureter in one. CONCLUSIONS: The technique of extra-mucosal ureteric reimplantation without the creation of submucosal tunnel is simple to execute without risk and complications and effectively provides an antireflux mechanism for the preservation of upper tract in bladder exstrophy. With the use of this technique, reflux can be prevented since the very beginning of exstrophy reconstruction.

Antireflux Status Post Roux-en-Y anastomosis: An Experimental Study for Optimal Antireflux Technique.

BACKGROUND: Roux-en-Y hepaticojejunostomy has been a gold standard to establish biliary-enteric anastomosis for various surgical indications, but associated with variable incidences of cholangitis. This experimental study was conducted to report a modification in Roux-en-Y anastomosis for possible better alternative to provide antireflux procedure after Roux-en-Y biliary-enteric anastomosis with the aim to minimize the possibility of reflux and its consequences. MATERIALS AND METHODS: For experimental study, the required fresh segment of Lamb's small intestine was procured. Three sets of Roux-en-Y anastomosis were created for each experiment. In set 1, there was simple Roux-en-Y anastomosis. In set 2, Roux-en-Y anastomosis along with 4-5 cm long spur between the hepatic and duodenal limbs was created. In set 3, in addition to Roux-en-Y with creation of spur, additional antireflux mechanism was created at the junction of upper two-third and lower one-third of the hepatic limb. Saline mixed contrast was infused by infusion pump to raise the intraluminal pressure to more than 10 cm of H2O. X-ray was taken at that time. RESULTS: In set 1, all preparations demonstrated reflux of contrast in the hepatic limb. The set 2 also demonstrated the same findings of 100% reflux in the hepatic limb. In set 3, No reflux was observed in 8 (80%) preparations while remaining 2 (20%) preparations reveal partial reflux. CONCLUSION: This experimental study suggests that the provision of spur and additional valve may be able to decrease the possibility of reflux in Roux-en-Y biliary-enteric anastomosis.

IschioPubic Osteotomy, A Simple and Effective Technique for Pelvic Ring Closure in Repair of Classic Exstrophy Bladder.

AIM: The aim of this study is to report the technique and outcome of ischiopubic (IP) osteotomy for pelvic ring closure in classic exstrophy bladder. METHODS: A total of 85 male classic exstrophies were selected based on trapezoid-shaped space between IP ramus on three-dimensional computed tomography pelvis. Using midline scroto-perineal approach, after bladder plate mobilization and radical corporal detachment; the pelvic surface of superior pubic ramus (SPR) was exposed. Above the obturator canal, H-shaped incision was made on periosteum of the SPR. The horizontal line of H was placed above the obturator canal. Rectangular periosteal flaps above and below the horizontal line was raised. In the subperiosteal plane, curved hemostats encircled the SPR that were divided using a bone drill. On the medial aspect of ischial tuberosity, a notch was created as hinge using a bone drill. Forks of bone holding forceps hooked the pubic bone and tightened for its midline approximation, lengthening of the SPR and inward rotation of IP ramus. Linea alba and pubic bones were approximated with interrupted Polygalactin sutures. RESULTS: Midline approximation of pubic bone and linea alba was possible in all. There was no injury to obturator nerve, vessels, or other structures . CONCLUSIONS: IP osteotomy is the safe and effective technique of pelvic ring closure in patients with specific pelvic configuration.

Spontaneous common hepatic duct perforation in a child: A rare case report.

Spontaneous perforation of common bile duct is a rare phenomenon; few cases are reported in literature. Hence, there is a dilemma for the management of these cases, but with modern radiological equipment and high degree of suspicion, it is possible to diagnose early. The overall prognosis of this condition is good, provided an early surgical intervention is instituted; we are reporting a case of a 6-year-old male with spontaneous perforation of common hepatic duct. Managed by repair of rent over T-tube, postoperative period was uneventful, T-tube was removed after 3 weeks, and the patient is doing well in follow-up.

Lipoencephalocele in a Child: An Uncommon Swelling in the Occipital Region.

Neural tube defects are common congenital malformations of the central nervous system. The 3 most common neural tube defects are anencephaly, myelomeningocele, and encephalocele. Lipoencephalocele is an extremely uncommon entity with sporadic reports in the literature. We treated a 4-year-old gild with occipital lipoencephalocele. This report presents the clinical presentation and management of the patient along with a review of the relevant literature.

Perineal Schwannoma in a Child-an Uncommon Presentation.

Schwannoma is rare peripheral nerve sheath tumor arising from Schwann cells. It is generally benign but may show malignant transformation. Perineal location of these tumors is very rare. We operated a 6-year-old boy with perineal mass, which turned out to be a schwannoma. We are presenting the description of the case and review of the relevant literature.

Ileal perforation associated with dengue in the paediatric age group: an uncommon presentation.

Acute abdomen in dengue, a common arboviral disease found in tropical and subtropical countries, is not uncommon and can occasionally present as acute surgical emergency requiring urgent surgical intervention. The spectrum of acute abdomen presenting as surgical emergency in dengue infection that raises suspicion of an abdominal catastrophe includes acute appendicitis, acute cholecystitis, appendicitis and, rarely, intestinal perforation. All cases of intestinal perforation including appendicular, gastric and jejunal perforation have been reported in adult patients during the course of dengue infection. However, intestinal perforation during the course of dengue infection in the paediatric age group has never been reported. We report two cases of ileal perforation in children occurring during the course of dengue infection.

Chest Wall Infantile Fibrosarcomas- A Rare Presentation.

Infantile fibrosarcoma is rare and represents less than 1 % of all childhood cancers. Commonly, it arises in the limbs followed by the trunk and head and neck. We present a rare case of infantile fibrosarcoma of trunk at two different sites in a newborn with brief review of the relevant literature.

Surgical anatomy of the penis in hypospadias: magnetic resonance imaging study of the tissue planes, vessels, and collaterals.

OBJECTIVE: To report the surgical anatomy of the penis in hypospadias with study of vessels in relation to fascial planes, glans, corpora cavernosa, and corpus spongiosum using magnetic resonance imaging. MATERIALS AND METHODS: Twelve hypospadias presenting at older age (8-20 years) were studied with 1.5-T magnetic resonance imaging scanner and a 3-inch surface coil. Precontrast and postcontrast images were acquired using fast-spin echo sequences in sagittal, coronal, and transverse planes. The findings were processed in Volume Share 4.5, version Workstation, of General Electric Healthcare. Anatomic findings were verified during surgery. With imaging and surgical findings, a 3-dimensional conceptual diagram of surgical anatomy was created. RESULTS: Distinct layers of the skin, dartos fascia, Buck fascia, tunica albuginea, glans urothelium, lamina propria of glans, and corpus spongiosum were delineated with their spatial relationship. Axial pattern vessels of the dartos and its anastomosis with branches of dorsal penile vessels at the coronal sulcus, perforators along the corpus spongiosum, subglanular extension of the fascia, and intraglanular branches of the dorsal penile artery forming an arcade were visualized. CONCLUSION: Dorsomedial and dorsolateral axial pattern vessels are present in penile dartos with relative avascularity at dorsal midline in most cases. Subglanular extension of Buck fascia fused with the basal lamina propria of glans forms a barrier between the tip of corpora and the intraglanular arcade of vessels. Collaterals are present at coronal sulcus, along the bifurcated corpus spongiosum, and the dartos enabling blood flow between the terminal most branches of the external and internal pudendal vessels.