Esophageal atresia associated with anorectal malformation: Is the outcome better after surgery in two stages in a limited resources scenario?

AIMS: To analyze whether outcome of neonates having esophageal atresia with or without tracheoesophageal fistula (EA±TEF) associated with anorectal malformation (ARM) can be improved by doing surgery in 2 stages. MATERIALS AND METHODS: A prospective study of neonates having both EA±TEF and ARM from 2004 to 2011. The patients with favorable parameters were operated in a single stage, whereas others underwent first-stage decompression surgery for ARM. Thereafter, once septicemia was under control and ventilator care available, second-stage surgery for EA±TEF was performed. RESULTS: Total 70 neonates (single stage = 20, 2 stages = 30, expired after colostomy = 9, only EA±TEF repair needed = 11) were enrolled. The admission rate for this association was 1 per 290. Forty-one percent (24/70) neonates had VACTERL association and 8.6% (6/70) neonates had multiple gastrointestinal atresias. Sepsis screen was positive in 71.4% (50/70). The survival was 45% (9/20) in neonates operated in a single stage and 53.3% (16/30) when operated in 2 stages (P = 0.04). Data analysis of 50 patients revealed that the survived neonates had significantly better birth weight, better gestational age, negative sepsis screen, no cardiac diseases, no pneumonia, and 2-stage surgery (P value 0.002, 0.003, 0.02, 0.02, 0.04, and 0.04, respectively). The day of presentation and abdominal distension had no significant effect (P value 0.06 and 0.06, respectively). This was further supported by stepwise logistic regression analysis. CONCLUSIONS: In a limited resources scenario, the survival rate of babies with this association can be improved by treating ARM first and then for EA±TEF in second stage, once mechanical ventilator care became available and sepsis was under control.

Chronic cervical lymphadenopathy in children: Role of ultrasonography.

AIM: To assess the usefulness of ultrasonography in the differentiation of causes of chronic cervical lymphadenopathy in children. MATERIALS AND METHODS: Children with palpable cervical lymph nodes were included. An ultrasonographic examination was performed to delineate multiple lymph nodes, irregular margins, tendency towards fusion, internal echos, the presence of strong echoes and echogenic thin layer. RESULTS: The total number of patients was 120. Echogenic thin layer and strong internal echoes were specific for tuberculosis. Long axis to short axis (L/S) ratio was more than 2 in most of the tubercular nodes (85.71%). Hilus was present in 50 (73.53%) tubercular lymphadenitis, 12 (40%) lymphoma and 10 (62.5%) cases with metastatic lymph nodes. Hypoechoic center was present in 60 (88.24%) tubercular lymphadenitis cases followed by 62.5% metastatic and 60% malignant lymphoma cases. CONCLUSIONS: Ultrasonography is a non-invasive tool for lymph nodal evaluation in children. It may be used to differentiate cervical lymphadenopathy with different etiologies in children. When correlated clinically, it may avoid biopsy in a patient.

Modified method of T-tube placement in cases of ruptured choledochal cyst having complete loss of anterior wall.

Survival rates for infants and children who have choledochal cyst with or without spontaneous rupture have improved dramatically in the past decades. Despite excellent long-term survival for patients with choledochal cyst who undergo elective surgery, many significant complications can occur in the patients being operated in emergency for rupture of the cyst. Spontaneous rupture of the cyst is one such problem resulting in considerable morbidity and mortality in these patients. Majority of surgeons manage these cases with T-tube external drainage. The conventional methods of T-tube placement for long period has remained simple as described in choledochotomies where there is no deficit of the walls of common bile duct (CBD). The present technique has been designed specially for the cases of ruptured choledochal cyst, where the wall of the CBD gets necrosed leaving behind a long gap between the two ends. In these cases, placement of T-tube with conventional method is not possible because there is no wall to suture together, and make the CBD water tight again to prevent leakage of bile. We found only two patients of spontaneous rupture of choledochal cyst with a long gap between two ends of CBD because of necrosed anterior wall. In both of these patients, it was not possible to put T-tube with traditional method and one would have to opt for primary definitive repair despite poor general condition of patients.

Vaginal agenesis: Experience with sigmoid colon neovaginoplasty.

AIM: Objective of this study is to report our experience with sigmoid vaginoplasty in adolescents. MATERIALS AND METHODS: A retrospective study of children with vaginal atresia and Mayer-Rokitansky-Kuster-Hauser syndrome. The sigmoid segment was used for vaginoplasty in all the cases. RESULTS: Eight children were studied over a period of 7 years. The postoperative complications were ileus in 2, mucosal prolapse of the neovagina in 1, and minor wound infection in 1 patient. Seven patients are on regular follow-up. All the neovaginas were patent and functional. One patient had unacceptable perineal appearance, that is, badly scarred perineum as a late complication. None of the patients had vaginal stenosis or excessive mucus discharge, during follow-up visits. Out of the 7 patients, 2 patients are sexually active and satisfied. CONCLUSIONS: Sigmoid vaginoplasty is a safe and acceptable procedure for vaginal agenesis with good cosmetic results and acceptable complications rate. Sigmoid colon vaginoplasty is the treatment of choice because of its large lumen, thick walls resistant to trauma, adequate secretion allowing lubrication, not necessitating prolonged dilatation, and short recovery time.

Renal leiomyoma in a child: a rare renal tumor.

Renal leiomyoma is a rare benign smooth muscle tumor of the kidney. Most cases are reported in adults, with very few cases described in children. We report a case of right renal leiomyoma in a 6-year-old boy which was suspected of being a Wilms tumor. Contrast-enhanced computed tomography could not differentiate leiomyoma in the kidney, and the mass was diagnosed as a Wilms tumor. Right radical nephrectomy was performed. Diagnosis of renal leiomyoma could only be achieved after histopathologic examination and immunohistochemistry. We recommend that total nephrectomy has to be done in most cases of pediatric renal tumors to avoid the risk of malignancy. There may be a role for nephron sparing surgery, provided the tumor is small, and one has a strong suspicion for the lesion being benign.

A diffusion tensor imaging study of deep gray and white matter brain maturation differences between patients with spina bifida cystica and healthy controls.

The aim of this study was to use diffusion tensor imaging (DTI) to identify differences in the maturation of deep gray matter (GM) and white matter (WM) between patients with spina bifida cystica (SBC) (n=29) with normal-appearing brains on conventional MRI, and age-matched and sex-matched healthy control participants (n=33). Changes in DTI metrics were calculated using a log-linear regression model. We observed increasing fractional anisotropy (FA) with age in the occipital, fornix, cingulum and middle cerebellar peduncles and decreasing FA with age in the genu and splenium of the corpus callosum (CC) and caudate nuclei in patients compared to controls. Increasing FA values in some of the WM structures probably represent faulty WM maturation, whereas decreasing FA values in the CC represents changes secondary to the affected WM fibers contributing to the CC. DTI changes in deep GM and WM in the absence of any abnormality on conventional MRI might provide the basis for cognitive decline in these patients.

Diagnosis and management of congenital bladder diverticulum in infancy and childhood: experience with nine cases at a tertiary health center in a developing country.

OBJECTIVES: The purpose of the study is to present the authors' clinical and surgical experience with congenital bladder diverticula in nine pediatric patients at a developing world tertiary care center. PATIENTS AND METHODS: Records of nine patients diagnosed and treated as congenital bladder diverticula from 2000 to 2007 were retrospectively reviewed for age, sex, chief complaints, associated anomalies, investigative work-up, operative notes, and postoperative follow-up. RESULTS: All were males. Age at presentation ranged from six months to eight years (mean three years). All were diagnosed postnatally by ultrasound and/or voiding cystourethrography (VCUG) and confirmed on urethrocystoscopy. Open surgical excision of diverticulum was done in all the patients. Ureteral reimplantation was simultaneously done only in three patients with VCUG-documented high-grade vesicoureteral reflux (VUR). With an average follow-up of four years, gradual resolution of symptoms was seen in seven of nine patients whose postoperative follow up records were available. There was no diverticulum recurrence at the defined mean follow-up. CONCLUSION: Pediatric patients with recurrent urinary tract infections and voiding dysfunction should always be evaluated for congenital bladder diverticulum. Although investigations such as intravenous urography (IVU), urodynamic studies, nuclear renal scanning, and, sometimes, CT scan and MRI, form an important part of preoperative diagnostic work-up and postoperative follow up, USG and VCUG may be enough when availability and cost are the constraints. Diverticulectomy, with ureteral reimplantation for high-grade reflux, provides good results without recurrence.

"Limited" double dorsal dartos flap coverage. An effective alternative to conventional flap coverage of the neourethra following Mathieu repair for subcoronal hypospadias.

OBJECTIVES: Double dorsal dartos flap coverage of the neourethra has been reported to be superior to single flap coverage for the prevention of urethrocutaneous fistula following hypospadias surgery. Conventional flap coverage involves covering the entire neourethra with vascularised flap. We describe a "limited" double dorsal dartos flap coverage of the neourethra following Mathieu repair for subcoronal hypospadias. MATERIALS AND METHODS: In a prospective study conducted between Feb 2003 and Feb 2007, 31 patients with primary subcoronal hypospadias who had a flat and narrow urethral plate with a healthy urethral meatus and minimal or no chordee underwent Mathieu hypospadias repair. A "limited" double dorsal dartos flap coverage of the neourethra was done in all patients. This involved covering only that portion of the neourethra which remained exposed after glans closure, i.e. the proximal neourethra. Primary surgical outcome assessed was the development of urethrocutaneous fistula following surgery. RESULTS: The mean age at the time of repair was seven years and mean follow up was eight months. There was no flap loss, urethrocutaneous fistula formation, or glanular dehiscence in any of the 31 patients. One patient developed meatal stenosis which was managed by dilatation. One patient developed superficial sloughing of the penile skin. Overall complication rate was 6.45%. CONCLUSION: "Limited" double dorsal dartos flap coverage of the neourethra seems to be an effective method to reduce the fistulous complication rate following Mathieu repair for subcoronal hypospadias. A larger comparative study needs to be done to evaluate conventional neourethral coverage with "limited" neourethral coverage.