Recurrent tracheo-esophageal fistula- management challenges: Case report and review of current literature.

Recurrent tracheoesophageal fistula is a rare complication of esophageal atresia surgery with an incidence of 3-15%. The presentation is subtle and is often missed, presenting as choking episodes during feed and recurrent chest infections. It is both a diagnostic and management challenge and requires a dedicated multidisciplinary pediatric surgical setup with adequate infrastructure for optimal management. We present a case of recurrent tracheoesophageal fistula which was diagnosed at our center. The patient underwent successful surgical management and is thriving well at six months follow-up period.

Assessing the Feasibility of Predicting Intermediate Outcomes of Esophageal Atresia Using Okamoto Classification: A Prospective Study.

Purpose: Several risk classifications based on various preoperative factors have been proposed to prognosticate the immediate survival of children operated for esophageal atresia. A major drawback of these classifications is that they only focus on immediate survival while ignoring the long-term morbidity and mortality in these children. Our study aims to bridge this gap in knowledge by studying the impact of one such classification (Okamoto's classification) on mortality and morbidity during a period of 1 year after discharge from the hospital in operated cases of esophageal atresia. Materials and Methods: After institutes ethical clearance, 106 children operated for esophageal atresia-tracheoesophageal fistula between 2012 and 2015 were studied prospectively for a period of 1 year after their discharge. The children were graded as per Okamoto classification. The primary objective was to determine the efficacy of this classification in predicting the survival rates in infancy and the secondary objective was to compare the complication rates in these children based on the classification. Results: Sixty-nine children met the inclusion criteria. There were 40, 15, 10, and 4 children in Okamoto Classes I, II, III, and IV, respectively. Twenty-one patients (30%) died during the follow-up period with the maximum number of deaths occurring in Okamoto Class IV (75%) and the minimum in Okamoto Class I (17.5%) (P = 0.003). There was a significant correlation between the Okamoto classes with the incidence of poor weight gain (P = 0.001), lower respiratory tract infection (P = 0.007), and failure to thrive (P = 0.01) higher in Okamoto IV and III as compared to I and II. Conclusion: Okamoto prognostic classification during the initial hospitalization is relevant even at 1 year follow-up with increased mortality and morbidity in Okamoto Class IV as compared to Class I.

A prospective observational study to evaluate the change in inhibin-B as a marker of sertoli cell function in children subjected to surgical correction for undescended testes.

Background and Objectives: Undescended testes (UDT) or cryptorchidism is a common congenital disability characterised by the absence of at least one testicle from the scrotum. The primary aim of surgical correction is to preserve fertility potential and prevent complications including trauma, malignancy, hernia and torsion. Often, children, especially in developing countries, present late with UDT. The effect of surgical correction in the recovery of Sertoli cell function in children aged more than 2 years is not apparent. The present study was conducted to study the change in inhibin-B level as a marker of Sertoli cell function in surgically corrected UDT in a heterogeneous population. Materials and Methods: A prospective observational study conducted over a 3-year period at a tertiary care paediatric surgery centre recruited 76 children with UDT undergoing surgical correction. Inhibin-B as a marker for Sertoli cell function was studied preoperatively and postoperatively. Continuous variables were summarised by calculating mean, standard deviation, median and interquartile range (IQR). Quantile versus quantile plotting was done to assess the distribution of the data. Data were analysed in two groups, with participants aged <2 years (Group A) and more than 2 years (Group B). Wilcoxon signed-rank test was used to compare the pre-operative and post-operative value. Results: In Group A (n = 39), the median (IQR) of pre-operative inhibin-B was 181 pg/ml (148-254) and post-operative inhibin-B was 230 pg/ml (176-296). In Group B, the median (IQR) of pre-operative inhibin was 70 pg/ml (44-104) and post-operative inhibin was 102 pg/ml (46-176). There was a significant increase in post-operative inhibin when compared to the pre-operative inhibin (P = 0.015 and 0.012, respectively, in Group A and B). Luteinizing hormone (LH) showed a significant decrease (P = 0.002) in Group A following surgery but bordering on significance in Group B (P = 0.43). On the other hand, follicle-stimulating hormone showed a significant decrease (P < 0.01) in Group B following surgery but not in Group A (P = 0.87). Conclusion: The mean post-operative inhibin-B levels were increased significantly as compared to the pre-operative levels indicating either a successful orchiopexy/adequate germ cell number or both. The benefit of orchiopexy may extend even to children presenting late for evaluation.

When Fryn met Edward: Two rare syndromes in a single patient.

A neonate born at our centre was diagnosed as Fryns Syndrome ie congenital diaphragmatic hernia with facial dysmorphism and distal limb anomalies, which is a rare disorder with only a few hundred cases reported till date.With high clinical index of suspicion and further evaluation, the diagnosis was confirmed. The baby was initially stabilized and later underwent repair of the diaphragmatic hernia. Despite best measures, the baby could not be salvaged. When severe, this can be lethal and diagnosis can only be made after autopsy. However, with early suspicion, better modalities of investigations available and improved NICU care, these babies can be salvaged. We report a case of Fryns Syndrome who was incidentally found to have Edward Syndrome as well. Such an extremely rare combination is yet to be reported in medical literature.Also with updated genetic studies, better diagnostics and treatment options coming up in future, there are chances to improve the survivability of these babies. It is prudent to document all such cases to aid in better understanding of the disease process.

Antenatally Detected Functional Adrenocortical Adenoma: A Case Report and Review of Current Literature.

Pediatric adrenocortical tumors (ACTs) are rare entities with an incidence of 0.2% of all pediatric tumors. Only two cases of antenatally detected ACT have been reported in the literature. Our case is the first report of an antenatally detected suprarenal mass which manifested with postnatal virilizing features and was proven to be adrenocortical adenoma on histology.

Common cloaca: An uncommon entity posing diagnostic and management challenges: Our experience with nine cases.

BACKGROUND: Cloacal anomalies are the severest and most complex of all anorectal malformations (ARMs). They require careful evaluation and meticulous surgery tailored to suit each variant. We present our experience in a series of nine cases. METHODS: This includes a retrospective review of 9 cases of cloaca managed at a tertiary care centre between 2015 and 2019. RESULTS: Associated anomalies were seen in 44% cases. The definitive surgery was performed at a mean age of 15.2 months (10 months-19 months), the definitive surgery being rectal separation with total urogenital mobilisation. The common channel as measured during panendoscopy was up to 3 cm in 7 patients (78%), and only 2 patients had a common channel of more than 3 cm (22%). Of the 34 procedures that these nine patients underwent, there were four complications (12%). The median follow-up period after stoma closure was 18 months (5-32 months), and the mean age at last follow-up was 38 months (22-48 months). Five children (63%) had spontaneous voiding and remained dry in the intervening period. Three patients (37%) had poor urinary stream with dribbling and high postvoid residue requiring clean intermittent catheterisation. Six patients had faecal soiling (66%); four had daily soiling; and two had occasional soiling. Four patients had constipation (44%). Seven patients (77%) required daily enemas for bowel evacuation and to remain dry. CONCLUSION: Cloacal anomalies are rare and complex ARMs. Satisfactory urinary and bowel continence rates can be achieved even in these complex anomalies.

Congenital pyloric atresia - nine new cases: Single-center experience of the long-term follow-up and the lessons learnt over a decade.

BACKGROUND: Congenital pyloric atresia (CPA) is a rare anomaly with an incidence of 1 in 100,000 live births. Depending on the type of anomaly patients can either present in the neonatal period or later in life with subtle nonspecific signs and symptoms. We present our institute's experience in handling these cases over the last decade and highlight lessons learnt. MATERIALS AND METHODS: We retrospectively reviewed records of patients diagnosed with CPA and managed at our centre between Jan 2006 to June 2016. We looked into the period of gestation, birth weight, gender, age at onset of symptoms, age at presentation to the hospital, symptoms, investigations, associated anomalies, management and outcomes and follow up periods. RESULTS: Nine patients were operated during the ten year period of study (6 males and 3 females). The median age at onset of symptoms was 06 months (01 day-36 months) and the median age of reporting to the hospital was 07 months (01 day-44 months) with a mean delay of 5 months between onset of symptoms and reporting to hospital. Six patients (67%) had associated anomalies including one with posterior urethral valve which has been reported for the first time in literature. Four out of five (80%) late presenters underwent an upper gastrointestinal endoscopy for diagnostic confirmation. All patients were operated upon and Type 1 CPA was seen in five patients (56%), Type 2 in two patients (33%) and Type 3 in one patient (11%). The overall survival was 89% as one patient with associated Epidermolysis Bullosa expired after 4 months due to fulminant sepsis. Three patients were lost to follow up and amongst the remaining five; the median follow up period is 36 months. CONCLUSION: CPA is a rare entity that may present late with subtle signs like failure to thrive and nonbilious vomit. A high index of suspicion is mandated in these cases and an Upper Gastrointestinal Endoscopy will help in early diagnosis and avoid further unnecessary investigations. A feeding jejunostomy may benefit malnourished sick children before definitive surgery. LEVEL OF EVIDENCE: Level 3, Type of study: Retrospective study.

Correlation between Quality of Life and Functional Outcomes in Operated Children with Anorectal Malformations Using the Krickenbeck Consensus.

OBJECTIVE: To correlate the functional outcomes in children operated for anorectal malformations (ARM) using the Krickenbeck consensus and their quality of life (QOL). METHODS: Thirty-three children operated at a tertiary care Pediatric surgery centre were studied 2 y after completion of all the surgeries and if more than 3 y of age. The functional stooling outcomes, type of anomalies and surgical procedures were tabulated using the Krickenbeck classification. The QOL questionnaire consisted of five parameters (Social habit, school attendance, daily activity, relation to peers and feeling) and the scoring ranged between 0 and 12: Good (8-12), Fair (5-7) and Poor (0-4). RESULTS: Median age of the children at the time of study was 7 y (Range 4-14). Comparison of stooling outcomes and QOL showed no significant difference (p 0.48). QOL was not affected by the age at evaluation, type of anomaly and type of surgical procedure. Children with vertebral, anorectal, cardiac, tracheoesophageal, radial and limb abnormalities (VACTERL) association had poorer QOL as compared to children without VACTERL association (p 0.02). Poor stooling outcomes were seen in children who underwent abdominoperineal pull through procedure (p 0.02). CONCLUSION: QOL appears to be independent of the stooling outcomes in operated children of ARM with majority enjoying a good QOL (93.9%).

Congenital cystic lesions of the lungs: The perils of misdiagnosis - A single-center experience.

BACKGROUND: A majority of cystic lesions in the western world are detected antenatally, whereas, the diagnosis in our setup occurs once the child becomes symptomatic. Surgical management is primarily dictated by the presence of symptoms, recurrent infection, and rarely by the potential risk of malignant transformation. MATERIALS AND METHODS: A retrospective analysis was carried out on all consecutive patients with cystic lung lesions managed at our center from January 2000 through June 2011 for antenatal diagnosis, presentation, diagnostic modalities, treatment, and complications. RESULTS: Forty cystic lung lesions were identified. Only 8% were antenatally detected. Out of 40, the final diagnosis was congenital cystic adenomatoid malformation in 19, congenital lobar emphysema in 11, and bronchogenic cysts and pulmonary sequestration in five each. Of these, 20% had received a course of prior antitubercular therapy and 30% had an intercostal drain inserted prior to referral to our center. Postoperative morbidity in the form of bronchopleural fistula, pneumothorax, and non-expansion of the residual lung was noted in 10% of the patients. CONCLUSION: Antenatal diagnosis of these lesions is still uncommon in third world countries. Prior to referral to a pediatric surgical center a large number of patients received antitubercular drugs and an intercostal drain insertion, due to incorrect diagnosis.