Clinical Profile, Outcomes and Predictors of Mortality in Neonates Operated for Gastrointestinal Anomalies in a Tertiary Neonatal Care Unit- An Observational Study.

Background: Gastrointestinal (GI) malformations have varied short-term and long-term outcomes reported across various neonatal units in India. Methods: This descriptive study was done to study the clinical profile, outcomes and predictors of mortality in neonates operated for congenital GI malformations in a tertiary neonatal care unit in South India between years 2011 and 2020. Details were collected by retrospective review of the case sheets. Results: Total of 68 neonates were included with esophageal atresia (EA) in 10, infantile hypertrophic pyloric stenosis (IHPS) in 9, duodenal atresia (DA) in 10, ileal atresia in 8, jejunal atresia in 5, anorectal malformations (ARM) in 11, meconium ileus/peritonitis in 9, malrotation in 2, and Hirschsprung's disease (HD) in 4. Antenatal diagnosis was highest in DA (80%). Associated anomalies were maximum in EA (50%), the most common being vertebral, anal atresia, cardiac defects, tracheoesophageal fistula, renal and radial abnormalities, and limb abnormalities association (VACTERL). Overall mortality was 15%. IHPS, DA, Malrotation, HD and ARM had 100 % survival while ileal atresia had the least survival (38%). Gestational age <32 weeks (odds ratio [OR] 12.77 [1.96, 82.89]) and outborn babies (OR 5.55 [1.01, 30.33]) were significant predictors of mortality in babies operated for small intestinal anomalies. None of the surviving infants were moderately or severely underweight at follow-up. Conclusion: Overall survival of surgically correctable GI anomalies is good. Among the predictors for mortality, modifiable factors such as in-utero referral of antenatally diagnosed congenital anomalies need attention. One-fifth had associated anomalies highlighting the need to actively look for the same. Although these neonates are vulnerable for growth failure, they had optimal growth on follow-up possibly due to standardized total parenteral nutritional policy during neonatal intensive care unit stay.

Yolk Sac Tumor of the Cervix in an Infant: A Surgical Dilemma.

A 5-month-old child presented with vaginal bleeding and found yolk sac tumor of the uterine cervix. She attained complete remission only with chemotherapy and on follow-up for more than a year. We report this for the unusual age and feasibility of avoiding surgery in selected cases.

A gastric outlet thickened mucosal fold associated with cytomegalovirus infection in an extremely preterm infant managed by surgical resection.

Non-bilious vomiting in preterm neonates discharged from neonatal intensive care units is a common complaint and is often associated with benign conditions such as gastro-oesophageal reflux. A neonate of 27 weeks gestation who presented later with vomiting owing to gastric outlet obstruction is described. He was discharged at 11 weeks of age and required re-admission 1 week later. He had persistent non-bilious vomiting from 7 weeks of age, failure to thrive and metabolic alkalosis. Clinical examination demonstrated visible gastric peristalsis, and hypertrophic pyloric stenosis was suspected. Ultrasound of the gastric pylorus and upper gastro-intestinal contrast studies were negative. Exploratory laparotomy after failure of conservative management revealed a thickened mucosal fold in the gastric pylorus, which was excised. Histopathology demonstrated inclusion bodies which are pathognomonic of cytomegalovirus infection. He was treated with valganciclovir for 6 weeks and was asymptomatic and thriving well at follow-up. Gastric outlet obstruction can be one of the manifestations of CMV infection of the gastro-intestinal tract. Diagnosis can be confirmed only by histopathology.Abbreviations: BPD: bronchopulmonary dysplasia; CMV: cytomegalovirus; H&E: haematoxylin and eosin; IHC: immunohistochemistry; IHPS: infantile hypertrophic pyloric stenosis; NEC: necrotising enterocolitis; PCR: polymerase-chain reaction; VGP: visible gastric peristalsis.

Laparoscopic management of gastric volvulus, diaphragmatic eventration and wandering spleen in a child.

Gastric volvulus (GV) and wandering spleen (WS) associated with eventration of diaphragm share a common pathological cause of absence or laxity of intraperitoneal ligaments. We herein report a rare case of a 13-year-old child presenting with an acute GV, WS, diaphragmatic eventration and an ectopic ascended kidney managed with a laparoscopic approach.

Urethral duplication in a 1 year old: a rare case report.

An 8-month-old male child presented with the complaint of two separate urinary streams from the penis. The child had no complaints of incontinence or recurrent UTI (urinary tract infection). Initial diagnosis of urethrocutaneous fistula was made and proceeded to micturating cystourethrography (MCU) and found to be having a urethral duplication. The duplicated urethra was excised and accessory urethra closed. Postoperatively, the child was followed up for 1 year and had no complaints of recurrence or incontinence. Through this case report, we learn about different classification systems and types of urethral duplication and their associated anomalies, and mode of management, which is mainly surgical. Further, management should be individualised to each patient based on their complaints and intraoperative findings.

Rare cause of extravasation from implantable venous access device.

Implantable venous access devices are routinely used, but they are not without complications. A 4-year-old male child with B cell acute lymphoblastic leukaemia was planned for chemotherapy. Chemo port was accessed through the right internal jugular vein. Check X-ray was done, which showed the correct placement of the catheter. Two months after chemo port insertion when the patient underwent chemotherapy, he developed a fever and he was started on intravenous antibiotics. On the next two admissions, the patient had a fever with chamber site oedema for which culture was done, which revealed Pseudomonas and Candida, which responded to antibiotic and antifungal therapies. In the successive admission, the patient had immediate local oedema on injecting chemotherapy. Exploration was done, which revealed chamber base perforation. It is an infrequent complication and has been reported in only three studies.

Diagnostic Dilemma of an Umbilical Mass in a Newborn Infant - a Twin or a Tumor?

BACKGROUND: We present the diagnostic dilemma of a neonate with two umbilical soft tissue masses. Case report: The baby had an umbilical mass herniating through the umbilical cord, and another mass hanging from the umbilical mass by a string of tissue. Both masses were amorphous solid soft tissues and the hanging mass had hair on the surface. Clinical diagnosis was umbilical cord teratoma. However, histopathological examination of the masses showed that tissues representing various organs were arranged in cephalocaudal order as in a fetus, revealing that it was a parasitic twin. The hanging mass was probably the cephalic part and the umbilical mass was malformed torso and limbs. Conclusion: This parasitic omphalopagus heteropagus parasitic twin presented as two amorphous masses without externally identifiable anatomic structure, The parasitic twin of omphalopagus heteropagus may have unusual presentations. Histopathological examination was essential to diagnose whether it is a twin or a tumor.

Implantable Venous Access Devices in Pediatric Malignancies - Institutional Experience in a Developing Nation.

BACKGROUND: Implantable venous access devices (IVAD) are preferred over long term external catheters in children due to less infection rates and better patient compliance in pediatric malignancies. Use of IVAD is a routine practice in developed part of the world. However it needs more emphasis for its widespread use in developing nation in order to improve the quality of care in children with malignancy. AIMS AND OBJECTIVES: We aimed at analyzing the outcome of IVAD in pediatric malignancies in a tertiary care set up of developing nation. Our objective is to enlighten the importance and feasibility of IVAD in childhood malignancies with review of literature. MATERIALS AND METHODS: There were 152 children who underwent IVAD insertion in the study period. The parameters analyzed were indications, patient demography, size of the port, laterality of insertion, method of access to internal jugular vein (IJV), duration of surgery, time for access, complications, indication for removal and the parental satisfaction. RESULTS: Mean age was 48 months. 112/152 patients had hematological malignancies. Right sided IJV was used by default in 97.4% patients, while remaining 2.6% had their left IJV cannulated. Open venotomy was used in 14 cases and 138 underwent ultrasound guided IJV access. The position of the catheter was reconfirmed in the X-ray, 6-8 hours after surgery. 149/152 ports were accessed 12 hours after surgery, whereas remaining 3 had a delay in access for 24 hours. Post operative complications were divided into early and late. 141 ports were removed after completion of chemotherapy, 4 were removed due to complications. 93 of parents gave the response as "satisfied". CONCLUSION: With proper training and expertise, insertion and care of IVAD is safe in pediatric malignancies without significant complications.

Androgen Receptor Expression in Hypospadias.

INTRODUCTION: The exact mechanism behind the development of hypospadias is unclear. Research studies on androgen receptor (AR) expression are controversial with results stating all possible outcomes - AR elevated, similar, or reduced when compared to normal. AIMS: The aim is to study the AR expression and hormone levels in hypospadias patients and compare them with children having normal genitalia. METHODS: Group 1 (controls) involved patients who underwent circumcision for phimosis while Group 2 involved hypospadias patients who did not receive any preoperative testosterone. Preoperative hormonal assay included luteinizing hormone, follicle-stimulating hormone, and free testosterone levels in all the patients. The foreskin specimen was analyzed for AR expression using immunohistochemistry (anti-AR antibody PathnSitu, clone R441, 1/100 dilution). AR staining was expressed as H score. The H score was calculated by multiplying the intensity of staining and the percentage of stained cells showing cytoplasmic positivity at high power (×40). RESULTS: There were 27 patients in Group 1 while 16 in Group 2 (distal 10; proximal 6).There was no significant difference in the age distribution. The mean H score was significantly higher (189.5) in hypospadias patients compared to controls (97.5) and was significantly higher in proximal (220) compared to distal (159) hypospadias. There was no significant difference in hormone levels between groups. CONCLUSION: AR expression was significantly elevated in hypospadias patients. It was higher in proximal compared to distal hypospadias, probably due to end-organ overexpression. Further larger trials are likely to through light into this controversial subject.

Laparoscopic Puestow Procedure for Chronic Pancreatitis in Children.

Chronic pancreatitis (CP) is a sequelae characterized by recurrent attacks of pancreatitis leading on to fibrosis and calcification of both ductal and parenchymal elements of the pancreas. Most of these, if left untreated at the right time, will lead on to burnt-out pancreas with complete atrophy of the parenchyma, leading on to endocrine/exocrine insufficiency. The pain in such patients is most likely due to ductal hypertension, parenchymal hypertension, and head mass causing perineural inflammation. Puestow procedure (longitudinal pancreaticojejunostomy) is the surgery of choice in selected patients with ductal hypertension where decompression of the duct provides symptomatic relief. Performance of such procedures in children by minimal access laparoscopic method is still evolving. We report a series of three patients (aged 10, 12, and 16 years) diagnosed with CP and successfully managed by laparoscopic Puestow procedure.

Clinicopathological profile of hepatoblastoma: An experience from a tertiary care center in India.

BACKGROUND: Hepatoblastoma is the most common primary hepatic malignancy in the pediatric population. Advances in pathological evaluation, imaging, risk stratification, neo-adjuvant chemotherapy, and surgery including transplantation have improved survival of these children in the western countries. However, a successful outcome in developing countries such as India with limited resources poses great challenges to the clinician and the family. Histology plays a major role in determining the prognosis of these patients. METHODS: A retrospective study was done on 10 children diagnosed with hepatoblastoma between January 2010 and December 2015 in our institution. Clinical, laboratory, radiological, histopathological diagnoses, treatment, and outcome data were collected and analyzed. RESULTS: The median age of these children at diagnosis was 11 months, and only 1 child was premature at birth. Most children were presented with abdominal distension. One child had lung metastasis at presentation. Elevated alpha fetoprotein levels were present in 90% of the children. The histological types were fetal, embryonal, macrotrabecular, and mixed epithelial-mesenchymal types. SIOPEL risk stratification was done, which showed 40% of the children to be of high risk. Three children had PRETEXT 1, 2, and 4, respectively. CONCLUSION: Our study is significant with respect to the information on PRETEXT staging, risk status, and histological favorability. In developing countries with limited resources and low-socioeconomic status, it is important to have a multidisciplinary team approach and tailor treatment to manage these patients effectively and improve the overall survival.

Institutional experience with laparoscopic-assisted anorectal pull-through in a series of 17 cases: A retrospective analysis.

AIMS: To retrospectively analyse the results of laparoscopically-assisted anorectal pull-through (LAARP) for high anorectal malformation (ARM) in male children in our institution. MATERIALS AND METHODS: We analysed the hospital records of patients who had undergone LAARP from October 2010 to December 2015 in terms of age, operative time, length of hospital stay and post-operative complications. RESULTS: Of 17 cases, 13 (76%) were in 6-12 months age group, whereas rest of them were in 12-18 months age group. The recto-prostatic urethral fistula was encountered in 82% (n = 14) of patients and rectovesical type in two cases (12%). The mean operative time was 132 min with mean length of hospital stay being 4 days. Rectal mucosal prolapse was the most common complication noted. CONCLUSIONS: LAARP is a feasible approach to male children with high ARMs with less post-operative morbidity.

Choice of Repairing Inguinal Hernia in Children: Open Versus Laparoscopy.

Inguinal hernia is a common disorder of childhood that requires surgical repair at diagnosis. Traditionally, it is operated upon by open inguinal incision. However, with the introduction of laparoscopic repair in 1990, opinion of scientific community is divided concerning the best method of pediatric herniotomy. Educated parents, who long to have the choicest of the best, often gather information from internet and prefer to discuss their concerns with primary care physicians. This descriptive review is intended to provide practicing pediatricians with updated evidence-based information which will enable them to counsel parents regarding the choice of hernia repair. Based on careful analysis of current literature, unacceptable standards are defined in this paper and rationalized recommendations are proposed. Laparoscopy appears to be beneficial in bilateral hernia of girls, giant hernia, recurrence following failed open repair and in hernia associated with undescended testis or ambiguous genitalia. On the other hand, open herniotomy appears to be advantageous in male inguinal hernia, unilateral female hernia, premature newborns, failed laparoscopic repair and in hernia associated with serious co-morbidity.

Stealth surgery: Subcutaneous endoscopic excision of benign lesions of head, neck and trunk in children.

BACKGROUND: Subcutaneous endoscopic excision of benign lesions of the head, neck and trunk is a new dimension in paediatric minimally invasive surgery. This study is a case series where cases are operated endoscopically with minimal scars. PATIENTS AND METHODS: This is a prospective study where in 13 patients who underwent Stealth surgery were enrolled in this study. Factors such as intraoperative time, blood loss, intraoperative complications, postoperative recovery and appearance and placing of minimal scars at inconspicuous sites were taken into consideration. RESULTS: All patients underwent successful surgery without converting to open surgery. Out of 13 patients, five were torticollis, four were suprasternal dermoid, one had chest wall swelling, one had swelling of arm, one had back lipoma and one had forehead lipoma. The mean operation time was 50 min (range 32-70). All patients were followed up regularly postoperatively. All patients were operated as daycare, less postoperative pain, no evident scars, lower complications related to scars. CONCLUSION: Subcutaneous endoscopic surgery addresses concerns related to scarring by replacing large visible incisions with smaller incisions placed in inconspicuous locations. This is a safe and effective procedure of early recovery.

Congenital hyperinsulinism: diagnostic and management challenges in a developing country - case report.

Management of congenital hyperinsulinemia of infancy (CHI) is challenging. A 4-month-old female infant with persistent hypoglycemia and elevated insulin levels was diagnosed with CHI. Gallium-68 DOTANOC positron emission tomography/computed tomography (PET/CT) scan (68Ga-labeled [1,4,7,10-tetraazacyclododecane-N,N',N'',N'''-tetraacetic acid]-1-NaI3-octreotide) demonstrated focal disease in the body of the pancreas. Genetic studies indicated paternal inheritance, making focal disease likely. She was started on diazoxide therapy with partial improvement in blood glucose levels. Due to a suboptimal response to diazoxide and the likelihood of focal disease amenable to surgery, a laparoscopic subtotal pancreatectomy with preservation of the head of the pancreas was performed. The biopsy demonstrated diffuse hyperplastic pancreatic islet cells on immunohistochemistry, indicative of diffuse rather than focal disease. Paternal inheritance is a recognized indicator of focal disease. Gallium-68 DOTANOC PET/CT scan is the only available imaging modality in South India as 18F-L-dihydroxyphenylalanine (DOPA) PET/CT scan is not available at present. A laparoscopic approach reduces the postoperative recovery time and morbidity in such patients. The absence of 18F-L-DOPA PET/CT scan and the limited supply of diazoxide makes the management of this complex condition more challenging in developing countries.

Testicular tumour in non-palpable undescended testis: A rare presentation and laparoscopic management in a 4-year-old.

Testicular tumour in an intra-abdominal undescended testis is a rare finding. We report a case of mature teratoma in an intra-abdominal testis of a 4-year-old boy. He presented with nonpalpable testis on the right side. Abdominal ultrasound was normal. Diagnostic laparoscopy revealed a large tumour arising from an intra-abdominal right testis, and histologic analysis identified a mature teratoma. The tumour was completely removed laparoscopically, and there was no recurrence at follow-up 1 year later.

Genetic diversity analysis of fungal pathogen Bipolaris sorghicola infecting Sorghum bicolor in India.

Bipolaris sorghicola (Lefebvre and Sherwin) is a well known and economically important seed-borne pathogen with the specific species of sorghum (Sorghum bicolor [L] Moench) as host. Thirty-two strains were obtained from different geographical area of sorghum growing places in India. Molecular characterization using three marker systems i.e., universal rice primers (URP), inter simple sequence repeat (ISSR) and random amplified polymorphic DNA (RAPD) was carried out. Molecular marker work revealed differences along with geographical origin clustering of various B. sorghicola strains which could not be revealed through conventional method of characterization. Out of 13 URPs, 20 ISSR and 50 RAPD primers screened, 8 primers each from URP and ISSR, and 10 primers from RAPD marker were found to result in reproducible banding pattern. One hundred per cent of polymorphic bands was recorded in all three molecular markers. Total number of bands was recorded 1986 with average of 248.25 in URP marker, and 2026 bands with average of 253.25 in ISSR marker and 2158 bands with average of 215.80 in RAPD markers. Maximum heterozygosity (Hn) was revealed by URP 17R (0.40), ISSR 10 (0.41) and RAPD marker OPC-5 (0.34). The polymorphism information content (PIC) values ranged between 5.89 to 8.28 in URP, 4.57 to 8.79 in ISSR and 4.44 to 9.64 in RAPD marker profiles. Maximum cophenetic correlation was found in URP (r = 0.910) followed by ISSR (r = 0.904) and RAPD (r = 0.870). The combined analysis of all three marker systems showed high cophenetic correlation (r = 0.911), which indicated a very good fit of the data for genetic diversity analysis. To best of our knowledge, this is a first report of genetic characterization of B. sorghicola. Hence, combined use of three marker systems would be more sensitive and reliable in characterizing genetic variability in B. sorghicola strains.

Day case laparoscopic cholecystectomy in children: A review of 11 cases.

AIM: The aim of this paper is to study the outcome of day case laparoscopic cholecystectomy (DCLC) in children. MATERIALS AND METHODS: A clinical pathway for day care laparoscopic cholecystectomy was followed with emphasis on the analgesia, post-operative nausea and vomiting (PONV), feeding, mobilization, pain scoring and patient satisfaction. Demographic and clinical data were recorded prospectively from March 2013 to November 2013. The setup allowed easy access to an overnight stay if needed. Hospital stay, complications, the need for medical advice after discharge, convalescence and patients satisfaction were analyzed. RESULTS: We admitted 11 children with symptomatic cholelithiasis for day case laparoscopic surgery. There were no intra- or post-operative complications. The incidence of PONV was 0/11. There was no readmission. 4/11 patients complained of shoulder tip pain on follow-up next day. There was no overnight stay. CONCLUSIONS: Adoption of a DCLC pathway is feasible and safe for children. Emphasis on adequate pain management and avoidance of PONV results in a high rate of day case surgery equivalent to that achieved in adult practice.

Ovarian preservation in children for adenexal pathology, current trends in laparoscopic management and our experience.

AIMS: Primary cysts and tumors of the ovary alongwith torsion are often regarded as an indication for open oophorectomy because of the fear of leaving an ischemic organ inside and chances of recurrence. We wish to report our experience of both modalities of management where we initially removed adenexal torsion by either laparoscopic salpingo-oophorectomy or oophorectomy, but later followed a more conservative approach of adenexal lesion removal with ovarian preservation. MATERIALS AND METHODS: Retrospective review of clinical records of patients with ovarian pathology who were managed laparoscopically. RESULTS: 46 cases of pediatric ovarian pathology were managed between March 2006 and March 2013 in two centers by a team of surgeons. The age ranged from 1 days to 18 years (average 14.3 years) and the pathology varied from 30 cases of a simple ovarian cyst with torsion, 3 cases of ovarian torsion without any cyst, 7 cases of a dermoid cyst with torsion in all, 1 case of secreting ovarian tumor and 5 cases of a paraovarian cyst with torsion. All patients had a normal tumor marker except 1 girl with a functional ovarian tumor who had elevated LDH and estrogen levels alongwith suppressed LH and FSH. In the initial period of our study we did 1 salpingo-oophorectomy for a suspected complex lesion and two oophorectomies for torsion with a simple cyst. In the later part of our study we performed laparoscopic cystectomy and ovarian preservation in 40 cases, including 7 cases of dermoid, where we performed laparoscopic detorsion with dermoid cystectomy and ovarian preservation in the same sitting. In three cases of chronic torsion who presented to us late, we could not preserve the ovary and had to resort to salpingo-ophorectomy. Histology showed a simple corpus luteal and follicular ovarian cyst in 31 cases, a paraovarian cyst in 5 cases with mature teratoma in 7 cases. Twenty-five patients with ovarian preservation following detorsion were subjected to follow-up ultrasound, who were found to have normal shape, size and blood flow compared to the contra lateral side. CONCLUSION: We outline our experience from the management of 46 cases of various ovarian pathologies with and without ischemia and found that ovarian torsion with a benign pathology and ischemia is not a contraindication for ovarian preservation, as pointed out in the current literature.