Journal of Neonatal Surgery

Oxidized regenerated cellulose gauze as a hemostat in infantile hypertrophic pyloric stenosis

Rahul Gupta — 2020-12-31

Background: The Ramstedt pyloromyotomy is a standard procedure for infantile hypertrophic pyloric stenosis (IHPS). However, continuous postoperative bleeding may occur from the pyloromyotomy site without the use of any hemostatic management. We aim to analyze the pre-operative and post-operative hemogram values with and without the use of oxidized regenerated cellulose gauze as a hemostat in IHPS.

Methods: A prospective study performed from January 2019 to March 2020. The patients were randomly distributed by chit method into two groups: Group A (Control) without the use of hemostat and Group B (Case) with oxidized regenerated cellulose gauze as a hemostat.

Results: There were 26 patients with 19 males and 7 females. There were 13 patients in Group A and 12 in Group B; while one patient succumbed before surgery. Ramstedt’s pyloromyotomy was performed in 25 (96.16%) patients. A total of 7 (28%) postoperative complications were recorded in our patients. One patient in group A with intraperitoneal blood collection required blood transfusion for anemia caused by peritoneal bleeding. There was one postoperative death (group A). Significant statistical differences between preoperative and postoperative RBC (106/mm3), hemoglobin (g/dl), and hematocrit (%) levels were observed in group A but insignificant differences among the values in group B.

Conclusions: We recommend that oxidized regenerated cellulose should be applied to the pyloromyotomy site to minimize perioperative bleeding associated with the Ramstedt procedure. It appears to be a safe, easily available, easy to use, and effective hemostatic agent available for IHPS.

Predictors of recurrent strictures after oesophageal atresia repair

Olugbenga Aworanti — 2021-01-20

Background: Anastomotic strictures continue to complicate the outcome after oesophageal atresia (OA) repair. Multiple variables contribute to the development of strictures, and oesophageal dilatations are the mainstay of treatment. We aim to analyse the factors that impact the timing for initiation of oesophageal dilatations, the duration, frequency, and success of the dilatation regimen for OA.

Methods: It was a retrospective review of data (13-year) of children who underwent repair for Gross type C OA (OA with distal tracheo-oesophageal fistula). Delayed anastomosis was performed for long gap OA. Leaks were clinically obvious or identified on contrast swallow. Strictures that were symptomatic underwent oesophageal dilatations.

Results: The data of 72 children were analysed. The stricture rate was 37.5%. Ten had delayed repair, out of which 50% developed strictures compared to 35.5% who had a primary repair (P=0.48). There was no statistical difference in the mean birth weight (BW) and gestational age (GA) of children who developed strictures compared to those with no strictures (2.74kg vs 2.63Kg; P =0.548; 37.4 weeks vs 37.3 weeks; P=0.9). Children that underwent a delayed repair required significantly more dilatation sessions (12 vs 2 median sessions; P =0.001) and had a significantly prolonged duration of treatment (610 vs 63 median days; P = 0.013). There was a significant negative correlation between the GA and BW and the number of dilatation sessions required (P=0.03 and P=0.02, respectively). Linear regression revealed that delayed repair was the most important factor related to the number of dilatation sessions required (p <0.001); this was followed by lower GA or BW (p = 0.0265) and early onset of dilatations (p=0.0471).

Conclusions: The early onset of oesophageal dilatation for oesophageal strictures or when they occur in premature babies or those that have had a delayed repair, it should be anticipated that they would be refractory or recurrent.

Long term pulmonary morbidity after esophageal atresia and tracheoesophageal fistula repair

Sathyaprasad Burjonrappa — 2021-02-04

Background: Respiratory morbidity can influence a patient's quality of life after successful EA/TEF (Esophageal Atresia with and without Tracheo-Esophageal Fistula) repair. Multidisciplinary clinics have made it easier to manage comorbidities in patients with complex congenital malformations. The aim of this study was to evaluate the impact of respiratory issues after EA/TEF repair. Secondarily we sought to evaluate the contribution of respiratory symptoms by Broncho-Pulmonary Dysplasia (BPD) and Gastro-Esophageal-Reflux-Disease (GERD) in patients with EA/TEF.

Methods: Retrospective review of the medical record of 50 patients, who underwent EA/TEF repair, needing hospital readmission for pulmonary morbidity, and subsequently followed up in a surgical clinic was performed. The data collected included patient demographics, presence and nature of significant respiratory comorbidity, findings on imaging studies and bronchoscopy, and results of pulmonary function tests (PFT).

Results: Respiratory issues were identified in 75% of the patients. Congenital malformations and tracheomalacia were found in n=7 (14%) of cases. Prematurity associated BPD and Gastro-Esophageal Reflux were not the major cause of respiratory symptoms. Respiratory morbidity in this population included recurrent pneumonia n=18 (36%), reactive airway disease n=16 (32%), bronchiolitis n=4 (8%), bronchiectasis n=2 (4%), laryngitis n=2 (4%) and empyema n=1 (2%).

Conclusions: Pulmonary complications significantly impact the quality of life in terms of respiratory events, after successful EA/TEF repair. While GERD is common in surgically repaired EA/TEF patients, its exact role in precipitating pulmonary morbidity needs further study. Tracheomalacia can be managed conservatively without resorting to aortopexy.

A comparison between primary endorectal pull-through and staged procedures for patients with Hirschsprung’s disease

Reda Zbaida — 2021-02-12

Background: Primary endorectal pull-through is becoming a standard of care across the globe. This study was done to compare the outcome of patients with Hirschsprung’s disease (HD) who underwent primary endorectal pull-through with patients who were treated with a staged approach.

Methods: A retrospective data review was done of all patients diagnosed with Hirschsprung’s disease (HD) at Tygerberg Children’s Hospital, a tertiary hospital in Cape Town, during an 11-year period (2007 – 2018). The patients were divided into 2 groups: the primary Endorectal pull-through (ERP) group and the staged group and the two groups were compared.

Results: Eighty patients with histologically confirmed Hirschsprung’s disease (HD) were seen at our institution during the study period (2007 – 2018). Four patients did not meet the inclusion criteria and were excluded. Of the remaining seventy-six who were included, forty-six patients (60.5%) had a primary endorectal-pull through (Primary group) and thirty patients (39.5%) had staged procedures (Staged group) with a stoma before the final ERP. The peri-operative complications were subdivided into major and minor complications. Minor perioperative complications in the primary group were less (13%) compared to the staged group (33%) with a p-value of 0.017, making the difference statistically significant. Anastomotic strictures were the most common complication in both groups with abdominal wound infection being more common in the staged group. The late complications were similar in both groups with a p-value of 0.43. Constipation was the most common complication in both groups, followed by soiling.

Conclusion: The two groups had a similar outcome without statistically significant differences. We can safely conclude that the primary endorectal pull-through for HD is at least as safe as the staged approach in Sub- Saharan Africa. With this technique, we avoid a stoma and the necessity for two surgical procedures with added potential complications.

Anterior abdominal wall defects: Demographic and clinical profile and outcome at a tertiary care hospital

Parveen Kumar — 2021-04-20

Background: The anterior abdominal defects, especially gastroschisis and omphalocele have high mortality rates in developing countries. Time to intervene has been hypothesized to be associated with morbidity and mortality. The aim was to determine factors affecting mortality in neonates with anterior abdominal wall defects.

Methods: This retrospective descriptive study was done at a tertiary pediatric care center. The medical records of patients with a diagnosis of anterior abdominal defects (omphalocele/gastroschisis/umbilical cord hernia) admitted at our center from Jan 2015 to Dec 2019 were retrieved. The demographic and clinical data were studied including age, sex, religion, gestational age, associated anomalies, electrolytes at admission, septic profile, operative details, length of hospital stay, and mortality. The statistical data was fed on a Microsoft Excel worksheet and analysis was done.

Results: Thirty-nine (39) neonates were included in the study. M:F ratio was 2:1, with 61.5% belonging to the Hindu religion. The majority had term gestation (87.2%). Birth weight ranged from 1.5 to 4 kg (mean 2.47 ±0.5 kg). Eighteen (18) neonates had gastroschisis, 15 omphalocele major and 6 omphalocele minor, with a median age of presentation at 1 day of life. Time to intervention ranged from 0 to 5 days (interquartile range 1-1.25 days) after admission. Primary closure could be achieved in the majority (66.7%), while ventral hernia was created in 17.9% and the silo was needed in the rest. The mean postoperative length of stay was 9.31 days (±9.85 days) with a survival rate of 38.5%. The mortality rate in gastroschisis and omphaloceles were 61.1% and 38.1% respectively. The significant factors for survival were birth weight, and primary abdominal wall closure.

Conclusions: The present study brings out a different clinical profile of anterior abdominal wall defect patients. We recommend early surgery soon after stabilization and primary abdominal wall repair whenever abdominal pressures permit.

Thoracic heteropagus twins with an omphalocele

Daniel Acosta Farina — 2021-03-16

Spontaneous duodenal perforation in a neonate

Daniel Acosta Farina — 2021-04-16

Spontaneous pneumoperitoneum: Discerning from radiological imaging

Rahul Gupta — 2021-01-10

Background: Pneumoperitoneum without any gastrointestinal (GI) perforation or peritonitis is entitled spontaneous pneumoperitoneum. We aimed to describe a radiological perspective in spontaneous pneumoperitoneum.

Methods: This case series presented data of 4 cases of spontaneous pneumoperitoneum managed at our institution.

Results: There were 85 patients with a provisional diagnosis of necrotizing enterocolitis (NEC) and/or pneumoperitoneum. Out of these, there were 4 patients with the final diagnosis of spontaneous pneumoperitoneum; three males and 1 female. At presentation, respiratory distress was seen in 3. It was preceded by mechanical ventilation in 3 patients. All 4 had soft abdominal distension, absence of features suggestive of peritonitis, and the presence of free air with an absence of air-fluid level in peritoneal cavity on erect abdominal radiographs. The Rigler sign was present in 3 patients. Abdominocentesis followed by abdominal drain placement was performed in 2 patients. Laparotomy was performed in 1 patient which could not point to any pathology (negative). An unfavorable outcome was seen in one patient with associated esophageal atresia. No patient had any evidence (either ultrasound/radiological or on laparotomy) of leakage of contents from the GI tract.

Conclusions: In infants, especially preterm neonates, presenting with soft abdominal distension with abrupt onset of pneumoperitoneum, without clinical features of peritonitis and preceded by mechanical ventilation, diagnosis of spontaneous pneumoperitoneum should be considered.

Vanishing gastroschisis with jejunal atresia and extreme short bowel syndrome: A case series

Naresh Pawar — 2021-01-12

Background: Vanishing gastroschisis may occur due to spontaneous partial or complete closure of anterior abdominal wall defect around the viscera, leading to small bowel ischemia and resultant entry/exit level atresia and extremely short length of the remaining bowel. The prognosis is very poor, even after aggressive surgery, and requires prolonged total parenteral nutrition.

Case Series: We report two female neonates, one with closed and another with closing vanishing gastroschisis, associated with jejunal atresia and extreme short bowel syndrome. In both patients, the antenatal scans showed gastroschisis without the evidence of vanishing gastroschisis. In both neonates, palliative surgeries were done. Both patients died after a few days due to short bowel syndrome and sepsis.

Conclusion: When antenatally detected gastroschisis presents with closed or closing anterior abdominal wall defect, (vanishing gastroschisis), the parents/caregivers must be counseled about the poor prognosis of this condition. A tailored approach to either palliation or aggressive therapy is essential in this rare condition.

Extralobar pulmonary sequestrations hiding congenital diaphragmatic defects: A case series

Elisa Negri — 2021-03-03

Background: Association between pulmonary sequestration and diaphragmatic hernia is well known. Extralobar sequestrations are masses of the non-functioning lung, surrounded by separate pleura, without bronchial communication, and with a systemic arterial blood supply. They may be placed in the thorax, within the diaphragm, or rarely in a sub-diaphragmatic position.

Case Series: We present three cases of extra-lobar extra-thoracic pulmonary sequestrations associated with different types of diaphragmatic defects. In none of the three cases, the diaphragmatic defect was detected prenatally.

Conclusion: Pulmonary sequestration may be involved in the embryological origin of the diaphragmatic defect. Simultaneously, it acts as an anatomical barrier and prevents the herniation of the abdominal content into the thorax. If extralobar pulmonary sequestration is diagnosed prenatally, a coexistent diaphragmatic hernia should always be considered.

Phytobezoar causing intestinal obstruction in a neonate: A case report

Ravi Patcharu — 2020-12-31

Background: Phytobezoars are concretions of non-digestible vegetative matter in the gastrointestinal tract and are a rare cause of intestinal obstruction in children.

Case presentation: We report a case of intestinal obstruction in a 2-day-old neonate with no specific radiological features pointing to any common etiology. On exploratory laparotomy, a swollen raisin was found impacted in the ileum causing intestinal obstruction. The history taken in retrospect revealed that the elder sibling had witnessed her father perform a traditional ritual of putting a drop of honey into the mouth of the newborn and she imitated the same with a raisin, which led to the obstruction.

Conclusion: A careful detailed history of local traditional rituals is at times, the most important pointer towards the etiology of a clinical condition. The basic clinical skill of history taking is still very important, despite the availability of advanced radiological investigations.

Non-immune hydrops fetalis complicating bronchopulmonary sequestration: A case report

Jui Mandke — 2021-01-10

Background: Non-immune hydrops (NIH) associated with bronchopulmonary sequestration (BPS) is quite rare with a 95% risk of intrauterine fetal death, without a fetal intervention.

Case Presentation: We describe a case of an antenatally diagnosed extralobar BPS with severe NIH, who underwent fetal thoracentesis, but had worsening of NIH requiring an emergency cesarean section. Postnatally, the baby required skillful intensive care management and timely surgical management in the form of a sequestrectomy.

Conclusion: We report the smallest neonate with antenatally diagnosed NIH complicating BPS treated successfully by early neonatal surgery.

Two simultaneous intussusceptions in a neonate with DiGeorge syndrome: A case report

Uday Bhaskar MNS Mokrala — 2021-01-25

Background: Double simultaneous intussusception is a peculiar and rare variety of intussusception with only 3 previously reported neonatal cases.

Case presentation: A 15-day-old male neonate with respiratory distress was found to have Tetralogy of Fallot and hypoplastic pulmonary stenosis. Small bowel intussusception was diagnosed on ultrasound abdomen following hematochezia on the next day. Emergency laparotomy revealed two intussusceptions, ileocolic and jejunojejunal, with bowel gangrenous requiring resection and anastomosis. No pathological lead point was identified. He recovered with supportive care and was discharged.

Conclusion: Simultaneously occurring double intussusceptions are extremely rare in neonates, and thorough examination of the entire small bowel in cases of intussusception is key to the diagnosis.

Tongue shaped oropharyngeal teratoma with cleft palate in a neonate: A case report

Samiul Hasan — 2021-02-23

Background: Atypical presentation of an atypical abnormality makes the diagnosis challenging. Oropharyngeal teratoma (epignathus) is a rare, potentially life-threatening neonatal tumor. Its atypical presentation may cause a delay in diagnosis and increase morbidity and mortality.

Case presentation: A newborn girl with oropharyngeal teratoma and cleft palate presented with feeding difficulty. The airway was patent. The tumor was tongue-shaped, smooth-walled, displacing the native tongue, and prevented the fusion of two palatine halves resulting in cleft palate. On the 4^th day of life, complete excision was done transorally. Histopathology revealed a mature teratoma. There was no sign of recurrence at three months of follow-up.

Conclusion: Presentation of the oropharyngeal teratoma varies according to its site, size, and extension. Timely diagnosis is essential to avoid life-threatening respiratory obstruction. Complete excision of the mass is usually associated with a good prognosis.

Nasal overshoot: Probably a new entity

V Raveenthiran — 2021-02-25

Background: Congenital masses of the nasal tip are extremely rare. Differential diagnoses include nasal-tip teratoma, nasal chondromesenchymal hamartoma (NCMH), and heterotopic nasal glioma. Herein, the case of a newborn is reported that does not match with any of these differential diagnoses.

Case presentation: A newborn male presented with a congenital pedunculated mass arising from the nasal columella. The mass was symmetrically bilobed which is very unusual for any hamartomatous or neoplastic lesions. Histologically, it was composed of cartilage and ciliated epithelium.

Conclusion: Based on the uniqueness of the case it is hypothesized that the lesion could be an embryological error of frontonasal process overshoot defying, hitherto unknown, controlling mechanism. More research is needed to know how the morphometric proportion of the human body is determined by Nature.

Tracheoesophageal fistula with complex injury to the existing anomaly - Youngest neonate to survive: A case report

Namasivayam Selvarajan — 2021-03-29

Background: Complex injury to the esophagus, trachea, and tracheoesophageal fistula in a case of esophageal atresia is rarely reported that requires prompt identification and management.

Case Presentation: A very-low-birth-weight preterm with a tracheoesophageal fistula on ventilatory support for respiratory distress syndrome was transferred to our hospital. The baby developed air-leak through the tracheoesophageal fistula into the stomach causing gross distension of the stomach and reduction in oxygen saturation. Repositioning and reintubation failed to reduce the abdominal distension and to improve the saturation. Subsequent intubation with large volume, low pressure cuffed tube, and replacement of Replogle tube resulted in pneumothorax and further deterioration requiring emergency thoracotomy. Complex tracheoesophageal injury to the esophagus, trachea, and fistulous tract was repaired.

Conclusion: The baby survived the early repair of the tracheoesophageal fistula and the iatrogenic complex tracheoesophageal injury. Pre-operative tracheo-esophagoscopy with stenting of the fistula with Fogarty catheter helped to carry out a successful repair technically.

Auto-amputated ovary- rare cause of intestinal obstruction in a neonate: A case report

Naeem Liaqat — 2021-04-08

Background: Congenital ovarian cysts rarely auto amputate. An unusual complication of this event is secondary intestinal obstruction. The mechanism may be consequent to a direct pressure effect or to adhesions induced by the cyst.

Case Presentation: A neonate presented with an abdominal mass and intestinal obstruction. On exploration, she had a mass hanging over the small intestine and one ovary was absent. Mass was excised and it turned out to be an auto-amputated ovary

Conclusion: Although it is a rare pathology in neonates, surgeons must suspect it, particularly if an ovary is absent.

Congenital hepatic hemangioma presenting with neonatal intestinal obstruction: A case report

Sumaira Noor Maham — 2021-05-03

Background: Congenital hepatic hemangioma usually presents with abdominal distension. Rarely it may cause intestinal obstruction. We present a case of congenital hepatic hemangioma causing neonatal intestinal obstruction.

Case Presentation: A 4-day-old neonate presented with clinical and radiological features of neonatal intestinal obstruction. On exploration, a loop of jejunum was found adherent with a hepatic mass, arising from the left lobe of the liver. The hepatic mass profusely bled in an attempt of removing the adherent jejunal loop. Thus, the adherent portion was isolated and jejunojejunal end to end anastomosis was done. The hepatic mass along with an adherent small piece of jejunum was also excised. Histopathology showed hepatic hemangioma. The patient is doing fine on the 10-month follow-up.

Conclusion: We report a rare presentation of congenital hepatic hemangioma with neonatal intestinal obstruction. Although the optimum therapy for hepatic hemangioma is medical management, at times, surgical resection becomes a necessary option.

Management strategies for functional intestinal obstruction of prematurity

Olugbenga Awolaran — 2021-02-20

Functional intestinal obstruction of prematurity (FIOP) is characterised by a partial or complete failure of meconium evacuation due to hypomotility of the immature bowel and this presents with features of intestinal obstruction typically within the first 2 weeks of life. It contributes significantly to the morbidity and mortality of extremely and very low birth weight preterm infants. This disorder has been described using many terminologies and there is controversy amidst clinicians as to the optimal approach to its management.

This review summarises the characteristic clinical and radiologic findings to aid timely diagnosis and initiation of prompt treatment. Available evidence on different treatment options and their limitations is reviewed and practical stepwise management is described. In most cases, FIOP can be successfully managed conservatively with proactive management and monitoring.

Overall outcomes are favourable and normal long-term gastrointestinal function is commonly experienced. Evidence for investigations to exclude cystic fibrosis and Hirschsprung’s disease in preterm infants with FIOP is evaluated and a link with focal intestinal perforation is highlighted.

Umbilical cord hematoma

Nadji Boughaba — 2021-01-09