Journal of Neonatal Surgery

Thoracic heteropagus twins with an omphalocele

Daniel Acosta Farina — 2021-03-16

Spontaneous duodenal perforation in a neonate

Daniel Acosta Farina — 2021-04-16

A case of hernia of the umbilical cord with an accessory liver

Shohei Maekawa — 2021-05-26

Multiple lesions of congenital epulis

Aamer Iqbal — 2021-06-10

Congenital adhesion bands causing neonatal intestinal obstruction

Maria Luís Sacras — 2021-06-15

Jejunal atresia with ileojejunal knotting: An unusual presentation

Naresh Pawar — 2021-07-23

Type III wide colonic atresia: An etiological introspection

Moaied Hassan — 2021-08-23

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.

Anorectal malformations: Early outcome analysis in a tertiary care center in India

Priya Mathew — 2021-05-11

Background: Delayed diagnosis, referral, and presence of associated anomalies may influence the outcome of Anorectal malformations (ARM). The aim of this study was to analyze the early outcomes (1 month postoperatively) of ARM presenting in the neonatal period.

Methods: A prospective study was carried out in our tertiary care teaching institute from December 2018 to March 2020. All neonates admitted in the NICU with ARM were studied.

Results: There were 315 neonates; out of these, 236 (74.92%) were male and 79 (25.07%) were female. High ARM (HARM) was present in 265 (84.13%) and low ARM (LARM) in 50 (15.87%). Associated congenital anomalies were noted in 121 (38.41%). Major associated anomalies consisted of gastrointestinal (GIT) (41.32%), oesophageal (31.4%), and genitourinary (GU) (19.83%). Out of 306 procedures for ARM, 196 (64.05%) neonates underwent left transverse colostomy (LTC). The most frequent postoperative complications were thrombocytopenia (115) followed by sepsis (98). Colostomy prolapse was uncommon (2). The overall mortality was 87/315 (27.61%) neonates - 82/265 (30.94%) in HARM and 5/50 (10%) in LARM. Neonatal mortality was significantly high with birth weight <2500gm 55/153 (35.94%; p value= 0.0001), associated malformations 82 (67.76%, p value= 0.003); and delayed presentation 40/87 (45%), and with primary perforation 5/6 (83.33%).

Conclusions: Higher mortality was associated with low birth weight, double/ triple atresia, neonatal GIT perforation, sepsis on admission, and those with oesophageal and cardiac anomalies. More than 1/3rd (38.41%) patients had associated anomalies; thus, a detailed systematic evaluation of all subtypes is paramount.

Primary Posterior Sagittal Anorectoplasty in male neonates with Anorectal Malformations: A tertiary care hospital experience

Charu Tiwari — 2021-06-08

Background: The conventional surgical management for a male neonate with intermediate Anorectal Malformation (ARM) involves three stages – the creation of a diversion stoma in the neonatal period, a definitive pull-through procedure/ Posterior Sagittal Anorectoplasty (PSARP) followed by stoma closure. With this background, we present our experience with Single-stage primary definitive repair in selected male neonates with ARM.

Methods: Medical records of male ARM cases managed from 2016 to 2018 were reviewed. Male neonates who underwent primary PSARP were analysed retrospectively.

Results: A total of 35 records were found, out of which 12 male neonates underwent primary PSARP. The median gestational age and birth weight were 36.7 weeks and 2.75 kg respectively. Fistula with urinary tract was documented in all. The mean operative time was 65 minutes +/- 15 minutes. Two neonates had minor superficial surgical site infection at neo-anus. Anal dilatations were started after 2 weeks. At follow-up period of 3 years, 11 patients were continent; one patient had constipation with pseudo-incontinence which was successfully being managed by bowel management programme.

Conclusions: A primary definitive procedure is feasible when performed on carefully selected male neonates with ARM and also avoids the morbidity of stoma and multiple surgeries and follow-up visits to hospitals.

Outcomes following the establishment of Qatar’s quaternary neonatal general surgical service in a hospital with a “Tabula Rasa” for service development

Basem A Khalil — 2021-08-09

Background: Sidra Medicine is the first dedicated Children and Maternity hospital in the State of Qatar. This paper describes the preparations for and results of activating quaternary neonatal surgical services in a brand new, "greenfield" hospital. We believe that these are the first published national results of neonatal general surgical services from the Gulf region.

Methods: A review of surgical babies below four weeks corrected age from 1st of April 2018 – 31st of March 2020 was undertaken. Patient demographics, primary diagnosis, surgical procedures, 30-day postoperative mortality, overall mortality, and cause of death were recorded.

Results: One hundred and sixty-nine babies (169) were identified (44.4% term and 55.6% preterm). Major surgery included laparotomy (76), congenital diaphragmatic hernia repair (20), surgery for anorectal malformations (25), and esophageal atresia (13). One set of conjoined twins were also separated successfully. Fourteen babies died, resulting in overall mortality of 8.3 %. Excluding babies who died of life-limiting conditions, two babies died within 30 days of surgery, resulting in 30 days postoperative mortality rate of 1.2 %.

Conclusions: The neonatal surgical mortality rate is comparable with those from top international centers. This low rate could be attributed to the high level of expertise preceded by months of preparations.

Dilemmas with surgical intervention in neonates with Necrotizing Enterocolitis without pneumoperitoneum

Manika Boipai — 2021-07-09

Background: The diagnosis of perforation in the absence of pneumoperitoneum in necrotizing Enterocolitis (NEC) remains difficult. The decision to operate should be taken up during the “golden period” which occurs after the onset of intestinal gangrene, but before intestinal perforation. This study was done to analyse the outcomes of two groups of surgically managed NEC patients based on the radiographic findings (presence or absence of pneumoperitoneum) and compare the actual surgical findings.

Methods: A prospective observational study was performed from October 2018 to February 2020. The operated patients of NEC were divided into two groups based on the preoperative presence (Group A) or absence of pneumoperitoneum (Group B). A “p” value of less than 0.05 was considered significant for comparative analysis.

Results: There were 81 patients with clinico-radiological signs of NEC during the study tenure; 51 infants underwent surgery. Group A had 35 (69%) and Group B had 16 (31%) patients. In 11/16 (69%) patients of Group B, perforation was detected and 5/16 (31%) had only gangrenous and necrosis of bowel segment. Out of 16 patients (Group B), predictors of NEC like abdominal wall erythema (Modified Bell's stage IIB) were present in 4 (25%), fixed bowel loops (stage IA/IB) 3 (19%), pneumatosis intestinalis (stage IIA) 1(6%), portal venous gas (stage IIB) 1 (6%), and ascites (stage IIIA) 2 (12%) were observed. The complication rate was 77% among patients of Group A and 44% in Group B (p=0.01). Survival was greater (63% vs. 29%) among Group B patients than Group A (p=0.02).

Conclusions: The importance of not relying only on the pneumoperitoneum on radiographs; early diagnosis and prompt surgical intervention is emphasized. Surgical indication for NEC should always be based on the patient’s cumulative clinical and radiologic assessment, especially in the absence of pneumoperitoneum to improve survival rates.

Erudition after neonatal gastric transposition for esophageal atresia at 10 years of follow-up

Kanika Sharma — 2021-08-07

Background: Gastric transposition (GT) is a well-established procedure of esophageal replacement (ER) for children with esophageal atresia. ER in the neonatal period is sparsely reported. We report characteristics of children who have undergone neonatal GT and have completed ten years of follow-up.

Methods: The cross-sectional study was conducted which included all children who underwent neonatal GT at the institute and have completed a follow-up of at least 10-years. Anthropometry, oral contrast study, Hepatobiliary Scintigraphy, Gastroesophageal reflux study, Gastric emptying test, spirometry, and blood investigations were done for all the children.

Results: Four children (three male and one female) were included in the study. The mean age at ER was 5.3 ±2.2 days with a mean birth-weight of 2.43 ±0.13 kg. Two children had primary GT, while the other two had GT following a leak in primary anastomosis. During the mean follow-up of 180.25 ±43.5 months, none of the children required esophageal dilatation or other surgical intervention or procedures. All children were below 3rd centile for weight-for-age while all except one were below 50th centile for height-for-age. There was no stricture on oral contrast study, however, one child had grade III reflux on GER scan. Persistent duodenogastric reflux on HIDA scan was seen in one child. Three children had restrictive parameters on spirometry. Symptomatically, all reported poor weight gain, one had left vocal cord palsy with hoarseness, and one had chest-wall protuberance.

Conclusions: Neonatal gastric transposition is a feasible alternative to delayed esophageal replacement for neonates with esophageal atresia with limited complications.

The Raveenthiran Syndrome—Ectopic testis within Spigelian hernia: Few additional remarks

V Raveenthiran — 2021-09-09

Meconium obstruction in a premature neonate: An etiology that may simulate spontaneous intestinal perforation

James Glasser — 2021-09-23

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.

Upper pouch oesophageal duplication cyst- A rare association with Tracheoesophageal fistula: A case report

Deepa P. Makhija — 2021-05-17

Background: Oesophageal atresia (OA) has associated anomalies in 50 to 70 % of cases. The usual associations include cardiac, gastrointestinal, anorectal, renal, vertebral, or skeletal anomalies. Oesophageal duplication cyst as an association in OA is extremely rare. Despite the related embryogenesis of these two anomalies, there are very few cases reported.

Case Presentation: We present a low-birth-weight neonate with Type C Tracheo-oesophageal fistula (TEF) with an associated oesophageal duplication cyst.

Conclusion: Upper pouch oesophageal duplication with TEF is very rare and this is the fourth case managed concomitantly at primary surgery in English literature available.

Neonatal oral teratoma: A case report

Helle Moustapha — 2021-05-23

Background: Teratomas of the oral cavity are rare and can pose danger to a patient's life in terms of obstruction to the aerodigestive tract. These are the germ cell tumors that arise from all three layers of primordial germ cells.

Case Presentation: A 2-day-old female neonate presented with a mass protruding from the oral cavity since birth with no significant antenatal history. Examination revealed a large (8 cm), firm, and irregular mass protruding from the oral cavity extending up to the inner surface of the upper lip and onto the gums, associated with cleft palate. Complete excision of the mass was done and histopathology confirmed the diagnosis. There were no postoperative complications.

Conclusion: Oral cavity teratoma is a rare tumor with a good prognosis especially in its mature form. Surgical excision with multidisciplinary collaboration is needed in order to avoid any complications.

Intussusception in a premature newborn: A case report

Daniel Acosta Farina — 2021-06-18

Background: Intussusception is rare in the neonatal period and even less common in premature babies.

Case Presentation: We present a case of a premature newborn with an insidious clinical picture characterized by irritability and multiple vomits. Ultrasound was diagnostic of intussusception. The baby had a jejunal intussusception without any pathological lead point, with a favorable outcome.

Conclusion: Intussusception is a rare cause of neonatal intestinal obstruction especially in premature neonates.

Congenital pouch colon with colonic atresia- An unusual embryological association: A case report

Praveen Mathur — 2021-06-30

Background: Congenital pouch colon (CPC) is a rare variant of high anorectal malformation. More and more varied associations of CPC with other entities are being added to the literature.

Case presentation: A 1-day-old male baby presented to the emergency room with marked abdominal distension and absent anal opening. On exploration, the baby was found to have CPC (Type 2) with colonic atresia. This association has not been reported in English literature.

Conclusion: This is a report of a case of type 2 CPC with colonic atresia. To the best of our knowledge, such an association has not been reported so far. The final embryological outcome is dictated both by the topography and timing of vascular insult.

Colonic and type IV jejunal atresias with apple-peel bowel segment as the content of right inguinal hernia: A case report

Deepa P Makhija — 2021-07-20

Background: Colonic atresia is an uncommon entity. It may be associated with multiple anomalies.

Case Presentation: We present a case, which was initially suspected to be jejunal atresia, but was found to have colonic atresia as well intra-operatively. The associated jejunal atresia with apple-peel configuration was present as the content of inguinal hernia.

Conclusion: This case report highlights the rarity of the condition and the postoperative issues in the management of such patients.

Congenital Spigelian hernia in a neonate associated with several anomalies: A case report

Samira Sinacer — 2021-08-13

Background: Spigelian hernia (SH) is a rare entity characterized by a defect of the anterior abdominal wall located along the Spigelian line, it may be congenital or acquired.Association with other anomalies is worth reporting.

Case Presentation: A 22-day-old male newborn was admitted with strangulated right inguinal hernia and operated on emergently. Clinical examination also revealed a Spigelian hernia in the left lower abdominal quadrant with bilateral cryptorchidism, associated with polydactyly of the small right finger and anal stenosis. At surgery, the SH contained a part of the small intestine and the ipsilateral undescended testis.

Conclusion: Pediatric SH is rare, but its association with undescended ipsilateral testis is frequent. Other abnormalities can be concomitant to this association.

Congenital segmental dilatation of intestine with different morphology: A case report

Muhammad Bilal Mirza — 2021-09-12

Background: Congenital segmental dilatation of intestine is a rare entity characterized by a localized dilated segment of the intestine. It manifests as neonatal intestinal obstruction. Occurrence with another surgical anomaly may masquerade it and complicate the treatment course.

Case Presentation: A 1-day-old male neonate with anorectal malformation underwent colostomy as first stage of the treatment. The stoma did not function postoperatively. At repeat surgery, congenital segmental dilatation of the ileum, with slightly different morphology, was found. Resection of the lesion and ileostomy were formed. Postoperative course remained uneventful.

Conclusion: Congenital segmental dilatation of intestine may be missed at initial surgery. Complete surgical resection is curative.

Cystic hygroma and esophageal atresia with tracheoesophageal fistula: Is there a genetic association?

Sravanthi Vutukuru — 2021-09-12

Background: Tracheoesophageal fistula (TEF) is associated with many other congenital anomalies like cardiac, renal, and vertebral, but the association with cystic hygroma is very rare.

Case Presentation: We report a neonate, antenatally diagnosed with cystic hygroma of the neck and incidentally diagnosed to have TEF. The baby was operated on after adequate stabilization and the cystic hygroma was managed with injection sclerotherapy. The genetic analysis for the FOX genes complex has been done in this case and the results showed no association between the two.

Conclusion: There is a need for future studies to find out if the association between cystic hygroma and TEF is by chance or if any embryological or genetic cause can be attributed to it.

Intraluminal pyloric duplication cyst- a rare cause of non-bilious vomiting in a neonate: A case report

Ravi Patcharu — 2021-09-23

Background: Duplications of the alimentary tract are rare congenital malformations, out of which, true pyloric duplications constitute only 2.2%. They present with non-bilious vomiting and mimic hypertrophic pyloric stenosis (HPS). Pyloric duplications that are intraluminal are not separately visible at laparotomy, making their diagnosis difficult.

Case presentation: Our case is a neonate with an intraluminal pyloric duplication cyst who presented with recurrent vomiting. The radiological evaluation suggested a duplication cyst medial to the second part of the duodenum towards the stomach’s lesser curvature with features of gastric outlet obstruction. Intraoperatively, a cystic mass of 1 x 2 cm intraluminally was found on opening the pylorus which was excised and pyloroplasty was done. Postoperatively the baby was discharged in a stable condition.

Conclusion: Intraluminal pyloric duplication cysts are rare and the clinical presentation mimics HPS. They should be considered as a differential diagnosis in a neonate presenting with features of gastric outlet obstruction.

A case of prenatally diagnosed prune belly syndrome variant and congenital pouch colon in the United States: A case report

Mignote Yilma — 2021-09-25

Background: Prune belly syndrome (PBS) and congenital pouch colon (CPC) are rare congenital syndromes with a low incidence in the United States (U.S.) with most CPC cases being from India. In this case report, we describe, to the best of our knowledge, the first PBS variant and CPC patient in the U.S.

Case Presentation: A 30-year-old G2P0010 woman was referred to a tertiary center after an 18-week ultrasound showed a fetal abdominal mass. A prenatal MRI showed a dilated loop of bowel containing a mixture of urine and meconium, oligohydramnios, and a protuberant abdominal wall. Born at 37 weeks, the child’s physical exam was notable for a distended abdomen with thin abdominal musculature, non-palpable bilateral testes, no anal opening, and flat buttocks. Intra-operatively, a dilated cecum/ascending colon was noted with an abrupt change in caliber at the transverse colon, bilateral enlarged ureters, a left testis at the internal ring and no visualized right testis. A colostomy and mucous fistula were created 5 cm from the sigmoid pouch.

Conclusion: While most reported cases of CPC undergo single stage repair (one operation) at 1 day of life, our patient underwent the first procedure of a staged repair at 16 hours of life given his clinical instability at the time as well as his unknown urological anatomy in the setting of urinary obstruction. This case demonstrates the importance of fetal imaging, multidisciplinary approach at a tertiary care center, and reinforces a staged repair when necessary.

Feasibility of primary anastomosis for right-sided colonic atresia: A case report

Muhammad Shamil Suhaimi — 2021-10-19

Background: Colonic atresia (CA) is a rare cause of congenital intestinal obstruction. The management of CA is challenging because of its rarity.

Case Presentation: We present a case of right-sided CA in a term male baby weighing 3006 gm, without any other comorbidity. Type 1 atresia was seen at ascending colon and upon decompression, a reasonable caliber was achieved for a primary anastomosis following distal Cheatle slit and proximal tapering. Postoperative recovery was uneventful.

Conclusion: Primary anastomosis is a feasible option in right-sided CA when no major comorbidity is present and a normal distal colon with reasonable caliber is certain.

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.

A formal support group for esophageal atresia and/or tracheoesophageal fistula in South Africa may improve the outcome of these children and their families

Corné de Vos — 2021-05-20

The psychosocial aspect of various congenital anomalies like esophageal atresia (EA) with/or without a tracheoesophageal fistula (TEF) being the cause of Post-traumatic stress disorder (PTSD), depression, and anxiety in parents of these children, is often overlooked by medical professionals. Support groups may be useful to address a part of this problem and form an important aspect of emotional support for any rare or congenital disease. A cross-sectional descriptive study was conducted to assess the need for a formal support group for parents of patients born with EA and to emphasize the role of emotional support for these parents and children. A questionnaire was sent to members of an informal WhatsApp group which included participants with diverse demographics across private and public platforms, across South Africa. The questions focused on 3 main areas: demographic data, diagnosis and the surgery, and information about the participant’s emotional support. All the data were inserted in an excel datasheet and descriptive statistical analysis was used. Questions were sent to 14 members of an informal WhatsApp group. Eight were included in the study. All 8 participants were mothers of children born with an EA with a TEF. The questions about the need for emotional support focus on the time after the baby was born. The majority (87.5%) of the mothers felt that they could bond with their babies during pregnancy, but in contrast, 75% felt that they missed an opportunity to bond with their babies after birth, mainly because they were not allowed to hold their babies for up to 11 days after birth. Only 1 of the mothers were in contact with other families with children born with EA, and 6 of the remaining 7 agreed that this would have helped them during this emotionally challenging time. All 8 thought that sharing their stories with others could have helped them emotionally and all agreed that an EA/TEF support group was a good idea. The family, as another patient, ought to be treated and included in the overall treatment plan if we aim to improve the quality of life for both the patients and their families. Disease-specific support groups can play a major role in the emotional well-being of both the children born with EA and their families.

Umbilical cord hematoma

Nadji Boughaba — 2021-01-09

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.