Anorectal Malformations in Male Monozygotic Twins and Review of Literature.

Anorectal malformations (ARMs) are one of the common congenital gastrointestinal malformations with a prevalence of 0.2-0.6/1000 live births. There are only a few reported cases of familial ARMs in literature suggesting different patterns of inheritance. Among them, isolated ARMs in monozygotic twins are quite rare with significant male preponderance. Most of these ARMs are either of intermediate or low anomaly type. We reported two pairs of male monozygotic isolated ARMs from a tertiary care hospital in India and reviewed the literature in details adding to the total of nine pairs of twins till date.

Musculoskeletal and Esthetic Complications after Neonatal Thoracotomy: Revisited.

Aim: The aim is to study the complications of neonatal thoracotomy and its preventive measures. Methods: We retrospectively reviewed 53 neonates who underwent thoracotomy from January 2017 to December 2019 for a period of 3 years. Patient demographic data, primary disease for which they underwent thoracotomy, postoperative complications (immediate and delayed) during follow-up were documented. Results: During 3-year period, 53 neonates underwent thoracotomy for various surgical pathologies. The indications were esophageal atresia with tracheoesophageal fistula (n = 49), eventration of diaphragm (n = 3), congenital lobar emphysema of left upper lobe of lung (n = 1). Most of them were right posterolateral thoracotomies (n = 48, 90.56%) and left posterolateral thoracotomy was done in only 5 cases (9.43%). Associated anomalies were seen in 22 cases, such as cardiac (n = 19), renal (n = 4) and gastrointestinal (n = 5). Associated comorbidities seen in 14 cases; preterm (n = 4), low birth weight (n = 13), delayed presentation (n = 6). Early postoperative complications such as pneumonia (34%, n = 18) and wound infection (11.3%, n = 6) were noted. Delayed complications include musculoskeletal abnormalities (n = 19, 35.8%) and esthetic complications such as asymmetry of chest (5.6%). Conclusion: Neonatal thoracotomy is associated with complications such as pneumonia, wound infections, and musculoskeletal abnormalities such as asymmetry of chest and scoliosis. These can be prevented by adequate postoperative pain relief, muscle-sparing thoracotomies, avoiding tight closures, and nerve injuries. Long-term follow-up is required because these complications may manifest later on also. Early detection and institution of physiotherapy may help.

Complications and Management of Retained Double-J Stents in Children During the Coronavirus Disease-2019 Pandemic.

Background: Double-J (DJ) stents were commonly used for internal drainage after major reconstructive procedures or in cases of obstruction and ureteral injuries. They should be removed or changed within the stipulated time; otherwise, they can lead to various complications such as stent occlusion, migration, breakage, encrustation, stone formation at either end of the stent, and entanglement of the two stents if bilateral stenting was done. The present study focuses on the complications and the management due to delay in the removal of the DJ stents due to the coronavirus disease-2019 pandemic. Materials and Methods: This is a cross-sectional study over a period of 9 months. Children <12 years were included in the study. The patients' demographic data, indication for DJ stenting, time gap between DJ stenting and removal, complication with delay in DJ stent removal, and its management were recorded. Indwelling duration for >4 months was considered a delay in removal. All patients were followed up for 3 months. Results: A total of 10 patients were included in the study. Encrustation, proximal migration, distal migration, knotting of the stent, and entanglement of the bilateral stents in the bladder were observed. These complications were managed by various endourological procedures such as ureteroscopy, percutaneous nephroscopic, and cystoscopic removal. During follow-up, all patients were symptom-free. Conclusion: Prolonged indwelling stents can cause various complications. Endourological procedures are an essential armamentarium for a pediatric surgeon to manage these complications. Proper patient counseling regarding indwelling stents and maintaining stent registry and sending automatic messages and e-mails to patients may prevent these complications.

Epithelioid inflammatory myofibroblastic sarcoma: the youngest case reported.

Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a rare variant of the inflammatory myofibroblastic tumor. It has an aggressive clinical course and a high rate of recurrence. EIMS primarily affects children and young adults. Hereby, we report this entity in a 4-month-old infant who presented with an abdominal mass. Imaging studies revealed a large hypodense mesentery-based lesion involving the right half and mid-region of the abdomen. The mass with an attached segment of the small bowel was excised in toto. Grossly, a large encapsulated tumor was identified arising from the mesentery of the small bowel. The histological examination showed a tumor consisting of epithelioid to spindle cells loosely arranged in a myxoid background with numerous blood vessels and lymphoplasmacytic inflammatory infiltrate. On immunohistochemistry, the tumor cells showed positivity for ALK1 (nuclear), desmin, SMA, CD68, and focal positivity for CD30. A final diagnosis of EIMS of the small intestine was rendered. To the best of our knowledge, this case is the youngest reported case in literature.

Delayed presentation of anorectal malformations in a tertiary care hospital in India.

AIM: To study delayed presentation of ARMs, management and its effect on surgical and functional complications. METHODS: It is a retrospective study from March 2015 to March 2020. All the patients satisfying the criteria of delayed ARMs, i.e., presenting 7 days after birth were included. Information regarding type of ARM, mode of presentation, time of presentation, associated anomalies, management strategy, postoperative complications and functional outcome was noted. Minimum follow-up period was 6 months. RESULTS: Out of 102 patients with ARM, 44 patients presented late. Among the 44 patients, 9 were males and 35 were females. Associated comorbidities observed are low birth weight (n = 9) and preterm (n = 13). Associated anomalies observed were cardiac (n = 18), renal (n = 8), other gastrointestinal (n = 5) and skeletal (n = 1). (1) Male: rectourethral fistula-2 (staged repair), anal stenosis-3 (anoplasty) and anocutaneous fistula-4 (anoplasty). (2) Female: vestibular fistula: 15 (6 primary definitive surgery + 9 staged repair), ectopic anus: 3 (staged repair), anal stenosis: 2 (anoplasty), urogenital sinus: 4 (staged repair), H-type ARM: 8 (staged repair) and persistent cloaca: 3 (staged repair). Primary repair was done in 15 patients (34%), and staged repair was done in 29 patients (65.9%). Anoplasty was done in 9 patients, ASARP (modified tsuchida's procedure) in 8 patients and PSARP in 27 patients. Postoperative complications observed were constipation (n = 21, 47.7%), fecal incontinence (n = 12, 27.27%) with perianal excoriation in 2 patients, anal stenosis (n = 3, 6.8%) and rectal mucosal prolapse (n = 2, 4.5%) CONCLUSION: Delayed presentation of ARMs is not uncommon and is more common in females. Management is almost similar to those who present early. Those who present with chronic constipation and megarectum require staged repair. Complications were more frequent with delayed presentation. Hence, every newborn should have careful examination of perineum and screened for ARM to avoid possible morbidity and mortality.

Epithelioid inflammatory myofibroblastic sarcoma: the youngest case reported

Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a rare variant of the inflammatory myofibroblastic tumor. It has an aggressive clinical course and a high rate of recurrence. EIMS primarily affects children and young adults. Hereby, we report this entity in a 4-month-old infant who presented with an abdominal mass. Imaging studies revealed a large hypodense mesentery-based lesion involving the right half and mid-region of the abdomen. The mass with an attached segment of the small bowel was excised in toto. Grossly, a large encapsulated tumor was identified arising from the mesentery of the small bowel. The histological examination showed a tumor consisting of epithelioid to spindle cells loosely arranged in a myxoid background with numerous blood vessels and lymphoplasmacytic inflammatory infiltrate. On immunohistochemistry, the tumor cells showed positivity for ALK1 (nuclear), desmin, SMA, CD68, and focal positivity for CD30. A final diagnosis of EIMS of the small intestine was rendered. To the best of our knowledge, this case is the youngest reported case in literature.

Pediatric Thoracoabdominal Trauma: Experience from a Tertiary Care Center.

AIM: The aim of this study is to assess the pattern and mode of thoracoabdominal trauma and anatomical organ involvement, type of management employed, and overall outcomes in the pediatric population. MATERIALS AND METHODS: A retrospective study conducted at a tertiary hospital over a period of 38 months with a total of 198 pediatric patients <12 years of age with a history of abdominal and chest trauma between July 2014 and September 2017 were reviewed. Case files of all pediatric patients were evaluated. Information regarding age, sex, mechanism of injury, site of injury, mode of injury, nature of the injury, definitive treatment required, whether conservative or surgical and outcome of patients was evaluated. site of injury, mode of injury, nature of the injury, definitive treatment required, whether conservative or surgical and outcome of patients was evaluated. RESULTS: A total of 198 patients of thoracoabdominal trauma patients were studied. The majority of pediatric thoracoabdominal trauma cases were observed in males (n = 128, 64.64%) and females comprise only 35.35% (n = 70). Fall was the most common mode of trauma (58.08%) followed by road traffic accidents (30.30%), thermal injuries (9.09%) assaults in order of decreasing trends. The abdomen was the most common anatomical site of the injury (45.95%) followed by combined thoracoabdominal trauma (32.32%) followed by thoracic trauma (21.71%). Among the thoracic trauma, the most common comprised the lung contusions (37.20%) followed by the lung laceration comprising (25.58%) and rib fractures comprised only 20.93%. Among the abdominal trauma, the most common organ of injury was the liver (36.26%) followed by splenic trauma in 19.78% of patients. Approximately, 85% of patients were managed conservatively, and only 15% required major surgical intervention in the form of laparotomy and repair of bowel perforation, thoracotomy and ligation of bleeding intercostal vessels, partial and total splenectomy, repair of the liver laceration, and nephrectomy for the patient of Grade 5 renal injury with expanding retroperitoneal hematoma. Three patients died, one of which was Grade 5 renal injury with expanding retroperitoneal hematoma, two others were cases of combined thoracoabdominal trauma with massive hemothorax and both liver and splenic injury. CONCLUSION: The study defines the pattern of thoracoabdominal trauma, mode of trauma, and the prevalence of different organs involved in both the chest and abdominal cavity. We concluded that main indications for performing an operative intervention included severe hemodynamic instability, pneumoperitoneum, massive pneumohemothorax with significant shift and definitive confirmation of oral contrast leak on computed tomography films. A detailed trauma registry in our set up seems important for managing pediatric thoracoabdominal trauma.