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.

Sepsis Screening of Neonatal Abdominal Surgery and Its Outcomes.

Aim: The aim of this study was to evaluate the early indicators of sepsis (sepsis screening) and their statistical correlation with sepsis in neonatal abdominal surgery. Materials and Methods: A prospective observational study was performed on thirty consecutive neonate cases aged between 0 and 28 days with surgical abdomen at the Paediatric Surgery Department, ABVIMS and Dr. Ram Manohar Lohia Hospital, New Delhi. The study duration was 18 months. Septic screening was done in all neonates on day 0, 1, 3, 7, and 14 days of surgery with serum procalcitonin, C-reactive protein, total leukocyte count, immature/total neutrophil ratio, and microerythrocyte sedimentation rate. A septic screening-positive patient (three or more positive parameters out of five) was correlated with sepsis and analysis was done. Results: A total of 30 neonates of abdominal surgical cases were included consequently, out of which 56.7% (n = 17) were male and 43.3% (n = 13) were female. Maximum cases were of congenital diaphragmatic hernia 20% (n = 6) and then anorectal malformation 16.7% (n = 5). About 70% of neonates were sepsis screening positive. Fifty percentage of neonates were diagnosed to have sepsis on the clinical or laboratory findings, so sensitivity and specificity of sepsis screening were 93.33% and 40%, respectively. There was total 30% mortality in this study. Conclusion: Sepsis screening is an early marker of sepsis, which can be used to help in early detection of neonatal surgical sepsis and timely intervention that can lead to decrease mortality and morbidity in neonatal surgery.

Prediction of Gap Length by Plain Radiograph of Chest with Nasogastric Tube in the Upper Esophagus in Patients with Esophageal Atresia and Distal Tracheoesophageal Fistula.

AIM: This study was aimed at prediction of the gap length between the two esophageal ends in cases of esophageal atresia and distal tracheoesophageal fistula (EA-TEF) by preoperative radiography with a nasogastric tube in the upper esophagus and its correlation with intraoperatively measured gap length. MATERIALS AND METHODS: All consecutive cases of EA-TEF were prospectively included in this study. Plain radiographs were taken with an 8 Fr nasogastric tube inserted in the upper esophageal pouch till its arrest. The patients were grouped into T1-T2; T2-T3; T3-T4; and T4 groups depending on the thoracic vertebral level of arrest of the NG tube on the radiograph. Intraoperative gap between the two esophageal ends was measured with Vernier caliper, and the patients were grouped into A, B, and C groups based on gap length (gap length >2.1 cm; >1-≤2 cm; and ≤1 cm). The operative gap groups were compared with the radiography groups. RESULTS: A total number of 118 cases were included over a period of 3 years. The arrest of nasogastric tube at T1-T2 and T2-T3 vertebral level corresponded to gap length Group A in 39/41 (95.12%) * patients. In gap length Group B, the arrest of tube at T2-T3 and T3-T4 vertebral level was seen in 44/44 (100%)* patients, in gap length Group C, the arrest of tube was noted at T3-T4 and T4 vertebral level in 31/33 (93.93%)* patients (*P < 0.001). CONCLUSION: Prediction of gap length by vertebral level of arrest of the nasogastric tube in the upper pouch in a preoperative chest X-ray correlated well with intra operatively measured gap length in cases of EA-TEF.

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.

Anorectal Agenesis with Rectovaginal Fistula: A Rare/Regional Variant.

AIMS AND OBJECTIVES: The anatomical types of female anorectal malformation (ARM) are complex and include several rare and regional variants. The aim of this report is to highlight the diagnosis and management of cases of anorectal agenesis with rectovaginal fistula (RVF). MATERIALS AND METHODS: This is a retrospective chart review of cases of RVF who underwent definitive surgery between November 2000 and September 2016 in a single institution. RESULTS: Of the 244 female patients with anorectal anomalies treated in our institution over a 16-year period, there were 15 cases of RVF with anorectal agenesis giving it an incidence of 6%. All cases of RVF with anorectal agenesis presented with absence of anal opening, varying degree of abdominal distension, and history of passage of fecal matter per vaginum. The mean age of presentation was 3 months (newborn to 1 year). Sigmoid colostomy was done in 13 cases. Distal colostogram demonstrated high RVF which was further confirmed by examination under anesthesia (EUA) and endoscopy before definitive surgery. With the posterior sagittal approach, division of the RVF and anorectoplasty was possible in 14 cases; however, one patient required an abdominal mobilization of the colon for pull through due to a very short distal bowel segment. During a mean postoperative follow-up of 5 years, cosmetic and functional results were satisfactory. CONCLUSION: RVF with anorectal agenesis is a rare/regional variant of female ARMs. Clinical examination along with distal colostogram, EUA, and endoscopy clinches the diagnosis. Anorectal reconstruction by posterior sagittal anorectoplasty results in a satisfactory outcome.

Epidemiology of Pediatric Trauma and its Pattern in Urban India: A Tertiary Care Hospital-Based Experience.

AIM: To assess the epidemiology, pattern, and outcome of trauma in pediatric population. MATERIALS AND METHODS: A total of 1148 pediatric patients below 15 years of age presenting in the emergency department of our hospital were studied over a period of 3 years. The patients were categorized into four age groups of <1 year, 1-5 years, 6-10 years, and 11-15 years. The data were compared regarding mode of trauma, type of injury, place of injury among different age groups and both sexes. RESULTS: The majority of the pediatric trauma cases were seen in males 69.86%, (n = 802) and females comprised only 30.13% (n = 346). Road traffic accident (RTA) was the most common mode of trauma in male children, i.e. 59.47% (n = 477) followed by fall injuries, i.e. 29.42% (n = 236). In females, fall was the most common mode of trauma, i.e. 52.31% (n = 181) followed by RTA (36.70%, n = 127). Fall injuries occurred mostly at homes. Among RTA, hit by vehicle on road while playing was most common followed by passenger accidents on two wheelers, followed by hit by vehicle while walking to school. Among fall, fall while playing at home was the most common. Out of total 1148 patients, 304 (26.48%) comprised the polytrauma cases (involvement of more than two organ systems), followed by abdominal/pelvic trauma (20.99%, n = 241), followed by head/face trauma (19.86%, n = 228). Out of total 1148 patients admitted over a period of 36 months, 64 died (5.57%). 75 (6.5%) patients had some kind of residual deformity or disability. CONCLUSION: The high incidence of pediatric trauma on roads and falls indicate the need for more supervision during playing and identification of specific risk factors for these injuries in our setting. This study shows that these epidemiological parameters could be a useful tool to identify burden and research priorities for specific type of injuries. A comprehensive trauma registry in our set up seems to be important for formulating policies to reduce pediatric trauma burden.

Pediatric esophageal substitution by gastric pull-up and gastric tube.

AIM: The aim of this study was to report the results of pediatric esophageal substitution by gastric pull-up (GPU) and gastric tube (GT) from a tertiary care pediatric center. MATERIALS AND METHODS: Retrospective analysis of the surgical techniques, results, complications, and final outcome of all pediatric patients who underwent esophageal substitution in a single institution was performed. RESULTS: Twenty-four esophageal substitutions were performed over 15-year period. The indications were pure esophageal atresia (EA)-19, EA with distal trachea-esophageal fistula-2, EA with proximal pouch fistula-1, and esophageal stricture in two patients. Mean age and weight at operation were 17 months and 9.5 kg, respectively. GPU was the most common procedure (19) followed by reverse GT (4) and gastric fundal tube (1). Posterior mediastinal and retrosternal routes were used in 17 and 7 cases, respectively. Major complications included three deaths in GPU cases resulting from postoperative tachyarrhythmias leading to cardiac arrest, cervical anastomotic leak-17, and anastomotic stricture in six cases. Perioperative tachyarrhythmias (10/19) and transient hypertension (2/19) were observed in GPU patients, and they were managed with beta blocker drugs. Postoperative ventilation in Intensive Care Unit was performed for all GPU, but none of the GT patients. Follow-up ranged from 6 months to 15 years that showed short-term feeding difficulties and no major growth-related problems. CONCLUSIONS: Perioperative tachyarrhythmias are common following GPU which mandates close intensive care monitoring with ventilation and judicious use of beta blocking drugs. Retrosternal GT with a staged neck anastomosis can be performed without postoperative ventilation.

Determinants of gap length in esophageal atresia with tracheoesophageal fistula and the impact of gap length on outcome.

AIM: This study was aimed at identifying factors which may affect the gap length in cases of esophageal atresia with tracheoesophageal fistula (EA-TEF) and whether gap length plays any role in determining the outcome. MATERIALS AND METHODS: All consecutive cases of EA-TEF were included and different patient parameters were recorded. Plain radiographs with a nasogastric tube in the upper esophagus were taken. Patients were grouped into T1-T2; T2-T3; T3-T4; and T4 depending on the thoracic vertebral level of the arrest of the tube. During surgery, the gap length between the pouches was measured using a Vernier caliper and the patients were grouped into A, B, and C (gap length >2.1 cm; >1-≤2 cm and ≤1 cm). The operative gap groups were compared with the radiography groups and the other recorded parameters. RESULTS: Total numbers of cases were 69. Birth weight was found to be significantly lower in Group A (mean = 2.14 kg) as compared to Group B (mean = 2.38 kg) and Group C patients (mean = 2.49 kg) (P = 0.016). The radiographic groups compared favorably with the intraoperative gap length groups (P < 0.001). The need for postoperative ventilation (70.83% in Group A vs. 36.84% in Group C, P = 0.032) and mortality (62.5%, 26.9% and 15.8% in Group A, B, and C, respectively, P = 0.003) co-related significantly with the gap length. CONCLUSION: Birth weight had a direct reciprocal relationship with the gap length. Radiographic assessment correlated with intraoperative gap length. Higher gap length was associated with increased need for postoperative ventilation and poor outcome.

Congenital pouch colon in girls: Genitourinary abnormalities and their management.

AIMS: To discuss the assessment and management of genitourinary (GU) tract abnormalities in 21 girls with Types I-III congenital pouch colon (CPC), studied over a period of 10 years. MATERIALS AND METHODS: Assessment included clinical and radiological assessment, examination under anesthesia (EUA), endoscopy of the lower GU tract, and evaluation of the surgical findings, operative procedures for the GU anomalies, and the results of management. RESULTS: Initial examination of the external genitalia showed a "clover-leaf" appearance (n = 6) and a single perineal opening (n = 6). In 9 patients, the openings of the urethra and double vagina were seen, of which a vestibular fistula was seen in 5 and an anterior perineal fistula in 1. Seventeen patients (81%) had urinary incontinence (UI) - partial in 10, and complete in 7. Renal function tests, X-ray sacrum, and abdominal US were normal in all patients. Micturating cystourethrogram (n = 9) showed a wide, bladder neck incompetence (BNI) with reduced bladder capacity in seven patients. EUA and endoscopy revealed a septate vagina in all patients and the urethral opening at a "high" position (n = 14) or at a relatively normal or "low" position (n = 7). In 8 patients, the intervaginal septum was thick and fleshy. Endoscopy showed a short, wide urethra, an open incompetent bladder neck, poorly developed trigone, and reduced bladder capacity in the patients with UI. The fistula from the colonic pouch opened in the proximal urethra (n = 4), high in the vestibule (n = 3), low in the vestibule (n = 8), perineum just posterior to the vestibule (n = 1), and undetermined (n = 5). Vaginoscopy (n = 8) showed normal cervices in all and cervical mucus in 4 patients. The subtypes of CPC were Type I CPC (n = 4), Type II CPC (n = 16), and Type III CPC (n = 1). All 21 patients had uterus didelphys. In four patients with UI, during tubular colorraphy, a segment of the colonic pouch was preserved for later bladder augmentation if required. A Young-Dees bladder-neck repair (BNR) was performed in four older girls for treatment of UI, with marked improvement in urinary continence in two girls, some improvement in one girl with complete urinary incontinence, and minimal improvement in one child. Division of the intervaginal septum was performed in three girls. CONCLUSIONS: GU abnormalities in girls with CPC need to be assessed and managed by a tailored protocol. UI is frequent, and its correction may require BNR. A segment of the colonic pouch can be preserved for possible future bladder augmentation. All girls have a septate vagina, often widely separated, and uterus didelphys. Gynecologic assessment and monitoring is required throughout adult life. Considering the wide opening of the vestibule, surgical management of the urogenital component by division of the intervaginal septum and if required, the vagino-fistula septum on each side results in a normal or a hypospadiac urethral opening and an adequate vaginal inlet.

Conservative management of isolated splenic abscess in children.

INTRODUCTION: Isolated splenic abscesses (SAs) are rare in children. We report a single-center experience with emphasis on their diagnosis, etiology, treatment, and outcome. METHODS: This is a retrospective review. RESULTS: Eighteen children (age, 3-16 years; male-female ratio, 5:1) were managed over a period of 8 years in a tertiary-care institution. Presenting symptoms included fever, abdominal pain, and anorexia. Splenomegaly was present in 12 (67%), leukocytosis in 9 (50%), and thrombocytosis in 12 (67%) patients. Associated diseases were thalassemia (1), tuberculosis (1), and typhoid fever (9). Solitary and multiple SAs were seen in equal numbers. Blood culture grew Salmonella paratyphi A in 1 case. Splenic aspirate culture was positive in 3 (Escherichia coli [1], S paratyphi A [1], Acinetobacter [1]). Widal serology was positive in 9 (50%) patients. Management consisted of intravenous broad-spectrum antibiotic therapy in all patients, together with percutaneous aspiration in 10 (56%) cases where the abscess size was greater than 3 cm. All patients responded, and complete resolution was observed. CONCLUSION: Isolated SA in children responds favorably to conservative treatment with intravenous broad-spectrum antibiotics and percutaneous drainage without the need for splenectomy.

The colovesical fistula in congenital pouch colon: a histologic study.

PURPOSE: The aim of this study was to perform a detailed histopathologic examination of the terminal colonic pouch and the colovesical fistula (CVF) excised during surgical management of male patients with the more severe forms (types I/II) of congenital pouch colon (CPC) associated with anorectal agenesis. METHODS: From January 2005 to December 2006, 25 male patients with types I/II CPC underwent abdominal exploration with dissection of the terminal portion of the colonic pouch and associated CVF till the bladder, division-ligation of the fistula, and excision of the colonic pouch. In 6 of the 25 patients, a complete dissection of the fistula to the bladder was possible, and in them, the terminal portion of the colonic pouch and the CVF were subjected to detailed histopathologic examination. The 6 patients included 3 newborns in whom this surgery was performed as a primary procedure, and 3 patients aged 3 months, 15 months, and 2 years, respectively, in whom a window colostomy of the pouch had earlier been performed. After due processing, multiple sections from the specimens were stained using the routine H&E method and examined under the microscope under different magnifications. RESULTS: In 4 specimens, the epithelial lining of the CVF consisted of transitional stratified epithelium with underlying anal/urethral glands. In 2 specimens, obtained from patients 15 months and 2 years old, respectively, the lining was of nonkeratinizing, stratified squamous epithelium. Other findings included aganglionosis in the muscle layers (n = 2), submucosal and subserosal fibrosis (n = 1), and thickening of muscle layers in the fistulous portion, suggestive of the presence of an internal sphincter (n = 2). CONCLUSIONS: The CVF in patients with types I/II CPC shows histologic features of the normal anorectal canal.

Spectrum of ectopic ureters in children.

We reviewed the clinicopathological features, diagnosis, and surgical treatment of ectopic ureters (EU) in children in order to evaluate the anatomy, renal function, outcome, and the existence of any racial or regional difference in this abnormality. The records of 27 consecutive patients with 33 EU managed over a period of 7 years were analyzed. There were 25 female and two male patients. Their age ranged from 1 to 13 years, with an average of 4.5 years. The presenting features included history of continuous dribbling of urine with otherwise normal voiding in toilet-trained female patients, incontinence of urine, urinary tract infection, abdominal pain, and renal failure. The diagnostic work-up included ultrasonography (US) in all 27 patients, intravenous pyelography (IVP) in 23, micturating cystourethrogram (MCU) in 19, nuclear imaging (NI) in 16, and computed tomogram (CT) in five patients. All patients underwent examination under anesthesia (EUA) and cystourethrovaginoscopy immediately before the operation. Single-system ectopic ureter (SSEU) was present in 19 patients with 25 ureters (six bilateral), and duplex with ectopic ureter was present in eight cases. The left side was involved in 14 cases, the right side in seven, and the anomaly was bilateral in six cases. Abnormalities noted in the imaging studies were USG 23/27, IVP 23/23, MCU 11/19, NI 13/16, and CT 5/5 cases. EUA and cystourethrovaginocsopy helped in localization of the ectopic site and evaluation of the bladder. The surgical treatment included nephroureterectomy (NUT) for non-functioning kidney in 7 patients, heminephroureterectomy (HNUT) for non functioning upper pole of duplex kidney with EU in 6 patients, ureteric reimplantation (UTR) in 13 patients (19 ureters, 6 bilateral), and ureteropyelostomy (UTP) in one patient. In the follow-up period ranging from 1 to 5 years, 20 patients achieved continence; however, six cases with bilateral SSEU and one case of unilateral SSEU, who also had a patulous bladder neck, continued to dribble urine. SSEU was more common than duplex with ectopic ureter. A large number of functioning renal units associated with SSEU deserved preservation. The success of surgical treatment in terms of achievement of continence was high and depended on the integrity of the bladder neck.

Bilateral adrenal abscess in a neonate.

A 23-day-old male baby with a history of perinatal hypoxia presented with refusal of feeds and abdominal distension. The child had a right-sided cystic upper abdominal mass and features of neonatal septicemia. Abdominal ultrasound (US) and contrast-enhanced CT scan showed bilateral adrenal abscesses. Laparotomy with drainage of the abscesses successfully treated the condition. The literature on the subject is reviewed.