Management of urinary incontinence in girls with congenital pouch colon.

PURPOSE: This study describes the management of urinary incontinence (UI) in eight girls with congenital pouch colon (CPC) associated with anorectal malformation (ARM). METHODS: From 2013 to 2015, six girls with CPC and UI underwent bladder neck reconstruction (BNR). Four girls had complete UI (CUI) and two girls partial UI (PUI). From 2019 to 2023, four girls, including two with failed BNR, underwent bladder neck closure (BNC) and augmentation cystoplasty (AC) with a continent stoma. Subtypes of CPC were Complete CPC (n = 7) and Incomplete CPC (n = 1). All girls had a double vagina; short, wide urethra; and reduced bladder capacity with an open, incompetent bladder neck (BNI). During BNR, a neourethra was constructed from a 1.5-2 cm-wide and 1.5-3-cm-long trigonal strip. During BNC, AC was performed using a 20 cm ileal segment (n = 3) and by a colonic pouch segment, preserved during earlier colorraphy (n = 1). Continent stoma included a Monti's channel (n = 3) and appendicovesicostomy (n = 1). RESULTS: BNR produced moderate improvement of UI (n = 2), while UI was still very severe (n = 4). During BNC, intraoperative complications included iatrogenic vaginal tears (n = 4). Early complications included partial dehiscence of the ileocystoplasty (n = 1), partial adhesive small bowel obstruction (n = 1), and difficulty in stomal catheterization with prolonged drainage from the pelvic drain (n = 1). Late complications included unilateral grade II vesicoureteric reflux (n = 2) and vesicovaginal fistula (VVF) (n = 2) needing trans-vaginal closure in one girl. Urinary stones (n = 2) with stomal leakage of urine in one girl needed open cystolithotomy twice (n = 1), and endoscopic lithotripsy (n = 1). At follow-up, all patients have high overall satisfaction with the procedure and their continence status. CONCLUSIONS: BNC with AC and a catheterizable stoma satisfactorily achieves continence in girls with CPC and UI, vastly improving quality of life. If lower urinary tract (LUT) anatomy is favorable, BNR with/without AC can be the initial surgical procedure. BNC should be the primary procedure in girls with unfavorable LUT anatomy and for failed BNR. LEVEL OF EVIDENCE: IV.

Evaluation of the Outcome of Pyeloplasty in Children with Poorly Functioning Kidneys due to Unilateral Ureteropelvic Junction Obstruction.

Aim: To analyze the outcome of upfront pyeloplasty in kidneys of children with unilateral ureteropelvic junction obstruction (UPJO). Materials and Methods: Thirty-three consecutive cases with split renal function (SRF) of ≤20% on dynamic renal scintigraphy (DRS) underwent upfront pyeloplasty with a nephrostomy tube and trans-anastomotic stent. Outcome was analyzed based on symptomatic relief, nephrostomy output, surgical complications and changes noted in pre-and post-operative findings on renal ultrasound (US), and DRS. Results: The most common symptom was abdominal lump in <5-year age group (79%) and abdominal pain in >5-year age group (93%). Postoperatively, symptoms were relieved in all (100%), parenchymal thickness (PT) on US improved in 82% and SRF improved significantly (>5%) in 75.8% of patients. The improvement was more significant in patients with abdominal lump and large kidneys. The mean nephrostomy output showed an inverse relationship with age at pyeloplasty and a direct correlation with the change in PT and SRF. The degree of improvement in SRF also was inversely related to the age at pyeloplasty with a significantly better outcome in <2-year-age. Although age at pyeloplasty, nephrostomy output and change in PT individually showed significant correlation with change in SRF, multiple regression analysis showed PT as the only significant factor. Conclusion: Upfront pyeloplasty should be the first option in children with poorly functioning kidneys as it has a favorable outcome in almost all the cases with a very low incidence of complications. The degree of improvement in SRF can be predicted by the nephrostomy output and improvement in PT on US.

Morbidity and Mortality of Necrotizing Fasciitis and Their Prognostic Factors in Children.

Background: This is a prospective study of the clinico-etiologic profile and factors affecting outcomes in 40 children managed for necrotizing fasciitis (NF). Materials and Methods: Demographic details, clinical characteristics, and laboratory parameters were recorded, and the Laboratory Risk Indicator for Necrotizing Fasciitis (LRINEC) score was calculated. Primary outcome (survival vs. nonsurvival) was noted, and prognostic factors were identified. Results: Initiating factors included boils (45%), i.v. cannula extravasations (22.5%), and blunt trauma (17.5%). Lesion (s) were predominantly on the lower limbs (35%) and trunk (25%). Twenty-two patients (55%) had <5% body surface area (BSA) involved. Severely deranged clinical and laboratory parameters were common. Ultrasound localized fluid collections. Pus cultures showed methicillin-resistant Staphylococcus aureus (52.5%), methicillin-sensitive S. aureus [27.5%], and polymicrobial growth (20%). Blood culture was positive in 24 patients (60%). Most isolates were sensitive to clindamycin and amoxy-clavulanate. Prognostic factors for mortality (n = 6; 15%) included categorization as "Sick," BSA involvement >10%, thrombocytopenia, raised serum creatinine, late debridement, and polymicrobial blood culture isolates. All six nonsurvivors had a LRINEC score of ≥8 and positive blood cultures. Six patients (20.7%) developed unsightly scars and 5 (17.24%) contractures across joints. Conclusions: Pediatric NF has significant morbidity and mortality. Patients with adverse prognostic factors can benefit from early referral to a facility with a critical care unit. Adequate wound management is essential to minimize residual deformity.

Correlation of the Position of the Anal Dimple with the Caudal Termination of the Striated Muscle Complex in Patients with Anorectal Malformations.

Background: In children with anorectal malformations (ARM), the vertical fibres of the striated muscle complex (SMC) are believed to be located within the limits of the anal dimple (AD). Methods: Forty five cases of ARM underwent posterior sagittal anorectoplasty (PSARP), median age 8.5 months. During PSARP the anterior, posterior limits and midpoint of the AD and SMC were marked. The location of AD was correlated with SMC. Results: A 'well developed' AD and SMC was seen in 80 % and 86.7 % patients respectively. The mean width of the AD and SMC was more in females than in males (20.96 vs. 18.98 mm and 14.24mm vs. 13.45mm respectively). In 36 cases (80 %), across the spectrum of ARM, the SMC was posterior in relation to the AD. In 7 cases (15.5 %) it correlated in position with the AD and in 2 cases (4.44 %), it was anterior to the AD. Conclusions: In the majority of cases AD and SMC were 'well developed' and the location of the SMC does not correlate with that of the AD. This has significant practical value in the important step of optimizing the placement of the rectum through the center of the SMC during repair.

Kluth Type I-2 Variant of Pure Esophageal Atresia - First Case Report and Challenges in its Management.

This is the first case report of the Kluth type I-2 variant of esophageal atresia. The peculiar anatomy of this variant does not suit (1) esophageal substitution via posterior mediastinal route, (2) esophageal lengthening for preserving native esophagus and (3) distal esophageal stump stoma for gastric feeds.

Congenital Pouch Colon in Girls: An Algorithm for Preoperative Diagnosis.

Aim: The aim of the study was o define the preoperative diagnostic clinical and radiological features in girls with congenital pouch colon (CPC). Materials and Methods: Records of 47 girls with CPC, managed from 1996 to 2018, were reviewed. There were two age groups: Group A (newborn to 12 months; n = 26) and Group B (>12 months to 20 years; n = 21). The important clinical and radiologic features to help in a preoperative diagnosis were noted. Results: The most common subtype was Type II (57.4%), followed by Type I (23.4%) and Type III (12.8%). The features common to both the groups were abdominal distension (A = 53.8%; B = 9.52%), severe perineal excoriation (A = 19.2%; B = 23/8%), and urinary incontinence (A = 30.7%; B = 85.7%). In addition, in Group B, fecaloma on abdominal palpation was noted in 28.6% of patients. The characteristic appearance of the perineum including external genitalia and findings on plain abdominal X-ray (AXR) were 100% accurate and hence diagnostic. These unique features helped us formulate an algorithm for preoperative diagnosis of this uncommon form of anorectal malformation in girls seen in North India. Conclusions: The characteristic features on clinical examination should alert one to the presence of CPC in the outpatient clinic. The AXR was diagnostic in 100% of cases and is mandatory. If any doubt persists, examination of the genitalia under anesthesia with more retraction of the labial folds and endoscopy can be performed for confirmation. These measures should enable a clinician to make an accurate preoperative diagnosis in every girl with CPC.

Results of Injection Sclerotherapy with Bleomycin in Pediatric Lymphatic Malformations.

AIM: The aim of the study was to evaluate the results of injection sclerotherapy with bleomycin in pediatric patients with lymphatic malformations. MATERIALS AND METHODS: In this prospective cohort study, all consenting pediatric patients with macrocystic lymphatic malformations were managed with injection bleomycin sclerotherapy (0.5 mg/kg, not exceeding 5 mg at a time) under ultrasound (US) guidance. After aspirating the cyst fluid bleomycin was instilled intralesionally in a ratio of 5:1 (aspirated cyst fluid volume: diluted bleomycin solution volume). Patients were reassessed at three weekly intervals. The response to therapy was assessed clinically as well as by size and volume on ultrasound Doppler study. The response was classified as excellent response, i.e., complete regression, good response >50% regression, and poor response <50% regression. RESULTS: Sixty patients with lymphatic malformations were enrolled in the study, the mean age was 3.22 years, and the male-to-female was 2.5:1. The most common site of lesion was in the neck (43.3%), followed by the axilla (15%) and flank (8.3%). The responses were excellent, good, and poor in 43 (71.6%), 12 (20%), and five (8.3%) patients, respectively. Two patients underwent surgical excision of the residual lesion. Complications noted were fever in six, local pain in five, and residual lesion in three patients. CONCLUSION: Sclerotherapy with bleomycin is simple, safe, and effective in the first line of management for macrocystic lymphatic malformations in children.

An Analysis of Safety and Efficacy of Day-care Surgery in Children in a Tertiary Care Hospital in India.

BACKGROUND: Advances in surgery and anesthesia have paved the way for the establishment of day-care surgery (DCS). Observations that children achieve better convalescence in the home environment along with significant economic advantages have led to this paradigm shift in clinical practice. AIMS AND OBJECTIVES: This study is aimed to evaluate the feasibility of performing various surgical procedures on day-care basis and assess parental satisfaction with DCS in children. MATERIALS AND METHODS: In this prospective observational study, all children >3 months of age undergoing various elective surgical procedures as day-care cases in our institution were enrolled. Types of operations, complications, including any unplanned admissions and parental satisfaction, were recorded. RESULTS: Between December 2015 and December 2018, a total of 654 day-care surgeries were performed in our institution by pediatric surgeons. The mean age was 5.5 years with M: F 5.5:1. Thirty different surgical procedures were successfully performed as DCS, the common procedures being inguinal herniotomy (31.5%), and orchidopexy (14.3%). Unplanned admissions were recorded in 2.29% (15/654) patients (scrotal edema-5, postoperative pain-8, and a long recovery from anesthesia-2). No major complications occurred; two minor complications during follow-up were superficial wound infection and drug reaction. Overall parental satisfaction was very high (100%)-preoperative prolonged fasting period and long waiting time in the preoperative room of afternoon shift patients (7.95% and 8.3%) were the reasons for their discontent. CONCLUSIONS: DCS in children is safe and effective with high parental satisfaction. It can substantially reduce the waiting list for several surgical procedures in children.

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.

Radiologic Evaluation of Uterovaginal Abnormalities in Girls with Congenital Pouch Colon.

BACKGROUND/PURPOSE: The radiologic investigations of 25 girls with congenital pouch colon (CPC), managed over 17 years, were retrospectively reviewed. In 13 girls who form the study group, the investigations provided information about the anomalous uterovaginal (UV) anatomy and these findings were studied. MATERIALS AND METHODS: Age at presentation was 2 months to 10 years. The subtypes of CPC were Type I (n = 2), Type II (n = 9), and not recorded (n = 2). All patients had a double vagina and a unicornuate uterus on each side in the pelvis. The radiologic studies, performed at varying periods after surgery, included an intravenous urogram (IVU) (n = 4), micturating cystourethrogram (MCU) (n = 3), distal ileostogram/colostogram (n = 6), and magnetic resonance imaging (MRI) (n = 7). RESULTS: IVU and MCU showed retrograde filling of the vaginas with contrast during micturition with a small-capacity urinary bladder and a relatively open bladder neck. The two vaginas were quite apart, but symmetrical in appearance and position. A distal dye study showed filling of the colonic pouch, its terminal fistula, and the two vaginas in six patients. Opacification of the bladder was seen in 3/6 girls, including one girl with left-sided Grade IV vesicoureteral reflux. MRI (n = 7) showed a monocornuate uterus on each side in the pelvis. The upper vaginas on each side were apart, being widely apart (n = 5) and somewhat closer (n = 2). The lower vaginas were closer with an intervaginal septum. Other findings were a widely open bladder neck and urethra in two girls with urinary incontinence and visualization of the terminal fistula of the colonic pouch (n = 2). The lumbosacral spine was normal in all patients. CONCLUSIONS: In girls with CPC, retrograde reflux of contrast into the vaginas during a distal dye study or an IVU/MCU may provide useful details of the anatomy of the vaginas. An MRI scan is recommended as essential for comprehensive evaluation of the anomalous UV anatomy.

The position of the anal dimple in newborns and infants with anorectal malformations and its correlation with the normal anal position.

BACKGROUND/PURPOSE: The anal position index (API) defines the normal anal position as the ratio of fourchette-anal distance to fourchette-coccyx distance for females and the scrotum-anal distance to scrotum-coccyx distance for males. In this study, measurement of the API in newborns and infants with anorectal malformations (ARM), using the center of the midline anal dimple (AD) to represent the center of the proposed neoanus, was performed to assess whether or not the AD was located in a significantly abnormal position as correlated with the normal anal position. METHODS: The cases comprised 65 consecutive newborn and infants with ARM, divided into 2 age-based groups (Group A: 1st day to 1month; Group B: 1-12months), without sacral or significant perineal abnormalities. Controls included an equal number of age and gender matched patients admitted for other conditions. The characteristics of the AD ('well developed' or 'moderately developed') as well as those of the midline perineal raphé in males and the perineal groove in females were also recorded. RESULTS: Combining both age groups, the mean API±SD was 0.41±0.012 in male cases and 0.53±0.07 in male controls (p value 0.003). The corresponding values for female cases and controls were 0.31±0.09 and 0.36±0.07 respectively (p = 0.040). In male newborns, the API in cases was significantly lower than the API in controls (p<0.001). Combining both males and female newborns, the differences between the API in cases and controls was also statistically significant (p<0.001). In older boys as well as in girls from both age groups, apart from cases of persistent cloaca, the API was lower in cases than in controls, especially in boys, although the difference was not statistically significant. On comparing the API in individual male ARM with that in male controls, cases of rectobulbar urethral fistula (RBUF) and rectovesical fistula had a statistically significant lower API (p<0.001). In the 4 cases of persistent cloaca, the mean API (0.40±0.1) was higher than the mean API in female controls and the AD was well developed with a well-delineated, narrow perineal groove. The majority (53/65; 81.53%) of patients had a 'well developed' AD. Twelve patients (18.47%) had a 'moderately developed' AD including 10 females and 2 males. There were 15 patients with a depressed AD (anal fossette); of these 12 (80%) were females. All boys had a well delineated perineal raphé in contrast to 10 girls (29.4% of total female ARM) who had a poorly delineated midline perineal groove. CONCLUSIONS: During definitive surgery for ARM, if the AD is taken as the site of the proposed neoanus, the neoanal position is likely to be anterior to the normal anal position in both males and females and especially so in males. Overall, girls with ARM appear to be more likely to have a relatively poorly developed and/ or depressed AD and a poorly delineated perineal groove. TYPE OF STUDY: Clinical Research. LEVEL OF EVIDENCE: Level III.

Congenital Pouch Colon.

Congenital pouch colon (CPC) is an unusual abnormality in which a pouch-like dilatation of a shortened colon is associated with an anorectal malformation. It is categorized into four subtypes (Types I-IV) based on the length of normal colon proximal to the colonic pouch. In males, the pouch usually terminates in a colovesical fistula just proximal to the bladder neck. In girls, the terminal fistula opens either into the urethra or in the vestibule, close to the urethral opening. Girls usually have a double vagina with a wide inter-vaginal bridge, a monocornuate uterus on each side, and urinary incontinence due to a widely open bladder neck. Associated major malformations are uncommon with CPC but sometimes, especially in reports from outside India, major abnormalities are present suggesting an early, severe error in embryogenesis. The more severe Types I/II CPC can usually be diagnosed by a large gas shadow or air-fluid level on X-Ray abdomen. For all subtypes of CPC, it is preferable to preserve a segment of the pouch by fashioning a narrow colonic tube for pull-through, the technique known as coloplasty or tubular colorraphy. Girls need additional management of the genitourinary abnormalities. Postoperatively, fecal continence levels are usually poor, especially with Types I/II CPC.

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.

Assessment of Nutritional Status of Patients of Congenital Pouch Colon Following Definitive Surgery.

PURPOSE: To assess the nutritional status in 31 patients of congenital pouch colon (CPC) who had undergone definitive surgery and closure of a protective stoma, if any, at least 1 year earlier and were below 14 years age. MATERIALS AND METHODS: The clinical history, demographic details, anthropometric measurements, and results of hematological and biochemical tests were recorded. In addition to collective data, analysis was also performed after grouping by age, subtype of CPC (Types I/II and Types III/IV CPC), and in Types I/II CPC patients, by whether the colonic pouch had been completely excised or else a segment preserved by tubular colorraphy (TC). RESULTS: Severe fecal incontinence (FI) was common (64.52%). Anthropometry showed a significant malnutrition in 53.85-95.45% patients, especially stunting which was most prevalent in the 0-5 years age-group. Serum Vitamin B12, folate, and Vitamin D were lower than normal in 38.71%, 22.58%, and 74.19% patients, respectively, without statistically significant difference among the various groups studied. Patients with Types I/II CPC had a statistically significant higher incidence of anemia, low serum ferritin, and severe FI than patients with Types III/IV CPC. Patients with Types I/II CPC, managed by excision of the colonic pouch, had a higher incidence of severe FI, wasting, and thinness than those undergoing TC. CONCLUSIONS: On follow-up of the patients of CPC, anthropometry shows a high incidence of malnutrition, especially stunting in the 0-5 years age-group. There is an adequate adaptation of fluid-electrolyte homeostasis. Although Types I/II CPC patients have a significantly higher incidence of anemia and severe FI than Types III/IV CPC patients, long-term anthropometric parameters are similar. In Types I/II CPC, preservation of the colonic pouch by TC offers long-term benefit.

Protocol-based management of 154 cases of pediatric liver abscess.

AIM: Although liver abscess (LA) is prevalent worldwide, especially in developing countries, there is a paucity of data regarding the protocol for management of LA in children. The aim of this study was to analyze the outcome of a protocol-based management of pediatric LA from a single institution. MATERIALS AND METHODS: This prospective observational study was conducted in a tertiary-care children's hospital and included all patients with LA managed over a 5-year period. Detailed clinical, laboratory, microbiological and imaging parameters were recorded, and the patients were treated according to a standardized management protocol. Small abscesses (<5 cm) and those with solid appearance on initial ultra-sonogram (US) were managed with antimicrobial therapy alone. Liquefied abscesses on imaging were additionally subjected to US guided percutaneous needle aspirations (PNA). Large abscesses pointing to the surface were treated by percutaneous drainage (PCD). Ruptured LA, abscess inaccessible to image-guided drainage and those not responding to other modes of treatment were subjected to open surgical drainage (OSD). Pleural collections were treated by aspirations or intercostal tube drainage. Outcome was analyzed in terms of duration of recovery and complications including mortality. RESULTS: Over the 5-year period, 154 pediatric LA patients underwent protocol-based management. The mean age of the patients was 6.76 years with the male:female being 1.26:1. Medical management alone, PNA, PCD and OSD were successful in 38 (24.6%), 76 (49.3%), 11 (7.14%) and 29 (18.8%) cases, respectively. Pleural collections developed in 43 (27.9%) patients out of which aspiration/drainage was needed in 24 (55.81%) cases. The mean duration of hospital stay was 20.67 ± 9.52 days. Ongoing sepsis and multi-organ failure lead to mortality in 6 (3.8%) cases; rest of the patients were doing well on follow-up. CONCLUSION: Ultra-sonography was useful for the initial diagnosis, monitoring the progress and management of LA in children. The outcome of a protocol-based management of LA in children was favorable.

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 associated with Pseudoexstrophy: Report of Two Cases.

This report describes two newborn girls with single perineal opening (cloaca), and pseudoexstrophy in the form of divergent pubic bones and rectus muscles, and a low-set umbilicus. Both patients had a type II congenital pouch colon (CPC) with one hemiuterus and vagina on each side in the pelvis. In one patient, a Meckel's diverticulum was present 5 cm from the ileocecal junction. In both girls, a diverting proximal ileostomy was the initial surgery.