Distal Atresia Research Articles

An Unusual Case Of Ileal Atresia: A Case Report

Background: Congenital cardiovascular abnormalities are an important cause of morbidity and mortality in the pediatric age group. GI system problems are not uncommon in patients with congenital cardiovascular abnormalities. These GI problems include both that are associated with the congenital defect, or they may occur due to cardiopulmonary bypass use to treat these problems, which can cause a redistribution and thus a decrease in blood flow leading to ischemia. Case: Male child, 25 days of life, was antenatally diagnosed to have a congenital heart defect, which on post-natal ECHO was diagnosed as Mixed Anamolous TAPVC with an Anterior Muscular Ventricular Septal Defect (VSD). After the TAPVC re-routing correction was done, on post-op day 3, the child developed abdominal distension and a pediatric surgical referral was taken. On examination, the child had fever, tachycardia and sluggish bowel sounds. Imaging suggested ileus which was managed conservatively. Patient was later started on oral feels which were not tolerated well so an exploratory laparotomy was planned which revealed distal ileal atresia. The anomaly was corrected in the same surgery and post-op course was uneventful. Conclusion: To summarize everything that has been stated so far, in neonates even with the history of stool passage, ileal atresia should still be considered as one of the differentials, particularly after the common ones have been ruled out.

Distal ureteral atresia – an embryological dilemma

Introduction: Distal ureteral atresia is a rare urinary tract anomaly generally associated with ipsilateral renal dysplasia and abnormalities such as multicystic dysplastic kidney, hydronephrosis and megaureter in the contralateral kidney. Despite burgeoning investigation modalities, definitive preoperative diagnosis of this condition is rarely feasible, also the embryological disarray of events that result in the development of this anomaly and the associated malformations is not clearly understood. Case presentation: We hereby report two cases of distal ureteral atresia and discuss the diversity in their presentations, diagnosis, atypical associations and management and review the possible embryological mal-development. Conclusion: Distal ureteral atresia with urogenital sinus as in Case 1 has not been documented so far and a plausible embryological explanation is deduced regarding its occurrence. The course of the affected kidney following timely and adequate relief of obstruction in Case 2 is depicted, highlighting the eventual management. Level of evidence: Not applicable

A Comprehensive Review of Intestinal Atresias

A Comprehensive Review of Intestinal Atresias

Proximal ureteral atresia in a duplex kidney with preserved renal function: A case report and review of literature

Ureteral atresia is a rare congenital anomaly and is usually associated with renal dysplasia. Distal atresia is more common and other associated urinary anomalies are rare. We report a case of an eight year old boy who presented with left flank pain and vomiting. Ultrasound abdomen and pelvis and renal scinitigraphy were suggestive of hydro-ureteronephrosis with obstructed drainage. He was suspected to have ureteric stricture and taken up for surgery. Cystoscopy and retrograde pyelogram revealed an incomplete duplication with lower moiety ureter ending blindly. Exploration of left renal fossa showed a duplex anomaly with normal upper moiety and hydronephrosis of lower moiety with an ureteric stump. Ureteropelvic anastomosis was done between the dilated renal pelvis and the blind ending ureter. Patient was doing well post operatively with good function in both moieties. Review of literature shows only two previously reported cases of proximal ureteric atresia. Preservation of renal function in an older child has also been reported only once. Ureteral atresia is usually diagnosed intra-operatively and various reconstruction options are available in patients with preserved function.

Nutritional role of amniotic fluid: clues from infants with congenital obstruction of the digestive tract

AimsTo investigate the role played by amniotic fluid in late fetal nutrition by analysis of infants born with digestive tract atresia.MethodsBirth weight (BW), gestational age and gender of infants born...

Cautery ablation of a remote recurrent tracheoesophageal fistula

Abstract Complications subsequent to repair of a congenital tracheoesophageal fistula (TEF) with esophageal atresia (EA) include leak at the anastomosis, stricture formation, and recurrent fistula. Recurrence is uncommon, and the optimal management for a recurrent fistula is debatable. As many of the symptoms of TEF recurrence are similar to common postoperative sequelae, diagnosis of these recurrences can be quite challenging. We report the case of a 12 year old patient with a history of Type B EA/TEF (proximal pouch fistula, distal atresia) who was discovered to have a TEF recurrence over a decade after his initial repair which was successfully treated with cautery ablation of the fistula tract.

Spontaneous pneumothorax in a neonate with TEF and double oesophageal atresia. A case report

Symptomatic Spontaneous pneumothorax is uncommon disease scenario in term neonates. But pneumothorax can occur in neonates who had CPR or are on mechanical ventilator for different causes of respiratory distress. Congenital double esophageal atresia is a very rare type of esophageal atresia. If only single membranous atresia it can be isolated esophageal membranous atresia or with trachea-esophageal fistula. This type of atresia was not mentioned in gross or Vogt classification. Previously, only a few cases with an obstructing web had been reported and also multiple esophageal atresias. But there is no any documented case report with TEF & multiple esophageal atresia. The choices of treatment vary from ligation of the fistula with excision of the membrane by esophagotomy and/or gastrotomy, thoraco-abdominal approach and end to end anastomoses of the proximal & distal atresia. Here we present a case of a neonate who had developed respiratory distress since birth with a cause of tension spontaneous pneumothorax and managed by a chest tube and also diagnosed to have oesophageal atresia with distal tracheo-esophageal fistula on further evaluation and showing additional distal membranous oesophageal atresia during the thoracotomy. And he was managed accordingly. Keywords: Spontaneous Pneumothorax, esophageal atresia, Membranous atresia, TEF

Application of Bishop-Koop stoma in refractory congenital intestinal atresia

To explore the feasibility and safety of Bishop-Koop stoma procedure in the treatment of neonates with refractory congenital intestinal atresia. Clinical and follow-up data of 25 neonates with refractory congenital intestinal atresia undergoing Bishop-Koop stoma procedure in our center from January 2011 to December 2014 were retrospectively analyzed. Of 25 neonates, 13 (52%) were male, 12(48%) were female, the birth weight was 1600-3800 g (mean 2920 g), the age of admission was 10 hours to 20 days, and the age of operation was 1-58 d (mean 7 d). Diameter ratio of proximal atresia intestine to distal atresia intestine was all greater than 4. Eleven cases(44%) were high jejunal atresia, 3 cases(12%) type III( b, 7 cases(28%) type IIII(, 14 cases(56%) were identified as complex meconium peritonitis, and 3 cases (12%) received reoperation. All the cases completed their Bishop-Koop stoma operations successfully with median operative time of 3 (1.2-4.5) hours and median intra-operative blood loss of 3.5(1-18) ml. The postoperative complication rate was 20%(5/25), including 3 cases of cholestasis, 1 case of ileus, and 1 case of neonatal necrotizing enterocolitis with septicemia who died 6 days after operation resulting in the mortality of 4%. Besides, 1 case gave up treatment because of economic reason. For the rest 23 neonates, the median first feeding time was 11 days and mean time was 11(5 to 20) days; the median time of postoperative total parenteral nutrition (TPN) was 15 days and mean time was 21 (5 to 68) days; the median hospital stay was 33 days and mean hospital stay was 25(12 to 81) days, respectively. Two-stage stoma closure operations were performed in all the 23 cases afterwards and no postoperative associated complications were found. When discharge after Bishop-Koop stoma operations, Z score of body weight was normal in 3 cases(13.0%) and lower than normal in 20 cases(87.0%), while in hospitalization for stoma closure, Z score of body weight was normal in 19 cases(82.6%) and lower than normal in 4 cases (17.4%). Of 23 cases, serum albumin level was normal in 9 cases(39.1%) before operation, in 3 cases (13.0%) when discharge and in 22 cases(95.7%) in hospitalization for stoma closure. Bishop-Koop stoma procedure is safe and feasible in the treatment of neonates with refractory congenital intestinal atresia, and can obviously improve the nutritional status.

Complexities of Müllerian Anatomy in 46XX Cloacal Exstrophy Patients

Study ObjectiveTo characterize Müllerian anatomy in 46,XX cloacal exstrophy patients. DesignRetrospective review of prospectively maintained, institutionally approved exstrophy-epispadias-cloacal exstrophy database. SettingTertiary care, high-volume exstrophy center (Division of Pediatric Urology, The Johns Hopkins Hospital, Baltimore, Maryland). ParticipantsWe included 31 patients who were genetically female with cloacal exstrophy for whom records included detailed evaluation of Müllerian anatomy. InterventionsNone. Main Outcome MeasuresMüllerian structures, method of evaluation, management, and sexual activity. ResultsOf our patients, 12.9% (3/31) had no identified abnormalities. Vaginal anatomy was described for 30 patients; 3/30 had vaginal agenesis, 14/30 had a single vagina, and 13/30 had vaginal duplication. Of 14 patients with 1 vagina, 5 had atresia/hypoplasia, and 1 had a lateral displacement. One patient with 2 vaginas also had distal atresia. Of the cervices evaluated, 9/14 were duplicated (2/9 with a solitary vagina), and 19/27 of the uteri were duplicated (6/22 with 1 vagina, 1/22 with no vagina). Five patients required imaging to fully characterize their anatomy, and 7 patients had studies that failed to identify Müllerian structures seen in the operating room or on physical examination. Common reconstructive surgeries included vaginoplasties, incisions of vaginal septa, colporrhaphies, and hysterectomies. Sexual activity was confirmed for 3 patients, 2 of whom had conceived. ConclusionMost female cloacal exstrophy patients exhibit abnormalities of the Müllerian system. Axial imaging and ultrasound are helpful diagnostic adjuncts but do not replace careful physical examination and assessment in the operating room. Further studies of sexual activity and fertility are warranted.

P17.06: Fetal rectal prolapse secondary to distal colic atresia

P17.06: Fetal rectal prolapse secondary to distal colic atresia

PO-0911 Herlyn-werner-wunderlich Syndrome And Distal Vaginal Atresia. Two Particular Cases

Background and aims Mullerian anomalies are rare, causing malformations of the female reproductive system as the Herlyn-Werner-Wunderlich Syndrome (HWWS) and distal vaginal atresia. These patients are usually asymptomatic until menarche. There may be associated with renal and urinary tract anomalies. We describe two cases of young girls with occluded vagina associated with urethrovaginal fistula. Cases presentation The first patient is a 3-years-old asymptomatic girl with prenatal diagnosis of left renal agenesis. Ultrasonography, performed 6 months prior to consultation, identified an anechoic cyst in hypogastrium. On genital examination, distal vagina occluded by a protruding membrane over the vaginal introitus. Magnetic resonance revealed a left paravesical formation suggestive of hydrocolpos. By the transmembrane puncture, urine was aspired. Laparoscopy confirms uterus didelphys. Membrane resection enable two hemivaginas. Based on the association of these anomalies, the diagnosis of HWWS was made. Girl present constant loss of urine and cystoureterography performed reveals urethrohemivaginal fistula. The second patient is a 4-years-old girl with occluded vagina suggesting distal atresia. Saline injected through the membrane eliminates by urethra. Cystoureterography confirms urethrovaginal fistula. Conclusions The diagnoses of HWWS (uterus didelphys, unilateral low vaginal obstruction and ipsilateral renal agenesis) also known by OHVIRA and distal vaginal atresia in infancy and early childhood are unusual. The reported cases represent examples off premenarche diagnosis. In both patients, we detected urethrovaginal fistula, association not reported before in literature. Early diagnosis allows appropriate therapeutic management and prevents subsequent complications.

Congenital Pyloric Atresia with Distal Duodenal Atresia- Role of CT Scan

The mainstay of diagnosis of congenital pyloric atresia is by plain X-ray of the abdomen showing a large gas bubble with no gas distally. But very rarely it can be associated with distal duodenal atresia when the baby may present as lump abdomen. In such a situation apart from the X-ray, another radiological investigation is needed to delineate the exact nature of the lump. Since the role of ultrasonography is limited in intestinal pathologies and contrast studies are not informative in atresias, the CT scan is the ideal choice. We had managed a case of pyloric atresia with similar presentation with preoperative CT scan.

Gastroschisis associated with an omphalocele and intestinal atresia

An omphalocele and gastroschisis represent two common pediatric congenital abdominal wall defects, but are rarely associated with each other. An omphalocele results from an incomplete closure of the lateral body folds leading to a midline defect in the anterior abdominal wall. This manifests as herniated intra-abdominal contents into a membrane-covered sac, made up of an inner peritoneal and outer amniotic layer [1, 2]. With an incidence of 1 in 3,000 births, an omphalocele has a higher prevalence in males and is associated with chromosomal abnormalities in up to 40 % of patients [2]. On the other hand, a gastroschisis is a congenital abdominal wall defect that typically occurs to the right of the umbilicus and may be separated by a skin bridge [3]. The incidence is 0.3–1 per 10,000 births with associated anomalies present in up to 10 % of cases [3, 4]. Distal atresias, with a normal incidence of 1 in 1,500–5,000 births, represent one such anomaly with the ileum most commonly affected. We present a case with all three congenital anomalies, a finding we believe has never been reported. Case report

Congenital leiomyoma of the distal ileum associated with ileal atresia: A case report

Association of distal ileal atresia with leiomyoma is a quite rare condition. Herein, we describe a newborn who underwent surgery for ileal atresia and was found to have leiomyoma on pathological examination in the atretic segment. The presence of leiomyoma may indicate a malformation during recanalization and could play a role in the etiology of intestinal atresia. Thus, some cases of atresia may be due to tumor (leiomyoma) in the bowel wall.

Is Minimal Access Surgery of Esophageal Atresia with Distal Esophageal Atresia by Thoracoscopy is better than Conventional Thoracotomy? A Multi-institutional Review of Literature to get the Answer

Is Minimal Access Surgery of Esophageal Atresia with Distal Esophageal Atresia by Thoracoscopy is better than Conventional Thoracotomy? A Multi-institutional Review of Literature to get the Answer

Is Minimal Access Surgery of Esophageal Atresia with Distal Esophageal Atresia by Thoracoscopy is better than Conventional Thoracotomy? A Multi-institutional Review of Literature to get the Answer

Topic: Is minimal access surgery of esophageal atresia with tracheoesophageal fistula by thoracotomy better than conventional thoracotomy? A multi-institutional review of literature. Objective: Minimal access surgical technique has been one of the most important surgical advances in the last few decades; we have reached now in such era that complex neonate surgical issue can be addressed safely by minimal access surgery without significant morbidity. Esophageal atresia (EA) with distal tracheoesophageal fistula (TEF) has been successfully treated by traditional thoracotomy, but now the trend has been shifted toward minimal access surgery via thoracoscopic repair of EA with distal EA. The quest of this multi-institutional review is to get the answer that is minimal access surgery is better than the traditional open approach. Materials and methods: A literature view was performed from 2005 to 2012 using the PubMed, science direct, OVID search EBSCOhost and search engines Google and Yahoo. The following search terms were used, thoracoscopic repair or thoracoscopic surgery, thoracotomy and EA. Inclusion criterion is EA with distal esophageal fistula with comparative study by open thoracotomy or by historical data. Exclusion criteria were other esophageal anomalies. Results: In 182 patients operated by minimal access surgery by thoracoscopy, the mean gestational age, weight, associated congenital anomalies, mechanical ventilation, perioperative pCO2, postoperative early and late complication are comparable with historical open thoracotomy. However MAS has a superadded advantage in markedly reduction in scar tissue, postoperative pain and no chest wall deformity. Conclusion: This multi-institutional review provides a recent comparison of the approached to EA with TEF without any worse effect of thoracoscopy and competes well with traditional open thoracotomy approach. There is dramatic advancement of pediatric MAS over the last decade and the result are comparable with open thoracotomy in perioperative, postoperative and long-term outcome with potential advantages of less scar tissue, less postoperative pain, less disruption of anatomy and function and better cosmoses with markedly reduced musculoskeletal complication. Thoracoscopic repair is a promising adjunct, but there are difficulties for setting it as the open thoracotomy and it still needs more subjective studies with the consideration of learning curve and long surgical time. However, thoracoscopic repair of EA with TEF is a favorable and effective procedure with good prognosis.

Familial distal foregut atresia in a family with likely autosomal dominant inheritance pattern

Familial occurrence of distal foregut atresia (DFA) (Type 1) is rare. Diagnosis is based upon the clinical symptomatology and confirmed by radiological studies, surgery and histology. A number of reports have described families in which several family members have been involved and suggested an autosomal recessive mode of inheritance. Little is known about the underlying genetic causes or indeed the likely pathogenic mechanism. We report a family in which there are five affected cases including three siblings where the DFA appears to be inherited in an autosomal dominant inheritance pattern with reduced penetrance.

Laparoscopic-assisted perineal pull-through vaginoplasty

Hematometrocolpos is the result of vaginal obstruction and can become an emergency in the pubertal period. The treatment of imperforate hymen is well defined, but the treatment of vaginal atresia is more complex. We report a case of hematometrocolpos secondary to distal vaginal atresia that was operated on in the pubertal period. The patient had isolated distal atresia without persistence of the urogenital sinus. A combined abdominal laparoscopic and perineal approach and a posterior vaginoplasty were carried out. Finally, an abdominoperineal pull-through was successfully performed. Neovagina was successfully developed with this method. The embryology and literature are reviewed. The classification, indications, and surgical technique are discussed. We suggest that this procedure is suitable for patients with vaginal agenesis and a normal uterus.

Use of a microporous polytetrafluoroethylene catheter balloon to treat refractory esophageal stricture: a novel technique for delivery of mitomycin C

PurposeEndoluminal application of mitomycin C shows promise as a nonsurgical approach to treating recalcitrant stricture but requires precise delivery to prevent mitomycin-mediated injury to adjacent normal mucosa. We describe a novel technique that uses a microporous polytetrafluoroethylene catheter balloon to endoluminally deliver mitomycin C to the target tissue while minimizing nontarget drug application. Materials and MethodsA newborn infant with proximal tracheoesophageal fistula and distal atresia underwent an uncomplicated repair. However, he developed recurrent esophageal stricture resistant to multiple attempts at pneumatic dilations. An image-guided endoluminal radiologic approach that uses microporous polytetrafluoroethylene catheter balloon was developed to precisely deliver mitomycin C to the mucosal lining of the stricture post-dilation. ResultsAfter uncomplicated pneumatic dilation under fluoroscopic guidance, we used a microporous balloon catheter to endoluminally deliver mitomycin C topically to the mucosa at the level of stricture. Three weeks post procedure, repeat esophagram showed resolution of the stricture with unobstructed flow of contrast material to the stomach. The patient had no observable side effects from mitomycin C application. ConclusionImage-guided therapies based on balloon dilation and drug-eluting microporous balloon techniques offer a safe, precise, and comprehensive approach to the treatment of recalcitrant esophageal strictures.

Comparative outcomes in intestinal atresia: a clinical outcome and pathophysiology analysis

To describe the outcomes of 130 intestinal atresias between 1982 and 2007. Records were analyzed for location, demographics, prenatal diagnosis, birth weight, associated anomalies, surgery, establishment of oral intake, re-interventions and mortality. Statistical analyses were performed using Fisher test and ANOVA. There were 59 duodenal (30 male), 63 jejuno-ileal (34 male) and 8 colonic atresias (3 male). Prenatal diagnosis was established in 27 (46%) duodenal (DA), 26 (41%) jejuno-ileal (JIA) and 1 (12.5%) colonic atresias (CA). The mean birth weights, 2,380.5 g (SD 988) DA, 2,814 g (SD 755) JIA and 3,153 g (SD 527) CA were significantly different (p = 0.011). The mean gestational ages were 36, 37 and 37 weeks in DA, JIA and CA, respectively (p-NS). Associated congenital anomalies were seen in 41 (76%) DA, 32 (52%) JIA and 3 (38%) CA (p = 0.08, NS). The median time to full oral feeds after surgery was 18 days in DA, 20 days in JIA and 15.6 days in CA, respectively (p > 0.05). Eight patients with DA and nine patients with JIA underwent repeat surgery for adhesive obstruction. Adhesive bowel obstruction was most common in the first year after surgery in both groups (15/17). Gastroschisis was seen in six (10%) of JIA and three (35%) of CA. Two patients in the JIA group underwent bowel lengthening. Patients with gastroschisis and those with associated anomalies needed prolonged duration of TPN after JIA correction. There was no mortality in the duodenal atresia and colonic atresia groups. Six patients in the JIA group died, three of severe atresias coupled with multiple anomalies and three of cholestasis and sepsis. Distal atresias are difficult to diagnose antenatally. Proximal atresias have a significantly lower birth weight than distal atresias. Associated anomaly screening is important in all atresias.