H-type Fistula Research Articles

H-type fistula in a newborn with hypoxic respiratory failure.

H-type fistula in a newborn with hypoxic respiratory failure.

Worth the Wait? The Impact of Timing of Repair of Esophageal Atresia With Tracheoesophageal Fistula on Outcomes

IntroductionInfants with esophageal atresia and tracheoesophageal fistula (EA/TEF) are at increased risk for respiratory compromise and gastric perforation until fistula ligation. We sought to describe current practice regarding the timing of EA/TEF repair and hypothesized that age at repair is a predictor of adverse outcomes. MethodsThe Pediatric Health Information System (PHIS) database was used to identify patients with EA/TEF who underwent fistula ligation and esophago-esophagostomy at US children's hospitals from July 2016–June 2021. Patients with a repair >10 days of age, a long-gap atresia, or H-type fistula were excluded. Comorbidities including prematurity and operative congenital heart disease were noted. Outcomes including anastomotic leak, gastric perforation, and post-operative respiratory failure were assessed for association with age and day of the week of operation. ResultsAmong 863 patients that were evaluated, the plurality of operations was on DOL 2 (36%) and 83% were on a weekday (random rate = 71%). Later operations had shorter LOS (p = 0.04) and more recurrent nerve injuries (p = 0.01). Weekend repairs were associated with equivalent outcomes. Gastric perforations occurred in 18 (2.0%) patients; 11 (61%) of these occurred after DOL 2. ConclusionsWe found no significant differences in outcomes other than more recurrent nerve injury and decreased LOS with EA/TEF repair at older ages. Although repair beyond DOL 2 was safe from a respiratory standpoint, most gastric perforations occurred after this point. In the absence of contraindications or significantly reduced weekend capabilities, we recommend repair of EA/TEF by DOL 2. Level of EvidenceIII.

Редкий вариант аноректальной мальформации: Н-фистула при нормально сформированном анусе

H-type rectovestibular fistula with a normal anus refers to rare types of anorectal malformation in both girls and boys. The clinical picture of the girls is characterized by leakage gas and stool through the vagina. The first clinical manifestations occur mainly in infancy. Objective. To describe the clinical observation of an H-type rectovestibular fistuls with a normal anus in a girl with the successful surgery of transanal excision of the fistula. At the age 45 days, the baby's mother noticed the leakage stool through the vagina. When examining the perineum under anesthesia, the child revealed an H-type rectovestibular fistula with a normal anus. Surgery was performed – transanal excision of the fistula with mobilization of the anterior wall of the rectum and closure of the defect. She was discharged from the hospital on the 10th day after the operation. At the control examination after 6 months, the stool is regular, there are no pathological secretions from the vagina, the cosmetic result is good. Conclusion. The advantage of transanal excision of an H-type rectovestibular fistula with the closure of the defect by the rectal wall is the absence of a perineal incision and the need for preventive colostomy, which allows you to maintain a normal appearance as much as possible and achieve a good cosmetic result. Key words: rare anorectal malformation, H-type rectovestibular fistula, transanal approach

Management of a double H-type tracheoesophageal fistula without esophageal atresia: a case report

BackgroundDouble congenital tracheoesophageal fistula (TEF) without esophageal atresia, known as double H-type fistula, is an extremely uncommon malformation. Due to its nonspecific symptoms and the small size of the fistula in infancy, H-type tracheoesophageal fistula is rarely diagnosed in the neonatal period and is also challenging to repair.Case presentationWe report a rare case of double H-type tracheoesophageal fistula in a 2-day-old newborn, identified with a time-lapse and repaired separately. The diagnosis was established through repeated bronchoscopy, with methylene blue injected through an esophagoscope. A right thoracotomy was performed to undertake the surgical repair after cannulating the fistula with a vascular guide wire. Five months later, the infant, who had a history of recurrent episodes of coughing and shortness of breath, was diagnosed with acute respiratory distress, and a second TEF was detected by bronchoscopy. A cervical incision was used to repair the second H-type fistula.ConclusionsDouble H-type tracheoesophageal fistula should be considered while correcting a tracheoesophageal fistula, especially if the symptoms continue after the first operation. Repeated radiological contrast studies and bronchoscopy with methylene blue injected through an esophagoscope help the early diagnosis.

Treatment of pediatric H-type rectovestibular fistula with tension-free repair of rectal seromuscular layer

ObjectiveThis study aimed to assess the outcomes of a 7-year experience with tension-free repair of the rectal seromuscular layer for H-type rectovestibular fistula in female children with a normal anus. MethodsBetween May 2016 and January 2023, 86 patients with H-type rectovestibular fistula underwent a standardized surgical procedure conducted by the same surgical team. The procedure involved: (1) Identifying and locating the vestibular orifice. (2) Dissecting the fistula. (3) Dissecting the anterior rectal wall. (4) Repairing the fistula. (5) Closing the internal opening of the fistula. During the dissection of the rectovaginal septum, care was taken to avoid any harm to the posterior wall of the vagina while exposing the anterior rectal wall by 10–25 mm. ResultsFollow-up, conducted via telephone or outpatient visits, ranged from 3 months to 6 years and 11 months (median, 3.5 years). In 82 cases (95.35% of 86), primary healing was achieved, resulting in satisfactory perineal appearance, smooth stool passage, and regular defecation. In 4 cases (4.65% of 86), fistula recurrence occurred within 5 to 10 days post-surgery. One case healed within 3 weeks with 3% boric acid sitz baths. The other 2 cases underwent debridement 7 days after the initial operation, leading to successful recovery. The final case experienced a recurrence 1 year after surgery following resection and repair of the perineal fistula, and no further surgical intervention was pursued. ConclusionTension-free repair of the rectal seromuscular layer represents a straightforward, safe, and effective surgical approach for managing H-type rectovestibular fistula with a normal anus in female children.

Isolated tracheo-esophageal fistula in children: Analysis of a large multicentric series and proposal of a comprehensive treatment algorithm

BackgroundIsolated tracheo-esophageal fistulas (TEF) are rare and challenging, possibly leading to severe complications. We evaluated the outcomes in a large series of patients treated in 9 referral Centers for airway surgery, and suggested an ideal management algorithm. MethodsA multicentric retrospective study on TEF was performed. Results186 cases were collected. Among 101 patients with recurrent TEF after EA repair, 1 was treated successfully with thoracoscopy, 23 with open surgery with TEF division and tissue interposition, with 15 complete resolution (65 %), 2 TEF recurrence (9 %) and 6 other complications (26 %), and 77 received TCA endoscopic cauterization, with complete resolution in 61 (79 %). Among 54 patients with congenital H-type fistula, 1 received conservative treatment with resolution; 4 underwent thoracoscopy (50 % resolution); 6 had endoscopic cauterization (33 % resolution); 44 received open surgery, with 43 (98 %) successes. In the last group, the majority of TEF was ligated and divided with tissue interposition. Among 26 post-traumatic fistulas (mechanical trauma, battery or caustic ingestion), 6 patients received endoscopic procedure and 5 of them (83 %) reached complete resolution. The other 19 received open surgery (trans-tracheal direct FTE closure with tracheal resection), and 12 had complete resolution (63 %). Two patients eventually died and complication rate was 23 %. Other 5 cases (3 Bronchial-Esophageal Fistulas and 2 TEF after cleft repair) were treated endoscopically or with open approach. ConclusionEndoscopic cauterization can be the preferred treatment for recurrent TEF after EA, while for the congenital H-type and post-traumatic TEF the open approach remains our first choice.

Endoscopic treatment in pediatric patients with recurrent and H-type tracheoesophageal fistulas - A systematic review and meta-analysis.

Endoscopic treatments for managing recurrent tracheoesophageal fistula (rTEF) and H-type TEF are being utilized lately; however, the preferred technique is yet to be determined. We aimed to systematically review existing publications on endoscopic treatment of rTEF and H-type TEF to analyze their success and complication rates. PRISMA guidelines were followed. MEDLINE, EMBASE, CINAHL and the Cochrane Central Register of Controlled Trials were comprehensively searched in accordance to a priori developed protocol, from 1975 until 2020. English, Spanish and German papers were included. Studies were independently screened and analyzed by two reviewers. 84 full texts were assessed for eligibility out of 581 screened studies, of these, 39 studies with 127 patients were eligible for inclusion (115 rTEF and 12H-type TEF). All included studies were cases reports or case series. Overall success rate was 45% with sealant injection, 87% with de-epithelialization and 80% with sealant injection and de-epithelialization combined. The mean number of required treatments for success was 1.9 (range 1-6). Mixed effect model meta-analysis of case series with n>1 showed that sealant injection had a significantly lower success rate of 50% (95% CI 1-99%, I2 72%) compared to de-epithelialization 90% (95% CI 72-99%, I2 27%), p=0.007 and the combination of both techniques 87% (95% CI 68-99%, I2 11%), p=0.02. Nine patients (7%) had transient respiratory distress. No mortalities reported. Endoscopic treatment for rTEF and H-type fistula is a minimally invasive technique with favorable outcome and considerably less morbidity compared to open surgery, suggesting it as a safe and effective first line treatment option. Repeated endoscopic treatment attempts can be expected to obtain complete closure. De-epithelization techniques with or without combined tissue adhesive injection had significantly better results than sealant injection techniques alone.

Gastro-esophageal and respiratory morbidity in children after esophageal atresia repair: a 23-year review from a single tertiary institution in Asia

BackgroundImproved survival of neonates with esophageal atresia with/without tracheoesophageal fistula (EA/TEF) has resulted in increased prevalence of gastro-esophageal and respiratory-related morbidities. However, long-term outcome data on these patients remains limited, making it difficult to substantiate any guidelines on their chronic care. The purpose of our study is to report on their post-operative outcomes especially the long-term gastro-esophageal and respiratory morbidities.MethodsThis was a retrospective review of 65 patients (39 males, 26 females) who underwent EA/TEF repair from 1996 to 2019 at a single tertiary institution. Follow up data pertaining to clinical characteristics, operative management and long-term gastro-esophageal and respiratory morbidities was analyzed.ResultsFifty-seven patients (87.7%) had Gross Type-C anatomy, followed by 5(7.7%) patients with Type-A, 1(1.5%) with Type-B and 1 with Type-D. One patient had a late diagnosis of H-type fistula (Type-E). Thirteen (20%) patients had long-gap EA.Median age at first surgery was day 1 (IQR 1–2) of life. All patients underwent bronchoscopy at their index surgery. All 52 non-long gap EA (LGEA) patients underwent primary anastomosis, while most (76.9%) LGEA patients underwent staged repair. Post-operatively, 4(6.2%) developed anastomotic leak which resolved with conservative management. Three (4.6%) had recurrent TEF, 2 underwent re-do ligation. Twenty (30.8%) patients developed anastomotic strictures, with 15 requiring serial dilatation.Long-term burden of gastro-esophageal and respiratory morbidity was high (63.1%; 64.6% respectively). The majority (n = 39,60%) of patients required active follow-up for a median duration of 5 years (IQR 1.5–10 years). Predominant conditions were gastroesophageal reflux disease (n = 28, 43.1%), dysphagia (n = 20, 30.8%), recurrent respiratory infections (n = 23, 35.3%), chronic cough (n = 19, 29.2%), and pneumonia (n = 19, 29.2%). Tracheomalacia was diagnosed in 22(33.8%), 2 of whom required tracheostomy for severe disease. Overall mortality rate was 10.8% (n = 7): 5 demised due to chronic respiratory failure, while 2 demised intra-operatively during the index surgery.ConclusionDespite successful surgical repair for EA/TEF, our data demonstrated significant morbidities among EA/TEF survivors, thus highlighting the importance of long-term multi-disciplinary care with collaboration between respiratory, gastroenterology, and otolaryngology specialists.Level of evidencePrognostic, Level IV.

A systematic review of the thoracoscopic treatment of H-type congenital tracheoesophageal fistulas

A systematic review of the thoracoscopic treatment of H-type congenital tracheoesophageal fistulas

The Outcome of Single Stage versus Two Stage Repair in Anorectal Malformations with Recto-Vestibular Fistula

Background: Imperforate anus with recto-vestibular fistula is one of the most common anorectal malformations in females. Wound infection and disruption after recto-vestibular fistula repair may affect the fecal continence and functional outcome. Fecal incontinence may cause long term social, economical and psychological problems in children. Although a protective colostomy reduces the infectious complications and dehiscence, it is also associated with many problems. Aim: To compare the safety, feasibility, post operative complications and functional outcomes of limited posterior sagittal anorectoplasty with or without colostomy. Study design: Prospective descriptive study. Place and duration of sudy: Department of Pediatric Surgery, Sahiwal Teaching Hospital Sahiwal, from 1st January 2019 to 31st December 2021. Methodology: Forty-one patients with congenital recto-vestibular fistula were managed by two techniques. Patients were divided into two groups. Group A (19 patients): were operated by single stage limited posterior sagittal anorectoplasty without covering colostomy. Group B (22 patients): were operated by two stage technique. Limited posterior sagittal anorectoplasty and covering colostomy in first stage and stoma closure was done in second stage. The patients in both groups were evaluated for hospital stay, operation time, postoperative complications, fecal continence, constipation, bowel function. Results: The age of patients ranged from 11-56 months (mean 26.93 months) in Group A and 9-60 months (mean 27.03 months) in Group B. The total length of hospital stay in Group A ranged from 7-11 days (mean 9.20 days); however, in Group B, it ranged from 4-6 days (mean 5.01 days) for first stage procedure and 8-11 days (mean 9.27 days) for second stage colostomy closure procedure. In Group A, complications were, wound infection 03 (15.78%), anal stenosis 02 (10.52%), mucosal prolapse 01 (5.26%), constipation 04 (21.05%), soiling 04 (21.05%), perineal excoriation 03 (15.78%) and recurrent H-type recto-vestibular fistula 01 (5.26%). In Group B, during the first stage of repair, one patient (4.54%) developed wound infection. Mucosal prolapse was observed in one patient (4.54%). Constipation was reported in 03 (13.63%) and soiling in 01 (4.54%). Peri stoma skin excoriation was seen in five patients (22.72%). Stomal prolapse was observed in two patients (9.09%). Two patients (9.09%) developed wound infection after colostomy closure. Conclusion: Single stage repair of congenital recto-vestibular fistula increases the risk of postoperative complications. Two stage approach is associated with less postoperative complications. However it is associated with lengthy hospital stay, long operation time and complications related to stoma formation and closure. Keywords: Recto-vestibular fistula, Limited posterior sagittal anorectoplasty, Single stage technique, Two stage technique.

Preoperative flexible bronchoscopy-aided cannulation of a neonatal H-type tracheo-oesophageal fistula assists intraoperative identification of the fistulous tract

BackgroundThe surgical repair of H-type tracheo-oesophageal fistulas situated below the level of the second thoracic vertebra requires an open thoracotomy or a thoracoscopy. We describe a novel technique that allows for the use of a cervical incision to repair a fistula situated in the thorax, thus diminishing surgical risk.Case presentationIn this report, we describe a 3-day-old term baby with an H-type tracheo-oesophageal fistula where flexible bronchoscopy and gastroscopy were used to cannulate the fistula with a soft ureteric catheter. This allowed for it to be tractioned into the cervical region where it was surgically dissected and isolated. There was no need for re-intervention in the first 3 months after surgery.ConclusionFlexible bronchoscopy-aided cannulation of H-type fistulas can assist in intraoperative identification of the fistulous tract as well as help traction it into a surgically more accessible area like the cervical region.

Recto-vestibular fistula with colonic duplication: report of three cases and review of literature

BackgroundAnorectal malformations associated with colonic duplications are a rare condition. Double terminations of tubular colonic duplication in the perineum are even rarer. Colonic duplication may have different presentations according to its location and size and incidence of less than 15% of all gastrointestinal duplications. We hereby report three such cases of recto-vestibular fistula along with underlying tubular colorectal duplication over a period of 10 years (2010–2020) managed successfully by different surgical approaches. These cases also illustrate the diagnostic challenge and variable presentation of colonic duplications in female patients with recto-vestibular fistula.Case presentationCase 1 is a patient with flank heteropagus with absent anal opening and recto-vestibular fistula, while cases 2 and 3 presented as suspected H-type recto-vestibular fistula with normal anal opening. All the cases were however managed with a specific surgical approach on case basis. Duplicated colon was excised in case 1, but in case 2 and case 3, duplicated colon was converted into single channel using proximal and distal staplers. Further definitive repair of female recto-vestibular fistula was done.ConclusionsColorectal duplication is a rare congenital malformation, and their association with anorectal malformations is even rarer. This association can have varied presentation, and surgical approach should be according to the congenital anatomical variations on case-to-case basis. Colonic duplications should always be ruled out in a female newborn child with history of passage of stools both from the vestibule and anus.

Congenital H-type ano-vestibular fistula with normal anus

H-type ano-vestibular fistula with a normal anus is a rare type of anorectal malformations in female children. Although most cases are considered to be congenital, some authors suggested that this was an acquired condition. There is a high incidence of associated anomalies reaching up to 20–60% of the cases. The most common anomalies are anal stenosis, ectopic anus, cardiac and urological malformations. Many surgical techniques have been developed since the first description of this condition by Bryndorf in 1960. However, there is no consensus regarding the optimal surgical procedure due to the low number of cases reported in the literature. After reviewing these surgical techniques and their outcome, we can conclude that the main principles for a good repair are good preoperative bowel preparation, complete excision of the fistulous tract, good closure of the intervening tissue between the rectum and vagina in layers, avoidance of apposing suture lines and good post-operative nursing to keep the wound clean and dry as much as possible.

H-type Rectourethral Fistula with Anal Stenosis in a Male Patient: A Case Report with Literature Review

Background: H-type rectourethral fistula is a rare variant of anorectal malformation that affects male neonates. It is usually associated with anal stenosis but with a normal anal position and shape, so it is difficult to be diagnosed early after birth. We present this case to evaluate the functional outcome of this rare anomaly in a male patient and compare it with other reported cases with different ages at diagnosis. Case Presentation: A nine-month-old infant presented with recurrent attacks of urinary tract infection and failure to thrive. After proper evaluation, the diagnosis of an H-type rectourethral fistula with anal stenosis was confirmed, and a diverting double-barreled colostomy was performed as an initial procedure. Posterior sagittal anorectoplasty with ligation and division of fistula was successfully done, followed by colostomy closure. Now the patient is five years old and is continent for urine and stool. Conclusions: The diagnosis of H-type rectourethral fistula with anal stenosis is challenging to physicians, and a high index of suspicion is required to reach the diagnosis as the anal opening had a normal shape and location. If this condition is diagnosed and treated early, these patients will have an excellent functional prognosis, as the muscle complex and anal sphincter are well developed.

Laparoscopic management of a congenital H-type rectovaginal fistula in an infant

Congenital H-type rectovaginal fistula with a normal anal opening is a rare malformation. The authors encountered such a case in a 2-month-old girl. Both direct inspection and fluoroscopic examination did not identify the location of the fistula, but passage of contrast media through the vagina was observed. Using a 3-port transperitoneal laparoscopic technique, complete resection of the fistula was successfully performed. Without a protecting colostomy, laparoscopic approach appeared to be safe and feasible in an infant with an H-type rectovaginal fistula and a normal anal opening. Division of pelvic muscle floor and perineal dissection could be successfully avoided.

H-type ano-bulbar urethral fistula with an ectopic perineal fistula in a male patient complicated with VACTER association

Congenital H-type fistula is a rare congenital rectourogenital anomaly and is less common in males than in females. A one-year-and-eight-month-old boy with an ectopic perineal fistula was incidentally diagnosed with H-type fistula when he underwent voiding cystourethrography for vesicoureteral reflux. Further examination revealed that the H-type fistula was an ano-bulbar urethral fistula. At two years of age, the patient underwent fistulectomy and also anterior sagittal anorectoplasty for the ectopic perineal fistula during the same surgery.

Newborn with annular pancreas and H-Type tracheoesophageal fistula

Esophageal atresia with tracheoesophageal fistula (EA/TEF) and annular pancreas are both rare congenital anomalies, with very few reports of neonates presenting with both. More specifically, there are no reported cases of neonates with both annular pancreas and the rarest form of TEF, the H-type fistula. Here, we present the case of a neonate with annular pancreas diagnosed at birth requiring duodenoduodenostomy with delayed diagnosis of concomitant H-type TEF requiring subsequent fistula ligation with favorable outcomes. When patients with known congenital anomalies have persistent symptoms of unidentified etiology, heightened suspicion for other concomitant congenital anomalies can lead to prompt diagnosis, timely surgical management and good outcomes.

H-type congenital tracheo-oesophageal fistula associated with oesophageal stenosis: anatomical variant.

The goal of this paper was to report a new variant of oesophageal atresia: an H-type congenital tracheo-oesophageal fistula associated with oesophageal segmental stenosis distal to the fistula. Although symptoms were present from birth, we did not differentiate the new anatomical variant preoperatively. The patient was treated by fistula ligation, segmental resection of the distal oesophagus and end-to-end anastomosis of the oesophagus by thoracoscopic surgery. Here we describe the clinical history and management of the newborn infant, together with diagnostic recommendations to prevent misdiagnosis in the management of this condition.

H-Type Tracheoesophageal Fistula Masquerading as Grade V Gastroesophageal Reflux in a Neonate

Tracheoesophageal fistula (TEF) without associated esophageal atresia is a rare congenital anomaly. Diagnosis in neonatal period is usually not made and most of the patients are treated as cases of pneumonia. We report a case of H-type TEF, which was initially diagnosed as grade V gastroesophageal reflux on contrast esophagogram and bronchoscopy done revealed H-type fistula. Through cervical approach, fistula was repaired and baby had uneventful postoperative outcome. High index of clinical suspicion and early diagnosis can provide a better prognosis.

Congenital H-type tracheo-oesophageal fistula: An institutional review of a 10-year period.

BackgroundCongenital H-type tracheo-oesophageal fistula (H-TOF ) accounts for 4%–5% of all congenital tracheo-oesophageal malformations. We present our experience in managing 18 cases with congenital H-TOF at a tertiary institute over a 10-year period.MethodsRecords of all patients with congenital H-TOF managed from January 2009 to December 2018 in the Department of Paediatric Surgery at a tertiary institute were retrospectively analysed based on the age at presentation, gender, antenatal ultrasonography findings; birth history; details of previous hospitalisations, previous treatment details, presenting symptoms and associated anomalies; time to diagnosis; radiological investigations performed, bronchoscopy findings, intraoperative details, complications and postoperative follow-up.ResultsTotally 18 patients with congenital H-TOF were managed over a 10-year period. There were 12 females and six males. Six patients had associated anomalies. There was wide variation in age at the start of symptoms (3 days–4 years) and presentation/referral to us (15 days–12 years). Four patients were diagnosed to have H-TOF at first admission. The most common presenting symptom was recurrent pneumonias (n=18). Bronchoscopy was done in all patients, and fistula was diagnosed and cannulated before surgery. The fistula was present at C8–T1 in 14 patients. The median age at surgery was 12 months. In 17 patients, the fistula was repaired by the cervical approach. There were two deaths, and 16 patients are doing well on median follow-up of 8 years.ConclusionCongenital H-TOF should be considered in differential diagnosis while managing patients with recurrent lower respiratory tract infection and ‘coughing and choking episodes’; early diagnosis and management of the associated H-TOF is important for improved survival and outcome.