Triple fistula: management of a double tracheoesophageal fistula with a third H-type proximal fistula

Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) is a relatively common congenital condition in which there have been several described anatomical variants. The most common type, EA with distal TEF, comprises more than 75% of cases in many reports. Less commonly, a smaller proximal pouch fistula (H-type) will be associated with this most common variant in 1.4% of these cases. Only 2% of all cases of EA/TEF will have 2 large fistulas between the trachea and esophagus in which the end of the upper esophageal pouch connects terminally to the midtrachea and the distal esophagus arises from the trachea near the carina. Here we describe the management of an infant with this type of EA/TEF who was also found to have an H-type TEF of the proximal trachea. The combination of this type of EA/TEF with an associated H-type TEF or “triple fistula” has been previously described in the literature in only 1 other patient.     

Primary repair of tracheoesophageal fistula secondary to disc battery ingestion: a case report

The authors report on a child with tracheoesophageal fistula secondary to disc battery ingestion. Through a low cervical collar incision with limited sternal split, the fistula was primarily repaired, and the omohyoid muscle and thymus were mobilized to cover the suture lines. There are no signs of recurrent fistula 6 months after the operation. The anterior cervical approach with limited sternal split provided excellent access to the fistula. To the authors’ knowledge, this is the first report of successful primary repair of a tracheoesophageal fistula secondary to disk battery ingestion.     

Esophageal atresia without distal tracheoesophageal fistula: high incidence of proximal fistula

Background

This retrospective study was performed to test our suspicion that the incidence of esophageal atresia with proximal fistula in our institution is much higher than is generally reported.

Methods

The charts of all patients with esophageal atresia and/or tracheoesophageal fistula admitted in the period 1982 to 2000 were analyzed. The type of atresia and/or tracheoesophageal fistula was noted, and the relative incidence was calculated and compared with the relative incidence in a cumulative series of 3492 patients taken from 9 published studies.

Results

In the period under study, 123 patients with esophageal atresia and/or tracheoesophageal fistula were identified. The relative incidence of esophageal atresia without distal fistula was statistically not different (10.6% in the present series against 8.49% in the reference group). A statistically significant difference in the relative incidence of esophageal atresia with proximal fistula, however, was found: 5.69% in the present series against 1.05% in the reference group (P < .0001). Looking at the subgroup of patients without a distal fistula, more than half of the patients did have a proximal fistula.

Conclusions

The relative incidence of esophageal atresia with proximal fistula in this series of children with esophageal atresia and/or tracheoesophageal fistula is significantly higher than reported in the literature. This is on the account of the subgroup of patients without a distal fistula in which the incidence of a proximal fistula is more than 50%. Especially in this subgroup, the existence of a proximal fistula should be ruled out preoperatively.     

Diagnosis of a proximal tracheoesophageal fistula using three-dimensional CT scan: a case report

Missed proximal tracheoesophageal fistulas (TEF) after repair of an esophageal atresia (EA) with distal TEF are rare. The diagnosis usually is made on the basis of bronchoscopy and esophagoscopy, which can be inaccurate. Recent advances in computerized tomography (CT) have allowed saggital, coronal, and 3-dimensional reconstruction of any structure or organ. The authors report a case in which 3-dimensional CT was used to assist in the diagnosis of a missed proximal TEF in a 16-year-old girl.     

Repair of postintubation tracheoesophageal fistula in polytrauma patients

OBJECTIVE: We present our modest experience in treating tracheoesophageal fistula (TEF) in polytrauma patients where tracheal resection turns to be risky due to the size of the fistula and to the general condition of the patient. MATERIALS AND METHODS: Four polytrauma patients, three male and one female were treated at our department due to postintubation TEF. Confirmation was obtained endoscopically and radiologically. Surgical treatment consisted of identification and dissection of the fistulous tract and closure of the tracheal defect by transposing strap muscles and suturing them to the defect. Finally, the oesophageal defect was closed and a flap of sternocleidomastoid muscle was interposed between oesophagus and trachea. RESULTS: There was no intraoperative mortality. Three of our patients had an excellent result. The one who unfortunately returned to mechanical ventilation due to respiratory insufficiency developed a recurrent fistula. CONCLUSIONS: (1) Repair of postintubation TEF should be delayed until the patient is fully stabilised and weaned from ventilation. (2) Indirect closure of the tracheal defect with strap muscle transposition and sternocleidomastoid muscle interposition between oesophagus and trachea can be lifesaving.     

Management of an acquired tracheoesophageal fistula with a fascial free flap

BACKGROUND: Failure in the primary repair of a benign acquired tracheoesophageal fistula limits the operative options available at revision. Use of a fascial free flap to treat this condition has not been previously reported. METHODS: We review the case of a patient who had a tracheoesophageal fistula develop after percutaneous tracheostomy, who had failed previous primary repair with strap muscle interposition. RESULTS: A radial forearm fascial free flap was used at revision and resulted in resolution of the fistula. CONCLUSION: Use of a fascial free flap to address a persistent acquired tracheoesophageal fistula, when an accompanying stenotic segment is not present, is a viable treatment option.     

A gasless abdomen does not exclude distal tracheoesophageal fistula: the value of a repeat x-ray

A “gasless abdomen” in babies with esophageal atresia (EA) has traditionally been thought to imply absence of a distal tracheoesophageal fistula (TEF). We present 3 neonates with a provisional diagnosis of EA, who had a gasless abdomen on an initial x-ray taken within the first 4 hours of life. These children were subsequently shown to have gas in the stomach associated with a distal TEF. Two children were examined on subsequent repeat x-ray of the abdomen, when gas was demonstrated in the stomach. Another child had a laparotomy for a gastrostomy when a distended stomach was found. This baby went on to have a thoracotomy and a routine repair of the distal TEF and an esophageal anastamosis. Based on our data of 65 cases of EA and distal TEF, it is recommended that an x-ray of the abdomen is repeated before surgery is undertaken if the gasless abdomen is documented during the first 4 hours of life.     

Tracheoesophageal fistula secondary to disk battery ingestion: a case report of gastric interposition and tracheal patch

The authors report a child with tracheoesophageal fistula secondary to disk battery ingestion. With respiratory compromise precluding expectant therapy and primary repair not achievable, gastric interposition and tracheal patch repair were undertaken in the acute phase. To the authors' knowledge, this is the first report of primary gastric interposition for traumatic tracheoesophageal fistula and suggest that immediate reconstruction can give an outcome at least as good as other reported approaches.     

Tracheal diverticula after tracheoesophageal fistula repair: case series and review of the literature

Background

Tracheoesophageal fistula (TEF) is the most common congenital tracheal abnormality, frequently associated with esophageal atresia. Respiratory symptoms are associated with all types of TEF, even after surgical repair of the fistula. Gastroesophageal reflux (GER) with aspiration of gastric contents, structural instability of the airways (tracheomalacia), abnormal respiratory epithelium, abnormal esophageal motility, recurrent TEF, and esophageal stenosis contribute to postsurgical complications.

Methods

We review 7 patients between 4 and 14 years of age with a history of TEF repair and persistent or worsening respiratory symptoms despite conventional airway clearance techniques and treatment of GER.

Results

Bronchoscopic evaluation in all 7 patients revealed tracheomalacia and a diverticulum on the posterior wall of the trachea at the fistula repair site.

Conclusion

We hypothesize that the diverticula impaired airway clearance and contributed to persistent respiratory symptoms. Possible mechanisms for the diverticulum contributing to poor airway clearance include facilitating the pooling of secretions and acting as a “barrier” to the lower airway clearance mechanism. The diagnosis of a diverticulum should be considered early in patients with persistent respiratory symptoms after management of GER and tracheomalacia. Early obliteration of tracheal diverticula might improve respiratory status in some patients.     

Congenital laryngeal atresia associated with esophageal atresia and tracheoesophageal fistula: a case of long-term survival

Congenital laryngeal atresia (LA) is a life-threatening anomaly in which appropriate perinatal management is essential for survival. The authors report a neonate with LA associated with esophageal atresia (EA) and tracheoesophageal fistula (TEF) who was successfully resuscitated by emergent tracheostomy. Before birth, the patient had a diagnosis of EA based on the findings of polyhydramnios and absent stomach bubble. Immediately after birth, severe respiratory distress, cyanosis, and sternal retraction were evident. Because either endotracheal or esophageal intubation was unsuccessful, an emergent tracheostomy was performed. A direct laryngoscope revealed a subglottic atresia with normal appearance of the vocal cords. Repair of EA with TEF was performed on the third day of life, and the postoperative course was uneventful. In 2 years follow-up, the patient has no mental retardation nor central nervous impairment. Because of the presence of TEF, the antenatal ultrasonogram did not demonstrate the characteristic findings of the congenital high airway obstruction syndrome. This case represents one of the very few reported cases of successful resuscitation of a neonate with an unanticipated LA in which emergent airway management is required immediately after birth.     

Feingold syndome: a rare but important cause of syndromic tracheoesophageal fistula

Esophageal atresia (EA) and tracheoesophageal fistula (TEF) are common congenital malformations and are associated with additional anomalies in approximately half of cases. Feingold syndrome is an important genetic cause of syndromic EA-TEF to consider in patients with associated microcephaly and digital anomalies. We present a case report of a male infant with EA-TEF, microcephaly, subtle facial dysmorphism, dysplastic kidney, short fifth fingers, second finger clinodactyly, and increased spacing between the first and second toes bilaterally. His clinical presentation was suggestive of Feingold syndrome, and genetic testing of the MYCN gene confirmed the diagnosis. Feingold syndrome is an autosomal dominant condition, and therefore, the diagnosis has important implications for genetic counseling.     

Repair of esophageal atresia with tracheoesophageal fistula via thoracotomy: a contemporary series

Background

A recent series detailing thoracoscopic repair of esophageal atresia with tracheoesophageal fistula (EA/TEF) reported lower complication rates compared with historic controls. This study provides a contemporary cohort of patients repaired via thoracotomy for comparison with the recent large multi-institutional thoracoscopic series.

Methods

Records of patients with EA/TEF between 1993 and 2008 were reviewed. Attention was focused on demographics and complications including anastomotic leak, recurrent fistulae, stricture formation, and need for fundoplication.

Results

Seventy-two patients underwent repair of EA/TEF via thoracotomy. Complication rates in the current series compared with the thoracoscopic series were anastomotic leak, 2.7% versus 7.6%; recurrent fistulae, 2.7% versus 1.9%; stricture, 5.5% versus 3.8%; and need for fundoplication, 12% versus 24%. Differences in complication rates did not reach statistical significance. Two children in this cohort developed mild scoliosis attributed to congenital vertebral anomalies, neither of whom required intervention.

Conclusions

Thoracoscopic repair of EA/TEF yielded complication rates similar to this contemporary series; however, trends toward increased anastomotic leaks and greater need for fundoplication were noted. No musculoskeletal sequelae were directly attributable to thoracotomy.     

Lung torsion after tracheoesophageal fistula repair: a case report and review of literature

Lung torsion is a very rare event that has been described after trauma, spontaneously, and post-thoracic surgery, with only 8 cases reported in the pediatric literature. We present the first case report of lung torsion complicating tracheoesophageal fistula repair. The diagnosis was suggested on chest ultrasonogram and Doppler and confirmed by computed tomographic scans. On exploration, a 90° rotation of the right middle and lower lobes in a clockwise direction was found. A complete interlobar fissure and an absent inferior pulmonary ligament were identified as predisposing factors. The lobes were untwisted, and lobar fixation was performed to prevent retorsion. Postoperatively, the patient recovered complete lung function, and the patient's chest radiologic and ultrasonographic examination results normalized. Lung torsion is an extremely uncommon event. It causes clinical deterioration in the face of normal arterial blood gases and requires a high index of suspicion for diagnosis. Resection of the twisted lung or lobe may be unnecessary if diagnosis is made early and vascular occlusion is partial. Patients with complete interlobar fissures may benefit from pulmonary lobe fixation as a prophylaxis against occurrence or recurrence of this complication.     

The effect of a right-sided aortic arch on outcome in children with esophageal atresia and tracheoesophageal fistula

During repair of an esophageal atresia with tracheoesophageal fistula (EA/TEF), a right-sided aortic arch (RAA) presents a technical challenge. We reviewed our experience with EA/TEF focusing on the impact of RAA.

Methods

A retrospective review of patients with EA/TEF at our institution from 1990 to 2004 was performed. Of 61 patients, 53 had a left aortic arch (LAA) and 8 had RAA.

Results

The mean gestational age and birth weight were similar between patients with LAA and RAA. There were more vascular rings in patients with RAA than with LAA (37.5% vs 3.8%, P = .009). A preoperative echocardiogram correctly identified the location of the arch in all with LAA but only 62.5% of those with RAA. Operative complications occurred in 25% of patients with RAA and 11% of patients with LAA.

Conclusions

Management of a child with RAA and EA/TEF is technically challenging. Preoperative identification of the great vessel anomalies may impact operative decision making. Therefore, careful diagnostic evaluation of a patient with an aortic arch that cannot be easily defined by ECHO should be considered before definitive repair.     

Closure of a large bronchial fistula with a latissimus dorsi myocutaneous flap

We describe a case of a large bronchial fistula and empyema after right upper lobectomy that was treated successfully with open window thoracostomy followed by a latissimus dorsi myocutaneous flap and limited thoracoplasty. A latissimus dorsi myocutaneous flap can provide immediate airtight closure of a large bronchial fistula, allowing lavage and curettage of the empyema cavity to reduce the chance of postoperative infection. An important aspect of this technique is that the deepithelialized skin side rather than muscle is sutured to an opening of the bronchus. As compared with other techniques, a latissimus dorsi myocutaneous flap is superior in that it requires a single incision and does not require an intraop-erative change of position. In addition, the technique causes little dysfunction of the chest and shoulder and preserves the vascular supply to ensure the viability of the flap even if it was divided in a previous operation.     

Eosinophilic esophagitis and intermediate esophagitis after tracheoesophageal fistula repair: a case series

Background

Eosinophilic esophagitis (EE) is often missed or underdiagnosed in children, particularly in the setting of reflux disease associated with tracheoesophageal fistula (TEF). Intermediate esophagitis (IE) is a recently described condition, which includes characteristics of gastroesophageal reflux disease and EE but does not present with enough eosinophils on biopsy to diagnose EE. Here we present a case series of EE and IE associated with TEF, and their clinical manifestations.

Methods

A retrospective analysis including clinical presentation, endoscopic and pathologic findings, and treatment of 4 patients with EE and 4 patients with IE who presented between 2003 and 2007 was performed.

Results

Male dominance was found equally in both groups (75%), and most patients had a personal history of atopy (87.5%). Food allergies were seen mainly in the EE group (75%). The most frequent primary symptoms in both groups were dysphagia for solids (75%) and for liquids (25%). The median age at diagnosis was 9.8 vs 11.2 years in the EE and IE groups, respectively. On endoscopy, both groups had similar findings including furrows (EE, 75%; IE, 66.6%) and white plaques (EE, 50%; IE, 33.3%). In both groups, almost all patients had basal cell hyperplasia on biopsy (EE, 100%; IE, 75%). Degranulated surface eosinophils (50%) and eosinophilic abscess (25%) were found in the EE group only. Elongated rete papillae were more often seen in EE biopsies (50%) compared with IE biopsies (25%). Peripheral serum eosinophilia was seen in all EE patients and in 33% of the IE patients. The only effective treatment with complete resolution of the symptoms was the topical or systemic application of steroids.

Conclusions

The diagnosis of EE and IE is frequently missed or delayed. Eosinophilic esophagitis should be suspected in reflux disease refractory to conventional treatment, particularly in the setting of TEF. Intermediate esophagitis represents an entity that includes findings of gastroesophageal reflux disease and EE. Endoscopic biopsies are diagnostic for both conditions and allow institution of specific medical treatment.