Primary tracheocutaneous fistula closure with immediate transition to nocturnal noninvasive positive pressure ventilation in two children with Congenital Central Hypoventilation Syndrome.

Transitioning children with Congenital Central Hypoventilation Syndrome (CCHS) from nocturnal invasive ventilation via tracheostomy to noninvasive positive pressure ventilation (NIPPV) is challenging due to the leak caused by the tracheocutaneous fistula (TCF), resulting in insufficient ventilation. Decannulation and primary closure of the TCF with immediate transition to nocturnal NIPPV was performed in two children with CCHS at a tertiary care children's hospital. Neither child developed significant adverse effects such as pneumomediastinum or pneumothorax. This technique is a novel approach that may improve decannulation outcomes and aid transition to NIPPV in this patient population.

Side-to-Side Tracheobronchoplasty to Reconstruct Complex Congenital Tracheobronchial Stenosis.

BACKGROUND: Long segment tracheobronchial stenosis is a rare congenital anomaly that can also occur in combination with abnormal bronchial arborization. Long segment tracheal reconstruction in the setting of a supernumerary bridging bronchus has been reported; however, these repairs can be particularly complex. We present our experience using the bridging bronchus to augment long segment tracheal stenosis with a side-to-side tracheobronchoplasty. METHODS: Four patients with complex long segment tracheobronchial stenosis involving a bronchus suis (right upper lobe bronchus) and a bridging bronchus presented with refractory respiratory distress requiring urgent tracheal reconstruction. Patient 1 was initially managed with modified slide tracheoplasty and tracheostomy. Patients 2, 3, and 4 were managed with single-stage procedures. All patients underwent definitive long segment tracheobronchoplasty consisting of a side-to-side anastomosis between the bridging bronchus and the right upper lobe bronchus. RESULTS: Age at surgery was 569, 69, 24, and 142 days, respectively. Weight at surgery was 9.3, 4.3, 2.7, and 5.9 kg. All patients were weaned from mechanical ventilation at 84, 13, 47, and 8 days after side-to-side tracheobronchoplasty. All patients were alive and free from tracheostomy at follow-up of 6.7, 3.8, 2.7, and 0.5 years. CONCLUSIONS: Side-to-side tracheal reconstruction is feasible in severe cases of long segment tracheal stenosis with a right upper lobe bronchus and a bridging bronchus. This technique can be successfully applied in high-risk patients and in the neonatal period and can provide excellent midterm results.

Anterior tracheal suspension for tracheobronchomalacia in infants and children.

BACKGROUND: Severe tracheobronchomalacia significantly complicates the postoperative course of infants and children with congenital heart disease, tracheoesophageal fistula, and tracheal stenosis. We have found that traditional approaches, including aortopexy, have been inconsistent in preventing acute life threatening events (ALTEs). In order to directly support the anterior tracheal wall, we have adopted the use of direct anterior tracheal suspension (ATS). METHODS: Twenty-one children, median age 5 months (35 days to 11 years) and weight 5.0 (2.3 to 28.0) kg have undergone anterior tracheal suspension for severe tracheobronchomalacia through median sternotomy; 15 for inability to ventilate despite mechanical respiratory support, 3 for intermittent ALTEs without mechanical respiratory support, and 3 for recurrent respiratory admissions. Nine procedures were performed as isolated ATS and 12 procedures were combined with at least 1 of the following: repair of ventricular septal defect; vascular ring; atrioventricular canal; tracheal reconstruction or arterial-pexy. Level of respiratory support was graded at preoperative (preop), discharge, and follow-up, and respiratory clinical status was graded at preop and follow-up. Median follow-up was 30.0 months (2.0 to 57.0 months). RESULTS: There was no mortality. Both level of respiratory support and the clinical status improved at all time points studied compared with preoperative score (p < 0.001) after ATS. Whether ATS was performed in isolation or combined with other procedures did not impact these findings. CONCLUSIONS: Anterior tracheal suspension is feasible and appears effective in dramatically improving respiratory clinical status. Tracheal suspension is applicable to a wide range of anatomic variants. Additional study is needed to characterize long-term functional outcomes.

Clinical characteristics of aero-digestive foreign bodies in neurologically impaired children.

OBJECTIVE: To determine if children with neurological impairment are at additional risk for foreign body aspiration or impaction and if they present with a different clinical course than normal children. DESIGN: Retrospective cohort study. SETTING: Tertiary care children's hospital. PATIENTS AND OTHER PARTICIPANTS: A retrospective chart review of 328 consecutive procedures for aero-digestive foreign body removal over a 6 year period was performed. Of these patients 52 (15.9%) were identified with neurological impairment. RESULTS: Compared with the non-impaired children, those with neurological impairment were older, diagnosed later, hospitalized longer and had a higher incidence of complications. CONCLUSION: A greater index of suspicion is needed to timely treat aero-digestive foreign bodies in children with neurological impairment. These children may not be receiving the necessary attention when presenting with non-specific symptoms.