Outcome of congenital tracheal stenosis in children over two decades in a national cardiothoracic surgical unit.

OBJECTIVE: To assess the outcomes of congenital tracheal stenosis among children. MATERIALS AND METHODS: A retrospective review of all children who underwent surgical repair of congenital tracheal stenosis reviewing charts, operative notes, echocardiograms, CT and MRI data from January 2002 to February 2019. RESULTS: Twenty-six children underwent surgical treatment for tracheal stenosis. The median age was 3 months (range 0.3-35 months) and the median weight was 4.7 kg (range 2.5-13 kg) at the time of surgical intervention. Stridor was the most common presenting symptom in 17 patients (65% of patients). Twenty-one patients (81%) had concurrent cardiac anomalies, with pulmonary arterial sling being the most common, present in nine patients (34%). Extracorporeal life support was utilised in seven patients (27%) pre-operatively. Laryngeal release was required in 16 patients. In 7 patients an end-to-end anastomosis was performed, in 18 patients slide tracheoplasty, and 1 patient had a double slide tracheoplasty. The median cardiopulmonary bypass time was 106 minutes (range 25-255 minutes). The median cross-clamp time was 30 minutes (range 5-67 minutes). The median post-operative duration of ventilation was 5 days (range 0.5-16 days). The median ICU length of stay was 12.5 days (range 2-60 days). There were three hospital mortalities with 88% survival. One patient only required reintervention with balloon dilation. Twenty-two patients (85%) remained symptom-free on median follow-up at 7.6 years (range 0.2-17 years). Two patients since 2017 had 3D printed tracheas produced from CT imaging to assist surgical planning. CONCLUSION: Congenital tracheal stenosis can be managed effectively with excellent outcomes and 3D printed models assist in planning the optimal surgical intervention.

Use of systemic corticosteroids in management of a large congenital haemangioma of the scalp.

Congenital haemangiomas are rare and are estimated to have a combined incidence of less than 3% of all infantile haemangiomas. They are fully grown at birth, having undergone their proliferative phase in utero. Congenital hemangiomas can present at birth or in some cases can be detected antenatally on imaging. In the majority of patients no therapeutic intervention is required. Congenital hemangiomas also differ from infantile hemangiomas by staining negatively with GLUT1 antibody. They fall into two major subtypes: rapidly involuting congenital hemangiomas (RICHs) and noninvoluting congenital hemangiomas (NICHs). Here we describe a case of RICH detected antenatally on ultrasound imaging. This lesion caused significant complications in the postnatal period due to the bulk of the lesion and the presence of incipient ulceration with the risk of possible catastrophic hemorrhage. A therapeutic trial of oral corticosteroid was commenced in an effort to accelerate involution due to the significant risk associated with other possible treatment modalities such as embolization or surgical intervention.

Mediastinal and neck kaposiform hemangioendothelioma: report of three cases.

Kaposiform hemangioendothelioma is an aggressive vascular tumor, named for its striking histologic resemblance to Kaposi sarcoma and locally invasive growth. Mortality is high, and ranges from 10% to 24% for all kaposiform hemangioendothelioma lesions, with a significantly higher mortality for deep soft-tissue or visceral lesions occurring in infants less than 6 months. Mediastinal and neck kaposiform hemangioendothelioma in particular merit special discussion, as involvement of these critical anatomic locations results in significant site-specific therapeutic challenges due to invasion of vital structures, inherent delays in establishing histopathologic confirmation, and difficulties in monitoring disease status. We report our experience with three cases of mediastinal and neck kaposiform hemangioendothelioma, emphasizing the unique diagnostic and management challenges, variable response to treatment and outcome of this anatomic variant of kaposiform hemangioendothelioma.