Life-threatening mesenchymal hamartoma of the chest wall in a neonate

Mesenchymal hamartomas of the chest wall are unusual tumours diagnosed in neonates. They mostly resolve spontaneously hence conservative management has been advocated. Some compress vital structures in the thoracic cavity or bleed warranting surgical intervention. We present a neonate with mesenchymal hamartoma of the chest wall presenting as unilateral multifocal lesions with life threatening complications. He responded well to surgical intervention and was successfully discharged

Thoracic versus abdominal approach to correct diaphragmatic eventration in children

Background: Plication of diaphragm (DP) for eventration (DE) can be done using thoracic or abdominal approaches. The purpose of our study was to compare outcomes between these approaches based on our experience and on systematic literature review. Methods: Retrospective records of children < 16 years who underwent DP (single-center, 2004–2018) were recorded and analyzed. Systematic review and meta-analysis of related studies was undertaken. Data are reported as median (range). Results: Eighty-nine cases were identified in thoracic (Congenital = 5, Acquired = 84) and 13 (Congenital = 10, Acquired = 3) in abdominal group aged 5.88 (0.36–184.44) and 10.0 (0.12–181.8) months. Improvement in diaphragm level post-DP was significantly higher in abdominal [2(0–4)] than chest [1.5(0–5)] group (p = 0.04). On Cox regression analysis, there was a non-significant trend to a longer time to extubation in the chest group (Hazard ratio (HR) = 0.539[0.208–1.395], p = 0.203). Patients operated transthoracically left intensive care unit after a significantly longer time (HR = 0.339[0.119–0.966], p = 0.043). Patients operated transabdominally tended to be fed later, although this was not significant (HR = 1.801[0.762–4.253], p = 0.043). On Kaplan–Meier analysis, there was a non-significant trend to a lower rate of recurrence in the abdominal group (HR = 0.3196[0.061–1.675], p = 0.1876). In the meta-analysis including three published studies as well as our data (total n = 181, Thoracic = 139, Abdominal = 42), no difference was found in the incidence of recurrence amongst the 2 groups (RD = -0.04, 95%CI = -0.25, 0.18, p = 0.74). Conclusion: This is one of the largest reports on outcomes of children undergoing DP for DE. There is no significant difference in recurrence rate, even though all recurrences in our series (15.7%) were in the acquired cases operated using a thoracic approach

Intrathoracic prosthesis in children in preventing post-pneumonectomy syndrome: Its role in congenital single lung and post-pneumonectomy situations

Background: Postpneumopnectomy syndrome (PPS) is an extreme rotation and malposition of mediastinum causing dynamic and symptomatic central airway compression, arising after pneumonectomy or more uncommonly, in congenital single-lung physiology. Affected patients present with severe respiratory compromise. Intrathoracic prosthesis placement is an evolving technique in children that mitigate the effects of thoracic dead space. / Research Question: Assessment of clinical recovery and functional benefit in children undergoing placement of intrathoracic prosthesis following pneumonectomy or in congenital single lung situations. / Study Design and Methods: Retrospective chart review of patients at Great Ormond Street Hospital from 2010 and 2020 was performed of all patients who underwent intrathoracic tissue expander placement. We summarize the outcomes of twenty-four children, including those with both congenital and postpneumonectomy PPS etiology. / Results: 24 children who underwent placement of intrathoracic prosthesis for PPS in the study period with median age of 3.5 months and weight of 5 kg. Single lung etiology was congenital in 15 children (6 agenesis, 9 hypoplasia), and postpneumonectomy in 9 children. In seven patients, there was associated long segment tracheal stenosis. Pre-operative ECMO was required in 2 patients, and pre-operative ventilation was required in 12 patients – all of whom had congenital single lung. Intrathoracic prosthesis placement was concurrent with intracardiac repair in 5 patients. There were no operative deaths, but one early postoperative death related to septicaemia. Median follow up was 75 months with 10 patients on continued respiratory support and 3 on nocturnal support with good quality of life. Two children needed reoperations for replacement of prosthesis. / Conclusion: The use of tissue expanders is within the armamentarium of most plastic surgeons’ practice. We also therefore advocate for a collaborative team approach involving Plastic and Cardiothoracic Surgery for surgical treatment of these patients. This multidisciplinary strategy has improved management of this rare and debilitating condition of PPS, thereby offering significant improvements in general progress of these sick children having single lung physiology. Evidence is still lacking on functional outcomes in these children and further work is necessary to prove that this is indeed achievable

Foreign body ingestion during the COVID-19 pandemic: a retrospective single centre review

Introduction: During the COVID-19 pandemic in 2020, there were many changes in the provision of healthcare as well as home and educational environments for children. We noted an apparent increase in the number of children presenting with ingested foreign bodies and due to the potential impact of injury from this, further investigated this phenomenon. Method: Using a prospective electronic record, data were retrospectively collected for patients referred to our institution with foreign body ingestion from March 2020 to September 2020 and compared with the same period the year prior as a control. Results: During the 6-month pandemic period of review, it was observed that 2.5 times more children were referred with foreign body ingestion (n=25) in comparison to the control period (n=10). There was also a significant increase in the proportion of button battery and magnet ingestions during the COVID-19 pandemic (p 0.04). Conclusion: These findings raise concerns of both increased frequency of foreign body ingestion during the COVID-19 pandemic and the nature of ingested foreign bodies linked with significant morbidity. This may relate to the disruption of home and work environments and carries implications for ongoing restrictions. Further awareness of the danger of foreign body ingestion, especially batteries and magnets, is necessary (project ID: 2956)

Micro-CT of tracheal stenosis in trisomy 21

A male infant with trisomy 21, born at 36 weeks' gestation, had care withdrawn at 2 months of age and was referred for postmortem investigations. The child had been ventilator dependent since the first week of life following surgery for intestinal perforation from necrotising enterocolitis. A CT thorax at 1 month of age demonstrated a tight tracheal stenosis, inferior to the tip of the endotracheal tube, with a luminal diameter of 1.2 mm over a length of 1 cm (figure 1). There were no associated cardiovascular or bronchial tree anomalies. Despite efforts to optimise the child’s condition for tracheal reconstruction, he was subsequently found to have an underlying immune deficit disorder with profound lymphopenia and continued to require iontropic support for haemodynamic compromise. After a further month of expectant clinical management without improvement, care was withdrawn

Thoracoscopic aortopexy for symptomatic tracheobronchomalacia

Aim: Symptomatic tracheobronchomalacia can be fatal. Successful treatment includes aortopexy. We report outcomes of the thoracoscopic approach in a single centre. Methods: All patients undergoing thoracoscopic aortopexies from 2009 to 2018 were retrospectively reviewed. Data was reported as median (interquartile range). Risk factors for subsequent tracheostomy were analyzed with logistics regression model, p < 0.05 as significant. Results: Twenty-one patients with mid to distal tracheomalacia (n = 17) and bronchial involvement (n = 4) were determined on bronchoscopy, tracheobronchogram, or CT thorax. Preoperative patient demographics and comorbidities, e.g., gastro-oesophageal reflux disease, prematurity, and cardiac anomalies were recorded. Indications for thoracoscopic aortopexy were apparent life-threatening event(s) (n = 14), recurrent chest infections (n = 5), and failure to wean invasive ventilation (n = 2). Thoracoscopic aortopexies (n = 20) with conversion to open (n = 1) were performed. Intraoperative bleeding (n = 2) occurred, and chest tube (n = 1) was inserted for monitoring. Intraoperative bronchoscopy (n = 17) confirmed improvement of tracheomalacia. Anesthetic time was 140 (90–160) minutes. Postoperatively, 2 patients had dehiscence of the aorta from the sternum. They underwent redo open aortopexy with posterior tracheopexy, and 1 required subsequent tracheostomy. Another 2 patients required tracheostomies. Potential risk factors for subsequent tracheostomy were investigated, and only the association of tracheobronchomalacia was close to significance (OR 16 (95% CI 0.95–267.03), p = 0.05). Follow up duration was 365 (72–854) days. Symptoms resolution occurred in n = 17 (81%) of patients. Conclusion: Different modalities were used to delineate the site of tracheobronchomalacia and its etiology. Tracheomalacia with bronchial involvement may be a risk factor for subsequent tracheostomy

Midterm results of the Ross procedure in children: an appraisal of the subannular implantation with interrupted sutures technique

OBJECTIVES: The support of the pulmonary autograft root by the fibromuscular left ventricular outflow tract is emphasized to address the concern related to the dilatation of the pulmonary autograft structures in the paediatric population. METHODS: This retrospective study analyses the outcomes of 75 children who were operated between 1998 and 2012 with the subannular interrupted sutures technique at a median age of 10.2 years (range, 5.3 months–18.0 years). Median follow-up time was 5.2 years (range, 3 days–13.2 years). RESULTS: There were no deaths, but there were 3 reinterventions on the autograft for regurgitation and 2 resections of left ventricular outflow tract obstruction. There was no significant autograft stenosis, and freedom from moderate-to-severe regurgitation was 95% (95% confidence interval: 89–100) and 88% (95% confidence interval: 77–99) at 5 and 10 years, respectively. Median z-scores at the latest follow-up examination were, at the annulus, 0.31 [interquartile range (IQR) = −0.81 to 1.2]; at the sinus of Valsalva, 2.7 (IQR = 1.5–3.5); and at the sinotubular junction, 3.1 (IQR = 1.7–4.2). The correlation between z-scores and time after the operation was negative at the level of the annulus (r = −0.29, P = 0.034) but positive at the level of the sinus (r = +0.37, P = 0.005) and the sinotubular junction (r = +0.26, P = 0.068). The median rate of change in the z-score at the annulus was low, 0.065 z-score/year (IQR = −0.13 to 0.43). CONCLUSIONS: The subannular interrupted sutures implantation technique is associated with acceptable risks and, in the midterm, delivers limited annular dilatation, autograft regurgitation and delayed need for autograft reintervention

Tracheal Replacement Therapy with a Stem Cell-Seeded Graft: Lessons from Compassionate Use Application of a GMP-Compliant Tissue-Engineered Medicine

Tracheal replacement for the treatment of end-stage airway disease remains an elusive goal. The use of tissue-engineered tracheae in compassionate use cases suggests that such an approach is a viable option. Here, a stem cell-seeded, decellularized tissue-engineered tracheal graft was used on a compassionate basis for a girl with critical tracheal stenosis after conventional reconstructive techniques failed. The graft represents the first cell-seeded tracheal graft manufactured to full good manufacturing practice (GMP) standards. We report important preclinical and clinical data from the case, which ended in the death of the recipient. Early results were encouraging, but an acute event, hypothesized to be an intrathoracic bleed, caused sudden airway obstruction 3 weeks post-transplantation, resulting in her death. We detail the clinical events and identify areas of priority to improve future grafts. In particular, we advocate the use of stents during the first few months post-implantation. The negative outcome of this case highlights the inherent difficulties in clinical translation where preclinical in vivo models cannot replicate complex clinical scenarios that are encountered. The practical difficulties in delivering GMP grafts underscore the need to refine protocols for phase I clinical trials