Sacrococcygeal teratomas: midline reconstruction improves cosmesis without compromising outcomes.

AIM: Several studies have addressed the long-term functional, psychosexual and psychosocial outcomes following sacrococcygeal teratoma (SCT) excision. It is well reported that the classical chevron incision and reconstruction can leave a cosmetically unsatisfactory result; however, there is little in the literature focussed on improving this outcome. In our institution the preference is to perform a midline reconstruction, where possible, this is felt to improve appearance without compromising the oncological or functional outcome. The aim of this study was to evaluate patient-perceived cosmetic outcomes of the midline reconstruction. METHODS: All patients undergoing surgery for SCT between 2007 and 2020 were included in the study. Patient demographics, operation type, functional outcome and recurrence were all recorded. The primary outcome measure was patient/parent satisfaction with the cosmetic appearance. This was assessed using both qualitative and quantitative methodologies. Following ethical approval parents were asked questions from two existing validated patient outcome questionnaires: "Patient and Observer Scar Assessment Scale" (POSAS) v2.0 and the "Patient Scar Assessment Questionnaire". RESULTS: Thirty-two patients underwent surgery at our institution for SCT during the study period. Twenty-four had a posterior approach with midline reconstruction, two laparotomy and excision (excluded from this study) and six had a combined approach. Median follow-up was 35 months (8.5-96 months). There were no recurrences. 4/30 (13%) have persistent urological symptoms, and 1/30 (3%) has constipation requiring bowel management. Questionnaires were sent to 26/30 families with a 77% return rate. Median total score was 11 (7.4-17.5) on a 60-point scale (6, as normal skin, 60, worst imaginable scar). Twenty (95%) reported that the scar never affects the child's activities and 15 (71%) said they are "not at all" conscious of the scar. CONCLUSION: Scars can lead to an array of cosmetic, functional, and psychological consequences and as such consideration needs to be given to scarring following surgery for sacrococcygeal teratomas. This study demonstrates that a midline reconstruction produces a cosmetically favourable outcome. We, therefore, recommend where appropriate a midline reconstruction should be considered for SCT.

Esophageal atresia and tracheoesophageal fistula associated with tetralogy of Fallot: a review of mortality.

INTRODUCTION: Oesophageal atresia ± tracheoesophageal fistula (EA/TEF) associated with congenital heart disease (CHD) carries a worse prognosis than EA/TEF alone. Though the Spitz classification takes major CHD into account, there are no data regarding survival with the specific combination of EA/TEF and Tetralogy of Fallot (TOF). With advances in postnatal care, we hypothesised that, survival is improving in these complex patients. This study reports morbidity and mortality outcomes of newborns with oesophageal atresia and TOF cardiac malformations METHODS: All patients with EA/TEF and TOF treated at Alder Hey Children's Hospital between the years 2000-2020, were identified. Data sets regarding gestation, birth weight, associated anomalies, operative intervention, morbidity, and mortality were analysed. RESULTS: Of a total of 350, EA/TEF patients 9 (2.6%) cases had EA/TEF associated with TOF (M:F 4:5). The median gestational age was 35/40 (range 28-41 weeks) with a median birth weight of 1790 g (range 1060-3350 g). Overall survival was 56% (5/9 cases) and all survivors remain under follow up (range 37-4458 days). Surgical strategies for managing EA/TEF with Fallot's tetralogy included 6/9 primary repairs and 3/9 cases with TEF ligation only (+ gastrostomy ± oesophagostomy). CONCLUSIONS: This study reports outcome data from one of the largest series of EA TEF patients with Fallot's tetralogy. Whilst outcomes may be challenging for this unique patient cohort, survival metrics provide important prognostic information that can be widely shared with health care teams and parents.

Role of adiponectin in the development of high fat diet-induced metabolic abnormalities in mice.

The adipokine adiponectin is decreased in severe obesity and is inversely associated with adipose mass. Adiponectin is associated with insulin sensitivity and cardioprotection. Obesity frequently results in the development of a "cardiometabolic syndrome" characterized by increased circulating insulin and leptin, and cardiac hypertrophy and dysfunction. This study examined if adiponectin-deficiency affects the development of metabolic and cardiac abnormalities in response to modest obesity. Mice were studied under normal conditions and with mild cardiac pressure-overload induced by abdominal aortic banding. After surgery, wild type and adiponectin-deficient mice were fed a high-fat diet for 8 weeks (45% energy from fat vs. 10%). In wild type mice the high-fat diet increased fat and whole body mass, which corresponded with elevated circulating insulin and leptin and a decrease the glucose/insulin ratio. On the other hand, in adiponectin-deficient mice the high-fat diet had less impact on body mass and no effect on fat mass, insulin, leptin, or glucose/insulin. There was modest cardiac hypertrophy with aortic banding, but no cardiac dysfunction or effects of adiponectin deficiency or diet. The results suggest that the increase in adipose mass, leptin and insulin induced by a high fat diet is dependent on adiponectin. The lack of accelerated cardiac hypertrophy and dysfunction in the adiponectin-deficient mice subjected to aortic banding and the high-fat diet suggest that adiponectin may not play a major role in protecting the heart during the early stages of diet-induced obesity.