A baby boy weighing 1605 g was born at 35 weeks’ gestation with an unexpected large abdominal wall skin defect. This had been an in vitro fertilisation pregnancy in which two embryos were returned, one of which divided resulting in two monochorionic fetuses and a further third fetus. Interstitial laser therapy was performed at 13 weeks to one fetus resulting in a dichorionic twin pregnancy. The other live twin was completely unaffected. Formal pathological examination of the placenta of the affected twin revealed a fetus papyraceus.
The skin defect was symmetrical extending posteriorly to the erectae spinal muscles, inferiorly to below the umbilicus and superiorly to just below the nipple. There was an island of normal skin around the umbilicus. The abdominal contents were visible through a partially translucent membrane (fig 1). The abdominal wall musculature could not be identified. There were no other abnormalities found.
Figure 1
Abdominal contents visible through a partially translucent membrane.
This presentation would fit the diagnostic criteria for type V cutis aplasia. There are nine recognised subgroups of aplasia cutis based on inheritance, skin defect pattern, location and associated abnormalities. Type V consists of a symmetrical defect of the trunk and/or limbs in association with a fetus papyraceus,1 which is more likely to be related to variation in blood flow through the placental blood vessels, embolic phenomena in vessels or a coagulopathy related to the dead fetus in utero.2
The baby has remained well but the skin defect has been slow to epithelialise. Treatment is currently conservative with regular dressings and there remains an option to skin graft. Skin grafting using allogenic cultured keratinocytes is thought to possibly reduce scar formation.3
The non-operative management of splenic injury in children is recommended widely, and is possible in over 95 per cent of episodes. Practice appears to vary between centres.The Trauma Audit and Research Network (TARN) database was interrogated to determine the management of isolated paediatric splenic injuries in hospitals in England and Wales. Rates of non-operative management, duration of hospital stay, readmission and mortality were recorded. Management in paediatric surgical hospitals was compared with that in adult hospitals.Between January 2000 and December 2015 there were 574 episodes. Children treated in a paediatric surgical hospital had a 95·7 per cent rate of non-operative management, compared with 75·5 per cent in an adult hospital (P < 0·001). Splenectomy was done in 2·3 per cent of children in hospitals with a paediatric surgeon and in 17·2 per cent of those treated in an adult hospital (P < 0·001). There was a significant difference in the rate of non-operative management in children of all ages. There was some improvement in non-operative management in adult hospitals in the later part of the study, but significant ongoing differences remained.The management of children with isolated splenic injury is different depending on where they are treated. The rate of non-operative management is lower in hospitals without a paediatric surgeon present.
A baby boy weighing 1605 g was born at 35 weeks’ gestation with an unexpected large abdominal wall skin defect. This had been an in vitro fertilisation pregnancy in which two embryos were returned, one of which divided resulting in two monochorionic fetuses and a further third fetus. Interstitial laser therapy was performed at 13 weeks to …
BackgroundCongenital anomalies are the fifth leading cause of mortality in children younger than 5 years globally. Many gastrointestinal congenital anomalies are fatal without timely access to neonatal surgical care, but few studies have been done on these conditions in low-income and middle-income countries (LMICs). We compared outcomes of the seven most common gastrointestinal congenital anomalies in low-income, middle-income, and high-income countries globally, and identified factors associated with mortality.MethodsWe did a multicentre, international prospective cohort study of patients younger than 16 years, presenting to hospital for the first time with oesophageal atresia, congenital diaphragmatic hernia, intestinal atresia, gastroschisis, exomphalos, anorectal malformation, and Hirschsprung's disease. Recruitment was of consecutive patients for a minimum of 1 month between October, 2018, and April, 2019. We collected data on patient demographics, clinical status, interventions, and outcomes using the REDCap platform. Patients were followed up for 30 days after primary intervention, or 30 days after admission if they did not receive an intervention. The primary outcome was all-cause, in-hospital mortality for all conditions combined and each condition individually, stratified by country income status. We did a complete case analysis.FindingsWe included 3849 patients with 3975 study conditions (560 with oesophageal atresia, 448 with congenital diaphragmatic hernia, 681 with intestinal atresia, 453 with gastroschisis, 325 with exomphalos, 991 with anorectal malformation, and 517 with Hirschsprung's disease) from 264 hospitals (89 in high-income countries, 166 in middle-income countries, and nine in low-income countries) in 74 countries. Of the 3849 patients, 2231 (58·0%) were male. Median gestational age at birth was 38 weeks (IQR 36–39) and median bodyweight at presentation was 2·8 kg (2·3–3·3). Mortality among all patients was 37 (39·8%) of 93 in low-income countries, 583 (20·4%) of 2860 in middle-income countries, and 50 (5·6%) of 896 in high-income countries (p<0·0001 between all country income groups). Gastroschisis had the greatest difference in mortality between country income strata (nine [90·0%] of ten in low-income countries, 97 [31·9%] of 304 in middle-income countries, and two [1·4%] of 139 in high-income countries; p≤0·0001 between all country income groups). Factors significantly associated with higher mortality for all patients combined included country income status (low-income vs high-income countries, risk ratio 2·78 [95% CI 1·88–4·11], p<0·0001; middle-income vs high-income countries, 2·11 [1·59–2·79], p<0·0001), sepsis at presentation (1·20 [1·04–1·40], p=0·016), higher American Society of Anesthesiologists (ASA) score at primary intervention (ASA 4–5 vs ASA 1–2, 1·82 [1·40–2·35], p<0·0001; ASA 3 vs ASA 1–2, 1·58, [1·30–1·92], p<0·0001]), surgical safety checklist not used (1·39 [1·02–1·90], p=0·035), and ventilation or parenteral nutrition unavailable when needed (ventilation 1·96, [1·41–2·71], p=0·0001; parenteral nutrition 1·35, [1·05–1·74], p=0·018). Administration of parenteral nutrition (0·61, [0·47–0·79], p=0·0002) and use of a peripherally inserted central catheter (0·65 [0·50–0·86], p=0·0024) or percutaneous central line (0·69 [0·48–1·00], p=0·049) were associated with lower mortality.InterpretationUnacceptable differences in mortality exist for gastrointestinal congenital anomalies between low-income, middle-income, and high-income countries. Improving access to quality neonatal surgical care in LMICs will be vital to achieve Sustainable Development Goal 3.2 of ending preventable deaths in neonates and children younger than 5 years by 2030.FundingWellcome Trust.
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