Background: Kaposiform Haemangioendothelioma (KHE) is a very rare, benign but locally aggressive vascular tumour. Its incidence is 1/100000, and it is most commonly found in the cutaneous tissues of the extremities. Presentation: A 6-month-old presented acutely after 2 days of increasing abdominal distension. Apart from distension, clinical examination was unremarkable. An abdominal x-ray demonstrated massive gaseous distension of bowel loops. A contrast enema demonstrated an irregular stenosis within the sigmoid colon. An urgent laparotomy was performed. Management: A thick and very inflammatory stricture was resected. Histopathology confirmed this as Kaposiform Haemangioendothelioma with margin involvement. Patient had a complicated post-operative period due to prolonged sepsis resulting in a cardiac arrest and two return journeys to theatre. An MRI brain was performed after patient stabilisation, demonstrating bilateral cerebral infarcts - the patient went on to develop cerebral palsy. Discussion: Mainstay management of KHE is primary resection. It is associated with Kasabach-Merritt Phenomenon - a consumptive coagulopathy, which is present in 70% of KHE patients and has an associated 10% mortality rate. Conclusion: There are a small number of cases of KHE with gastrointestinal involvement in the literature. A number of these presented with bowel obstruction but no other had such a complicated post-operative course.

AimAerodigestive clinics (ADCs) are multidisciplinary programmes for the care of children with complex congenital or acquired conditions affecting breathing, swallowing and growth. Our objective was to describe the demographic, clinical, etiological and investigational profile of children attending the inaugural ADC at a tertiary paediatric centre in Queensland.MethodsChildren referred to the ADC at Queensland Children's Hospital from August 2018 to December 2019 were included. Data on clinical, growth and lung function parameters, bronchoscopy and upper gastrointestinal endoscopy findings, thoracic imaging and comorbidities were retrospectively analysed.ResultsFifty‐six children (median (range) age 4 years (3 months–15 years); 18 female) attended the ADC during this 17‐month period. Forty‐six (82%) children had previous oesophageal atresia with tracheo‐oesophageal fistula; 43 of these were type C. Previous isolated oesophageal atresia, congenital diaphragmatic hernia and congenital pulmonary malformation were the underlying disorder in three (5%) children each, with one child having a repaired laryngeal cleft. Vertebral Anal Tracheo Esophageal Renal Limb anomalies (VACTERL)/Vertebral Anal Tracheo Esophageal renal anomalies (VATER) association was seen in 21 (38%) children. Growth was adequate (median weight and body mass index z‐score −0.63 and −0.48, respectively). Thirty‐four (61%) children reported ongoing wet cough, with 12 (21%) requiring previous hospital admission for lower respiratory tract infection. Fourteen (25%) had bronchiectasis on computed tomography chest and 33 (59%) had clinical tracheomalacia, apparent on bronchoscopic examination in 21 patients. Dysphagia was reported in 15 (27%) children, 11 (20%) were gastrostomy feed‐dependent and 5 (9%) had biopsy‐proven eosinophilic oesophagitis.ConclusionHigh proportion of children attending the ADC have ongoing respiratory symptoms resulting in chronic pulmonary suppuration and bronchiectasis. Potential benefits of this model of care need to be studied prospectively to better understand the outcomes.

BACKGROUND:A sutureless ward reduction (SWR) protocol was implemented in the neonatal intensive care unit of a tertiary level hospital in 1999. Although the short-term outcomes associated with SWR have been documented, the long-term outcomes are unknown.METHODS:Retrospective data were collected from the medical records of all neonates with gastroschisis from September 1999 to December 2010. Data on their growth and development and the prevalence of any health problems were collected.RESULTS:Eighty-eight patients with gastroschisis were managed over an 11 year period. Forty-four of these patients received SWR, with 2 deaths in the neonatal period. In the 42 survivors, 35 patients were reviewed at a median age of 7 years and 10 months (range, 6-134 months; interquartile range, 37-124 months). One patient experienced failure to thrive and developmental delay, and later died of a medical complication. Thirty-two patients (91.4%) developed an umbilical hernia, only 2 of whom required umbilical herniotomy. Four patients (11.4%) developed small bowel obstruction, all within the first year.CONCLUSION:Most patients with SWR exhibited normal growth with minimal bowel complications. Despite the high incidence of umbilical hernia, the majority resolved spontaneously and did not require subsequent herniotomy.

Milk curd obstruction is one of the less common causes of neonatal bowel obstruction. It has been described in premature infants who received high caloric formula feeds. We report presentation, management and outcome of premature neonates who developed milk curd obstruction while being fed fortified expressed breast milk.A retrospective case note review of babies who were treated for milk curd obstruction in Royal Children's Hospital and Mater Children's Hospital in Brisbane between 2001 and 2007 was performed.Nine preterm neonates developed milk curd obstruction (mean gestational age 27 weeks). All babies received ortified expressed breast milk. Symptoms presented were those of bowel obstruction in the majority of cases. Laparotomy was required in eight babies, one had a pre-existing ileostomy that was washed out. Two babies died shortly after surgery, while two followed several months later.The diagnosis of milk curd obstruction should be considered in all premature babies with signs of bowel obstruction who are fed expressed breast milk with caloric fortification.