Neonatal sigmoid volvulus.

BACKGROUND: Neonatal sigmoid volvulus is a rare entity. It is associated with Hirschsprung's disease. Presentation is acute abdominal distention, vomiting and obstipation. Abdominal radiograph will show the "coffee bean" sign, but this is frequently missed and the diagnosis requires a high index of suspicion. Treatment options include contrast enema, colonoscopy or laparotomy, depending on the condition of the baby and local availability. POPULATION AND RESULTS: During the last 6years, 6 infants with sigmoid volvulus were treated in our department. Four presented during the first 48h since birth, and 2 presented at the age of 2 and 7weeks of age. One child was operated and 5 had primary contrast enema with radiologic de-volvulus. Rectal biopsy was performed in all cases; three children had Hirschsprung's disease. Those with normal biopsies responded well to rectal washouts. Two patients had early one stage transanal pullthrough and one had 2 further occasions of sigmoid volvulus prior to definitive surgery. All three recovered with an uneventful course. CONCLUSIONS: Neonatal sigmoid volvulus requires a high level of suspicion. Contrast enema is efficient for primary de-volvulus. Rectal biopsy should be performed and if positive for Hirschsprung's disease, surgery should be performed sooner rather than later.

[PREDICTION OF LATE ONSET SEPSIS IN VERY LOW BIRTH WEIGHT INFANTS BY A SOFTWARE APPLICATION--ARE WE THERE YET?].

INTRODUCTION: Early prediction of late onset sepsis is imperative in order to improve survival and reduce long-term complications. Since clinical deterioration is detrimental, empiric antibiotic treatment is initiated once sepsis is suspected. Symptoms that may indicate invasive infection are non-specific. Previous risk scores offered to improve clinical decision-making but provided low predictive values. AIMS: To evaluate the quantitative early alert of software application compared to clinical judgment by the treating physician, and the "gold standard" of positive blood and/or positive cerebrospinal fluid. METHODS: Weight, heart and respiratory rates, episodes of bradycardia and desaturation, and temperature were collected for each neonate and loaded daily into the system for a period of 30 days by a registered nurse. The medical team and the registered nurse were blind to the system alerts. Analysis of the correlation between the software alerts, the clinical suspicion of sepsis and bacteremia was conducted. RESULTS: Forty-five very low birth weight consecutively born infants who did not have early onset sepsis and survived, were evaluated, of whom 17 infants had culture proven bloodstream infection. The software positive predictive value was 6%, 23%, 31%, at 12, 24, 48, hours respectively for alerts approximately to positive cultures. The positive predictive value of clinical suspicion of LOS was 28% but increased from 25% with low levels of clinical suspicion to 34% with high levels of clinical suspicion. DISCUSSION: The software application did not improve sepsis prediction. However, further trials may develop a more accurate algorithm that will alert the physician to be more attentive to infants in special cases.

Congenital fulminant Kaposiform hemangioendothelioma of the leg.

Kaposiform hemangioendothelioma is a rare locally aggressive vascular tumor associated with Kasabach Merritt syndrome. We present a case of congenital Kaposiform hemangioendothelioma of the leg in a female infant who was born to a mother treated with various medications including etanercept, a TNF antagonist, due to rheumatoid arthritis. The neonate suffered from a fulminant form of Kasabach Merritt syndrome with disseminated intravascular coagulation (DIC) resulting in multi-organ failure which led to her demise.