Changing survival rate of infants born before 26 gestational weeks: single-centre study

This study aimed to evaluate the changing survival rate and morbidities among infants born before 26 gestational weeks at the Sultan Qaboos University Hospital [SQUH] in Muscat, Oman. This retrospective study assessed the mortality and morbidities of all premature infants born alive at 23-26 gestational weeks at SQUH between June 2006 and May 2013. Infants referred to SQUH within 72 hours of birth during this period were also included. Electronic records were reviewed for gestational age, gender, birth weight, maternal age, mode and place of delivery, antenatal steroid administration, morbidity and outcome. The survival rate was calculated and findings were then compared with those of a previous study conducted in the same hospital from 1991 to 1998. Rates of major morbidities were also calculated. A total of 81 infants between 23-26 gestational weeks were admitted to the neonatal unit during the study period. Of these, 58.0% were male and 42.0% were female. Median gestational age was 25 weeks and mean birth weight was 770 +/- 150 g. Of the 81 infants, 49 survived. The overall survival rate was 60.5% compared to 41% reported in the previous study. Respiratory distress syndrome [100.0%], retinopathy of prematurity [51.9%], bronchopulmonary dysplasia [34.6%], intraventricular haemorrhage [30.9%] and patent ductus arteriosus [28.4%] were the most common morbidities. The overall survival rate of infants between 23-26 gestational weeks during the study period had significantly improved in comparison to that found at the same hospital from 1991 to 1998. There is a need for the long-term neurodevelopmental follow-up of premature infants

Coarctation of the aorta, known yet can be missed

The clinical presentation of coarctation of aorta [CoA] is well known; however, it is the most common congenital heart disease in the newborn period to be missed, with significant mortality and morbidity associated with missing the diagnosis. We report a 20-day-old newborn boy who presented with congestive heart failure and weak femoral pulses. Chest X-ray [CXR] showed cardiomegaly and pulmonary edema and electrocardiography [ECG] showed extreme right axis deviation and absent left ventricular forces in the left precordial leads. Based on these, clinical suspicion of coarctation of aorta was made and confirmed by echocardiography. After initial stabilization with prostaglandin E2 infusion, child underwent urgent coarctation of aorta repair with uneventful post-operative course. High index of suspicion for coarctation of aorta in a newborn with such presentation is required and urgent referral to Pediatric Cardiology and cardiac surgery center is mandatory to reduce the morbidity and mortality associated with missing the diagnosis

Successful treatment of cardiomyopathy due to very long-chain acyl-coa dehydrogenase deficiency: first case report from Oman with literature review

Very long-chain acyl-CoA dehydrogenase deficiency [MIM 201475] is a severe defect of mitochondrial energy production from oxidation of very long-chain fatty acids. This inherited metabolic disorder often presents in early neonatal period with episodes of symptomatic hypoglycemia usually responding well to intravenous glucose infusion. These babies are often discharged without establishment of diagnosis but return by 2-5 months of age with severe and progressive cardiac failure due to hypertrophic cardiomyopathy with or without hepatic failure and steatosis. An early diagnosis and treatment with high concentration medium chain triglycerides based feeding formula can be life saving in such patients. Here, we report the first diagnosed and treated case of Very long-chain acyl-CoA dehydrogenase deficiency in Oman. This infant developed heart failure with left ventricular dilation, hypertrophy and pericardial effusion at the age of 7 weeks. Prompt diagnosis and subsequent intervention with medium chain triglycerides-based formula resulted in a reversal of severe clinical symptoms with significant improvement of cardiac status. This treatment also ensured normal growth and neurodevelopment. It is stressed that the disease must be recognized by the pediatricians and cardiologists since the disease can be identified by Tandem Mass Spectrometry; therefore, it should be considered to be included in expanded newborn screening program, allowing early diagnosis and intervention in order to ensure better outcome and prevent complications.