The objective of this paper is a retrospective study of all infants treated for congenital chylothorax at the Royal Children's Hospital (RCH), Melbourne, Australia and King Fahad National Guard Hospital (KFNGH), Riyadh, Kingdom of Saudi Arabia. The charts of all infants with congenital chylothorax admitted to RCH over a period of 13 years, June 1982-August 1994, and admissions to KFNGH over a 7-year period, June 1992-August 1998 inclusive, were reviewed including management outcome and complications. There were 19 infants, 13 from RCH and 6 from KFNGH; 11 females and 8 males. Three infants were managed antenatally. Fifteen infants presented immediately after birth. Seven were born with hydrops fetalis, 6 infants had syndromes and 10 infants were born prematurely. Regular infant feeding formula and/or breast milk were used successfully in 12 infants, while in 7 infants medium chain triglycerides (MCT) rich formula was used. Sixteen infants were mechanically ventilated with 75% of them ventilated for < or = 28 days. Fifteen infants received total parenteral nutrition (TPN), and in 80% for < or = 32 days. Hydropic infants had longer duration of mechanical ventilation and hospital stay with mean (range) of 33.9 (3-120) and 115 (23-225) days, respectively, compared with 18 (1-62) and 34.3 (14-88) days for nonhydropic infants. Five infants underwent surgery with failure in four. Sepsis and bronchopulmonary dysplasia were the main complications. The survival rate was 100% regardless of the mode of therapy. The prognosis of Isolated congenital chylothorax in term, and preterm infants is good even in the presence of hydrops. Breast milk and/or regular infant feeding formula should be used initially before proceeding to MCT-rich formula, which may be necessary in some cases. Surgery should be considered if conservative management of congenital chylothorax fails after 4-5 weeks.
Umbilical anomalies arise from fetal structures such as the omphalomesenteric duct (OMD) or urachus or from failure of closure of the umbilical fascial ring. Persistence of the OMD may lead to several anomalies including umbilical sinus, umbilical cyst, Meckel's diverticulum, or patent OMD (POMD). A POMD is usually associated with the ileum, but rarely may be with the caecum or appendix. We describe a POMD of the vermiform appendix and discuss the possible pathogenesis and management.
Despite many advances in the management of congenital diaphragmatic hernia (CDH), the condition carries a mortality rate of 40-50% usually consequent to pulmonary hypoplasia and/or persistent pulmonary hypertension. Several centers have reported improved survival with preoperative stabilization and delayed surgery, which is now an accepted method of management. This is a retrospective analysis of all neonates with respiratory distress at birth due to CDH who were treated at our institution with neither extracorporeal membrane oxygenation nor nitric oxide being used. The medical records of all neonates with CDH and respiratory distress at birth who were treated at this institution from August 1, 1992 through March 1, 1997 were reviewed. There were 21 patients, 11 male and 10 female. There were 17 full-term and 4 premature infants; two premature infants at 30 and 34 weeks' gestation were not resuscitated because of severe associated congenital anomalies. Surgery was performed from 5 to 144 hr (mean 45 hr) in 18 infants. One infant died during preoperative stabilization from severe pulmonary hypoplasia and pulmonary hypertension and one infant died postoperatively from the same conditions. Seventeen of 19 infants (89.5%) survived and were discharged home. Three infants (17.6%) who failed to thrive due to severe gastroesophageal reflux (GER) required fundoplication. Eleven infants (64.7%) who had sepsis proven by blood culture responded satisfactorily to appropriate antibiotics. Preoperative stabilization and delayed surgery has been a satisfactory form of management in our series. The significant complication was sepsis, which must be addressed.
Prognosis of congenital infantile cortical hyperostosis (Caffey's disease) is poor particularly in premature babies. Two cases are presented of congenital Caffey's disease in premature babies. The first baby was hydropic at birth and had cortical hyperostosis involving the mandible and long bones of right upper limb and both lower limbs. The second baby had cortical hyperostosis of the nasal bones causing severe nasal nonchoanal stenosis that needed surgery, in addition to involvement of long bones of the four extremities. Both babies recovered from the disease and were discharged home well. These cases suggest that the improved outcome of congenital of infantile cortical hyperostosis may reflect improvement of neonatal mechanical ventilation and availability of neonatal total parenteral nutrition.
The incidence of severe hyperbilirubinaemia was significantly higher among the G6PD-deficient Saudi infants born at term than in non-deficient babies (34% vs 9%) (p less than 0.005). No apparent offending factors were detected in either the babies or their mothers. All babies who developed hyperbilirubinaemia did so during the 1st week of life. The highest mean bilirubin level for all jaundiced G6PD-deficient babies was recorded on the 4th postnatal day. Although the incidence of severe hyperbilirubinaemia among our neonates was relatively high, only two of them (7%), a boy and a girl, required exchange transfusions. Five of 29 jaundiced babies with G6PD deficiency were readmitted after discharge because of significant jaundice. One required exchange transfusion. Since G6PD deficiency seems to be a relatively common cause of neonatal jaundice in Saudi newborns, early detection of this enzymopathy by cord blood screening is justified to avoid morbidity and deaths.
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