Three children with cardiac tumors are described: a 12-year-old female child who had left atrial myxoma, and two males having rhabdomyoma of the right ventricle associated with tuberous sclerosis. The child with left atrial myxoma was symptomatic and the tumour was subsequently excised. The other two children with rhabdomyoma were managed conservatively.
We describe three children with Noonan syndrome with cardiopathy. One female child had cardiopathy and ocular abnormalities. The other two male children had congenital heart disease of which one had uncommon association of tricuspid valve dysplasia with regurgitation associated with endocardial cushion defect. Karyotypes of the female and one of the male children were normal. The growth hormone and thyroid hormone studies in the first and second male children were normal. All the three children were managed conservatively and followed-up.
Tracheobronchial foreign bodies, which are common in children, are a leading cause of accidental deaths in children under four years of age. Three cases of tracheobronchial foreign bodies in children less than two years old are described. One of the foreign bodies was unsuspected; the other two were probably related to food habits. All three cases improved without sequelae following prompt bronchoscopic intervention. Young children should not be given food containing bones or hard chitinous shells.
A 7-year old female child was admitted for recurrent bronchopulmonary since one week of life. She was diagnosed to have ventricular septal defect and was treated conservatively. At seven years of life, repeat echocardiogram revealed a large perimembranous ventricular septal defect, absent pulmonary valve with overriding of aorta, narrow pulmonary artery annulus, and dilated main pulmonary artery and its branches. She was treated conservatively, discharged and follow-up at the National Heart Institute Kuala Lumpur, for corrective surgery.
A 3-day-old male baby with neonatal necrotising enterocolitis had a central line (Cavafex No. 18) inserted through the left femoral vein for administration of parenteral nutrition. Forty-eight hours later, he developed progressive abdominal distention with evidence of free abdominal fluid. A contrast injection done through the left femoral vein revealed retroperitoneal leakage of contrast from a traumatic rupture of the left iliac vein. The intravenous catheter was removed and he was treated conservatively with eventual recovery.
A term female neonate with monolateral adrenal haemorrhage associated with haemorrhagic disease of newborn is described. Diagnosis and follow-up of adrenal haemorrhage was done clinically and sonographically which revealed reduction in the size of adrenal haematoma over a month with no evidence of adrenal insufficiency. She was discharged well and followed up.
A post-dated intra-uterine growth retarded male Malay baby was born to a 30-year-old mother gravida II by Caesarean section. Her previous pregnancy ended in still-birth. The baby was severely asphyxiated at birth. He was intubated and immediately admitted to the neonatal intensive care unit. He had anasarca, anaemia, purpura and firm, massive hepatosplenomegaly. X-rays revealed ascites and bilateral metaphysiitis of the long bones. The haemoglobin level was 5.0 gm/dl and PCV 18.3%. Coombs' test was negative. Prothrombin time (PT) and partial thromboplastin time (PTT) were prolonged. The baby and mother were positive for Venereal Disease Research Laboratory (VDRL) and the treponema pallidum haemagglutination assay (TPHA) tests. The baby was actively resuscitated but expired at three and a half hours of life due to overwhelming sepsis associated with severe anaemia and disseminated intravascular coagulation.
We describe two Malay male term neonates with congenital limb reduction defects. The first neonate had hypodactyly of limbs associated with micrognathia, microstomia, glossopalatine ankylosis and congenital mitral stenosis. He developed gram-negative septicaemia and died on day 14 of life. The second neonate had tetraperomelia without any other associated congenital abnormality. He developed staphylococcal skin infection which was treated conservatively. Very few cases of congenital limb reduction defects have been reported in the Asian population and we are not aware of any other reports describing Malay infants with the congenital abnormalities described in this report.
A single nostril associated with alobar holoprosencephaly is a rare congenital lesion. This paper reports two female term neonates with holoprosencephaly. The first neonate asphyxiated at birth had a single nostril, hypotelorism, posterior cleft palate, inferonasal coloboma of the iris and disc and persistent tunica vasculosa lentis. The other neonate had cleft lip and palate and recurrent convulsions. Both neonates had gross motor and developmental delay. Cranial sonography and CT scan showed features consistent with alobar holoprosencephaly. Karyotyping for the first neonate and her family members was normal. Both of them were treated conservatively and supervised.
A 37-week gestation male boy was born to a gravida seven para six mother by spontaneous vertex delivery at home. The baby cried at birth. On day 3 of life, he was admitted for respiratory distress. Physical examination revealed ectrodactyly, thin dry skin, anomalous tear duct with cardiomegaly. X-ray revealed absent radii, cardiomegaly and hemivertebra at L1. Echocardiogram revealed perimembranous type of ventricular septal defect. A diagnosis of Ectodermal Dysplasia Ectrodactyly Clefting Syndrome with ventricular septal defect was made. He was managed conservatively in the nursery. However, he expired on day 27 of life following short spell of fever apnoeic episode due to neonatal sepsis.