Over a seven month period in 1986, 164 (1.1%) of a total 15,131 neonates delivered in the Maternity Hospital, Kuala Lumpur were born to mothers with prolonged rupture of the fetal membranes. Septic work-ups were performed on 163 of these babies shortly after birth, 77 of them had no bacteriological, radiological or other clinical evidence of infection. The remaining 86 had additional history of illness. Nine of these 86 babies had proven infection: four with septicemia, one with meningitis and four others with congenital pneumonia. This study showed that neonates had risk of infection only when maternal history of prolonged rupture of the fetal membranes was associated with other clinical evidence of infection. However, even in this group of neonates at risk, 89.5% of them were not infected. Therefore, there is still a need to develop a simple, rapid, reliable and cheap laboratory method to help early identification of the infected from the non-infected neonates in order to prevent unnecessary antibiotic treatment and hospital stay.
The problems of pediatric cardiology in Asia can be summarized as follows. In the countries like India, Indonesia, Malaysia, Pakistan and some other countries especially with government support like Thailand, the priority is primary health care. Except in Japan, cardiac care for children is not well supported financially. Training in pediatric cardiology is not readily available. Consequently small numbers of pediatric cardiologists, pediatric cardiac surgeons and active centres are available even in Japan. It is importannt to reduce the incidence of rheumatic fever and rheumatic heart disease by prevention of streptococcal infections and to increase the standard of living and environment. Transportation and referral system diagnosis, and treatment and follow-up is a special problem in neonatal care in relation to heart disease. Concentration of physicians, medical schools and cardiac centers in urban areas, old customs and poor understanding of people about the treatability and preventability of heart diseases in children complicate the improvement in the management of children with heart diseases.
A thirty six week gestation male baby weighing three kilogram was born to a twenty five year old mother by spontaneous vaginal delivery. At four hours of life, the baby developed respiratory distress with cyanosis and was admitted to the neonatal intensive care unit. There was clinical and radiological evidence of bilateral pleural effusion. Thoracentesis revealed a transudate. Repeated thoracentesis was necessary to relieve the respiratory distress. Subsequently, multi resistant Klebsiella aerogenes was isolated from the blood. The baby expired due to gram negative sepsis.
Gastrointestinal pathology leading to the death in paediatric age group is uncommon. The diseases that encountered were mostly intestinal obstruction, peritonitis and gastrointestinal bleeding. Due to the severe symptoms, most of the patients presented to hospital in time and were treated appropriately. However, with the presence of contributing factors, certain gastrointestinal pathology can progress rapidly leading to the death. We report a rare case of intestinal volvulus in a 3 years old girl where the deceased presented with one day short history of vomiting before her demise. The contributing factors were bronchopneumonia sepsis and underlying intestinal malrotation identified via post-mortem examination.
This study examined the trend of major congenital anomalies (CA) in the state of Penang using the ICD 10 database from 1999 to 2004. The data was collected from various health centres and hospitals. The aim was to study the magnitude of the problem for congenital anomalies in the state of Penang in terms of trends and also to calculate the incidence rate by districts. If a trend was noticed, this in turn will determine whether to carry out further in-depth studies in the future and to find out the linkages to the environment if any.
A prospective study was done to determine the incidence of cardiovascular malformations in neonates with Down's syndrome. 17/34 (50%) of the babies with Down's syndrome born at the Maternity Hospital, Kuala Lumpur, Malaysia had congenital heart defects. These included 7 cases of ventricular septal defect (VSD), 3 cases of patent ductus arteriosus (PDA), 2 cases of atrio-ventricular canal defect, 2 cases of ventricular septal defect with patent ductus arteriosus, 1 case of hypertrophic cardiomyopathy, 1 case of hypertrophic obstructive cardiomyopathy and 1 case of complex cyanotic heart. Only 8/17 (47%) of these babies had any clinical signs suggesting underlying cardiac defects. In view of the common occurrence of cardiac anomalies, it is recommended that echocardiographic screening should be carried out on all neonates with Down's syndrome.
Teratoma of the neck is a rare extra-gonadal teratoma. Although it can be seen in adult, most of the cases present in fetal life or early childhood. Grading and classification of teratoma is important for management and prognosis. Decision making between mature and immature teratoma is sometime challenging if it is not composed of extensive unusual neuroectodermal tissue component. We reported a rare case of huge congenital mature cystic teratoma composted of various neuroectodermal tissues in an 8-month-old boy; it was located at the lateral neck and mimicked congenital cystic hygroma. A literature review was done to grade and classify the tumour.
Matched MeSH terms: Head and Neck Neoplasms/congenital; Teratoma/congenital; Lymphangioma, Cystic/congenital
Rubella infection in pregnant women during the first trimester of pregnancy can lead to fetal anomalies, commonly known as congenital rubella syndrome (CRS). The objective of our study was to analyze the serological test results among infants suspected of having CRS aged < or = 12 months compared with their clinical status. Between January 2002 and December 2011, 3,279 serum samples from infants aged < or = 12 months from government hospitals in Malaysia were examined for rubella specific IgM and IgG antibodies using a Axsym, automated analyzer (Abbott Laboratories). Forty-eight samples were positive for rubella specific IgM antibodies and 494 samples were positive for rubella specific IgG antibodies. These were then age stratified and their clinical history reviewed for any CRS symptoms. Fifteen of 38 rubella IgM positive infants (39.5%) aged < 3 months, had a clinical appearance compatible with CRS. However, only 1 IgM positive infant aged 3 to 6 months and one infant aged 7 to 11 months had clinical appearance compatible with CRS. The most common abnormal findings in these cases were congenital heart defects and cataracts. Forty-eight point eight percent of IgM positive cases and 53.1% of IgG positive cases, had inadequate information in the chart to determine the presence of CRS. Clinical findings and timely laboratory diagnosis to determine the presence of CRS are important in infants born with congenital defects. Physicians should also be aware of the appropriate interpretation of these findings.
Congenital adrenal hyperplasia (CAH) is the commonest cause of ambiguous genitalia for female newborns and is one of the conditions under the umbrella term of "Disorders of Sex Development" (DSD). Management of these patients require multidisciplinary collaboration and is challenging because there are many aspects of care, such as the most appropriate timing and extent of feminizing surgery required and attention to psychosexual, psychological, and reproductive issues, which still require attention and reconsideration, even in developed nations. In developing nations, however, additional challenges prevail: poverty, lack of education, lack of easily accessible and affordable medical care, traditional beliefs on intersex, religious, and cultural issues, as well as poor community support. There is a paucity of long-term outcome studies on DSD and CAH to inform on best management to achieve optimal outcome. In a survey conducted on 16 patients with CAH and their parents in a Malaysian tertiary center, 31.3% of patients stated poor knowledge of their condition, and 37.5% did not realize that their medications were required for life. This review on the research done on quality of life (QOL) of female patients with CAH aims: to discuss factors affecting QOL of female patients with CAH, especially in the developing population; to summarize the extant literature on the quality of life outcomes of female patients with CAH; and to offer recommendations to improve QOL outcomes in clinical practice and research.
A 3-year-old child presented with congenital bilateral facial nerve palsy with bilateral profound sensorineural hearing loss. High Resolution Computed Tomogram (HRCT) of the temporal bones found bilateral atresia of cochlear nerve canals, incomplete partition of the cochleae and narrow facial nerve canals. Magnetic resonance imaging (MRI) revealed bilateral hypoplasia of facial nerves and aplasia of both vestibulocochlear nerves. There have been no other reported cases with this presentation. The possible aetiology and treatment options for the patient are discussed. We highlighted the review of aplasia/hypoplasia of the facial nerve and hypoplasia of cochlear nerve canal.
Peripartum cardiomyopathy (PPCM) is an uncommon form of congestive heart failure, afflicting obstetric patients around the time of delivery. The epidemiology of PPCM is infrequently reported. To the best of our knowledge, there has been no report from Asia.
The incidence of congenital dislocation of the hip (CDH) in Singapore and Malaysia has been reported as being lower than in the West. In our hospital, we have seen an increasing number of congenital hip dislocation as well as dysplastic hips. We undertook a prospective study from December 1989 to December 1994 of 20,000 live births. The neonates were all screened by a consultant neonatologist and the findings were confirmed by a consultant paediatric orthopaedic surgeon. All babies had plain X-rays at 3 months and an acetabular index (AI) of 30 degrees or more was considered dysplastic. All babies with positive signs were followed up for 1 year and again had radiographs taken at 1 year. Comparison of plain X-rays and ultrasound assessment in a subgroup of 130 neonates showed that 64% of patients with AI > 20 degrees had hip dysplasia by ultrasonographic (alpha angle < 60 degrees) The incidence of dysplastic hips was 16.8 per 1000 live births. The overall incidence of neonates with dislocated hips was 4.7 per 1000 live births. The Malays were most affected with an incidence of 5.4 per 1000 live births. The incidence of developmental dysplasia of the hip in Singapore is higher than previously reported, with the Malays having the highest incidence. A significant number of babies with clicking hips have radiological evidence of acetabular dysplasia (AI > 30 degrees). One-third of the babies' hips were still dysplastic at 1 year of age. A well-organised screening programme with experienced examiners has proved to be useful in making early and accurate clinical diagnosis.
Matched MeSH terms: Hip Dislocation, Congenital/diagnosis; Hip Dislocation, Congenital/epidemiology*; Hip Dislocation, Congenital/prevention & control
The prevalence of ocular abnormalities was studied in 165 children from a Malaysian school for the deaf. Ninety-five children (57.6%) had one or more ocular abnormalities. Rubella retinopathy was the commonest form of ocular abnormality (35.2%). Refractive errors were found in 23 children (13.9%). Refractive errors in the rubella group were significantly more common than in the non-rubella group of deaf children (p < 0.001) (chi 2 test). Thirteen children had congenital anomalies causing significantly impaired vision. Ophthalmological examination of deaf children helps in the detection of cases with rubella eye signs and thus helps to identify the cause of deafness. Since deaf children are at greater risk of visual and ocular abnormalities, periodical ophthalmological examination should be carried out in these children.
One hundred and sixty-five children from the school for the deaf were screened to find out the aetiology of deafness. The screening included routine ophthalmological examination. Aetiology was unknown in 33 children (20%). Prenatal infection was found to be the cause of deafness in 61 cases (36.96%). Rubella eye signs were found in 59 children (35.76%), whereas the history of rubella infection during the first trimester of pregnancy was found in only 33 of these cases (55.93%). Perinatal complications were seen in 20 children (12.12%). Twenty children (12.12%) had meningitis during their early childhood period. Routine ophthalmological examination helped us to pick up cases with rubella eye signs. Ophthalmological examination should be included as part of any study done to find out the cause of deafness.
Between February 1990 and May 1993, 13 cases of early congenital syphilis (ECS) were managed in the Paediatrics Unit, University Hospital, Kuala Lumpur. Twelve mothers were unbooked with 10 inborn babies. Only one mother had antenatal booking at this hospital but she defaulted antenatal follow-up. Several risk factors associated with ECS were identified: inadequate or no prenatal care (5/13), failure to repeat a serological test for syphilis in the third trimester when it was tested negative at first booking (5/13), sexual promiscuity, substance abuse and a past history of contracting sexually transmitted disease. All 10 mothers who had their serological test repeated at delivery were found to have a positive VDRL and TPHA. Adequate antenatal care early referral of infected, expectant mothers for treatment, and a repeat serological test for syphilis could have prevented these cases of ECS.
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.