experience with four cases of split notochord syndrome and review of literature

To evaluate the rarity of split notochord syndrome, to access frequency of various associated anomalies and to find out compatibility of this condition with survival. All those cases who presented with vertebral abnormalities and those who presented with spina bifida associated with gastrointestinal and urological abbarasion were further investigated for possible association of split notochord syndrome. The study was conducted at the Department of Paediatric surgery the Children Hospital Pakistan Institute of Medical Sciences from 1987-2001. All those children and aborted fetuses who had gross spinal cord defect, accessory limb, or teratoma like lesion associated with mylomeningocele or those who had gross duplication of pelvic genitourinary organs were further assessed by detailed examination of the local lesions and further radiological, contrast and histopathological studies. We could identify four such cases, each had completely different anatomical presentation. One was an aborted fetus of 24 weeks gestation. Another child died soon after the delivery. One had pelvic skeletal and organs duplication with enteric fistulae, double exstrophy of bladder, double penis, and split lumbosacral spine, and the fourth case had complete duplication of external genitourinary organs in the form of fully formed double vulvae and split spinal column in the region of sacrum and coccyx. Split notochord syndrome is perhaps rare abnormality, but often it may not be identified especially if it is not specifically looked for. Fifty percent of our cases were either aborted or still-born, therefore to assess the actual incidence of split notochord syndrome it is suggested that study should be conducted by antenatal ultrasounds and all fetuses who show spinal column defects and aborted should be examined with detailed dissection and histopathology analysis of the tissues

Degenerative brain diseases and neuroimaging in children

The degenerative brain diseases represent one of the most common problems in paediatric neurology. Findings of computerized tomography and magnetic resonance imaging in 39 children with progressive neurological disorders were evaluated. Twenty five patients were boys and 14 girls. The ages ranged from 10 weeks to 12 years [mean 6.5 years]. Children had either CT scan [34 cases] or MRI brain [5 cases]. Thirty [77%] of CT [27 cases] and MR[[3 cases] were abnormal, and abnormalities included demyelination, cortical atrophy, calcification, hypo/hyperdense areas and cerebellar atrophy. MR[or CT scan brain, whenever possible, should be part of evaluation of a child who is suspected of having a neurodegenerative disorder in order to strengthen on diagnosis

Development delay in infants and young children

To determine the prevalence and risk factors of developmental delay in infants and young children seen at Paediatric Neurology Clinic in Children's Hospital, Islamabad. It was a retrospective case analysis. Children's Hospital Institute of Medical Sciences, Islamabad between January and December, 1995. Three hundreds and eighty three children presented with developmental delay. Motor delay [88 percent] and mental retardation [72 percent] were the commonest disabilities. The most important risk factors included cerebral palsy [38 percent] and neurodegenerative disorders [33 percent]. Developmental delay in infants and children is a significant problem. Improvements in primary health care, prenatal and perinatal services can play an important role in prevention

EEG and brain death in children

In a retrospective study, clinical and neuropthological characteristics of 25 children who met the criteria for brain death and had a [flat] EEG were analyzed. Age of the patients varied from 3 months to 12 years. All patients included in the study were on ventilator and without brain-stem reflexes at the time of death. After documenting electrocerebral silence on EEG, ventilation was stopped and patients were declared [dead]. None of the patient survived. We conclude that a clinically brain dead patient does not need an EEG, and if done and shows electrocerebral silence, patient should be disconnected from the ventilator

Guillain-barre syndrome in children

Case histories of twelve patients with clinical diagnosis of Guillain-Barre syndrome were analyzed retrospectively. Mean age at the time of presentation was 9.5 years. All children presented with inability to walk and progressive weakness. Three children needed ventilatory support. Morbidity was very high and only two patients were able to walk independently after six months of illness. In a third world country like Pakistan, where poliomyelitis has not been eradicated, diagnosing Guillain-Barre syndrome may not be easy especially in young children. Diagnosis in this.study was based on clinical grounds and supported by CSF.findings. Objective of this study is to describe our experience of Guillain-Barre syndrome in Children Hospital and review the current literature