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

Idiopathic infantile hyperinsulinism: a case report and review of literature

We are reporting our first experience with idiopathic infantile hyperinsulinism which has also been termed nesedioblastosis. There are several causes of hyperinsulinism and hypoglycemia in neonates. It is of prime importance to diagnose hypoglycemia in newborn early to prevent hypoglycemia related brain damage. In contrast to many hyperglycemic hormones, insulin is the only major hypoglycemic hormone. Normally there is a fine control of blood glucose level which if falls below normal levels insulin secretions stops and other hormones like epinephrine, norepinephrine, glucagon etc. are at once poured into the blood to combat hypoglycemia and vice versa. This fine control is lost in idiopathic hyperinsulinemic hypoglycemia of new born in which there is persistently high levels of blood insulin levels despite very low and life threatening levels of blood glucose. There are many theories of this idiopathic increase in insulin levels and so are the various models of treatment. Here we discuss our own experience with such a case