Persistent hyperinsulinaemic hypoglycaemia is a rare metabolic disorder of glucose regulation. It is however the most common cause of persistent hypoglycaemia in the neonatal period. Various drugs have been used with generally poor results, but diazoxide and a long-acting somatostatin analogue, octreotide, have been found to be rather successful. When medical therapy fails, early pancreatectomy is recommended to maintain euglycaemia. Since pancreatectomy seems to carry the long-term risk of diabetes mellitus, some authors recommend long-term medical therapy as an alternative to surgery. The outcome of treatment seems to correlate with neurological status prior to surgery. Even in early recognised and treated patients, publications suggest that a subtle neurological deficit may be present despite apparently normal intelligence. In view of the varying recommendations on treatment and the variations in outcome, we reviewed our experience over a period of three years (1992-1995) to determine whether we could formulate a rational approach to the management.
To determine the clinical and radiological features of the patients who were found to have cavum septum pellucidum (CSP) on the cranial computerized tomographic (CT) scans.
A prospective study was carried out to (i) compare head growth patterns of 103 very low birthweight (VLBW, < 1500 g) Malaysian infants and 98 normal birthweight (NBW, 2500- < 4500 g) controls during the 1st year of life; and (ii) examine the relationship between neurodevelopmental outcome at 1 year of age and occipito-frontal head circumferences (OFC) at birth and at 1 year of age in VLBW babies. When compared with those of NBW infants at birth, mid-infancy and 1 year of age, the mean OFC ratios (observed/expected OFC at 50th percentile) of VLBW infants were significantly lower (p < 0.001). Small-for-gestational-age (SGA) VLBW babies had significantly lower mean OFC ratios than their appropriate-for-gestational-age (AGA) VLBW counterparts at birth (p < 0.001), but this difference was no longer seen at mid-infancy or at 1 year of age. Logistic regression analysis showed that abnormal late neonatal cranial ultrasound findings (odds ratio 8.5, 95% confidence interval 4.12-22.07; p < 0.001) and each additional day of oxygen therapy (odds ratio 1.15, 95% confidence interval 1.00-4.45; p = 0.045) were significant risk factors associated with neurodevelopmental disability at 1 year of age, while mean OFC ratios at birth or at 1 year of age were not. Poor postnatal head growth per se did not predict disability, but probably reflected the consequences of "brain injury" as evidenced by abnormal brain scans.