The Neurological Outcome of Isolated PVL and Severe IVH in Preterm Infants: Is It Fair to Compare?

OBJECTIVE: We compared the neurological outcome of isolated periventricular leukomalacia and severe intraventricular hemorrhage in a cohort of very low birth weight infants born and managed at single tertiary-care center in Saudi Arabia. METHODS: We undertook a descriptive retrospective chart review of the neurological status of very low birth weight infants who were born and managed over a 5-year period at King Abdulaziz Medical City, Riyadh. The neurological outcome of neonates with isolated periventricular leukomalacia and severe intraventricular hemorrhage (grades III and IV) was studied and compared in relation to developmental delay and cerebral palsy. RESULTS: A total of 20 patients with isolated periventricular leukomalacia and 26 with severe intraventricular hemorrhage (grades III and IV) were identified for this study. Of 20 patients with isolated periventricular leukomalacia, 9 (45%) had good developmental outcome and 11 (55%) had bad developmental outcome. Of 26 patients of severe intraventricular hemorrhage, 14 (54%) had good developmental outcome and 12 (46%) had bad developmental outcome (P = 0.55). Significant motor neurological deficit affecting function is distributed as follows: 11/20 (55%) in the isolated periventricular leukomalacia group and 7/26 (27%) in the severe intraventricular hemorrhage group (P = 0.05). Cerebral palsy was diplegic in 7/11 (64%) and quadriplegic in 4/11 (36%) in the isolated periventricular leukomalacia group, and hemiplegic 3/7 (43%), diplegic in 1/7 (14%), and quadriplegic in 3/7 (43%) in the severe intraventricular hemorrhage group (P = 0.03). Distribution of the neurological outcome according to periventricular leukomalacia grade was as follows: for periventricular leukomalacia grade I (n = 8), 6/8 (75%) had good neurological outcome and 2/8 (25%) had bad neurological outcome. In periventricular leukomalacia grade II (n = 4), good neurological outcome was seen in three patients (75%) and bad neurological outcome was seen in one patient (25%). All patients (n = 8) with periventricular leukomalacia grade III had bad outcome (P < 0.01). CONCLUSION: About half of patients with isolated periventricular leukomalacia and severe intraventricular hemorrhage had a poor developmental outcome. However, the severity of cerebral palsy was greater in the isolated periventricular leukomalacia patients and correlates highly with periventricular leukomalacia grade. Symmetrical diplegic cerebral palsy is the most common motor deficit associated with isolated periventricular leukomalacia, whereas asymmetrical hemiplegic cerebral palsy is seen exclusively with severe intraventricular hemorrhage.

Risk factors for isolated periventricular leukomalacia.

Periventricular leukomalacia, a major cause of neurologic disabilities in preterm infants, can be isolated or associated with intraventricular and periventricular hemorrhage. To determine the risk factors for isolated periventricular leukomalacia, we retrospectively studied the characteristics of all very low birth weight infants affected by isolated periventricular leukomalacia who were delivered over a 5-year period and compared them with a control group of very low birth weight infants, matched within 2 weeks for gestational age, with no central nervous system pathology, and born during the same period. In total, 20 affected infants were compared with 98 control infants. Neonatal sepsis caused by coagulase-negative Staphylococcus (P = 0.014) and neonatal seizure (P = 0.026) were associated with isolated periventricular leukomalacia only on univariate analysis. Three variables demonstrated statistically significant associations with isolated periventricular leukomalacia on both univariate and multivariate logistic regression analysis as independent risk factors: birth weight (odds ratio, 4.31; 95% confidence interval, 1.54-12.06; P = 0.005), early neonatal hypotension requiring combined inotropic therapy (odds ratio, 4.90; 95% confidence interval; 1.22-19.68, P = 0.025), and delayed surgical closure of hemodynamically significant patent ductus arteriosus beyond age 7 days (odds ratio, 1.20; 95% confidence interval, 1.06-1.35; P = 0.003).

An inborn error of metabolism presenting as hypoxic-ischemic insult.

This case report profiles two children whose sole presentation is intractable seizures. The index case is a 1-year-old female. She presented to the emergency department with intractable seizures. Her initial metabolic evaluation was nonconclusive. Electroencephalogram was abnormal. Brain magnetic resonance imaging yielded a picture consistent with profound ischemic hypoxic injury. The second case was the 8-year-old brother of the index case. He suffered from intractable seizures since birth. On examination he was microcephalic with spastic quadriparesis and bilateral dislocation of lenses. Computed tomography of the brain revealed a low-density area in the white and cortical matter consistent with hypoxic-ischemic injury. His urinalysis for sulfocysteine produced findings consistent with isolated sulfite oxidase deficiency.