Acyclovir use in sick infants.

BACKGROUND: Infantile herpes simplex virus encephalitis (HSVE) infection remains a significant cause of morbidity and mortality. Diagnosis is often difficult in this population, where a specific pattern of clinical and laboratory signs are lacking. This often results in unnecessary treatment of infants with empiric acyclovir. This study evaluates the use of empiric acyclovir at the Kentucky Children's Hospital and attempts to correlate any laboratory or clinical findings that may be highly suggestive of HSVE. METHODS: Medical records of infants younger than 1 year admitted and treated with acyclovir were evaluated for any consistent pattern of clinical findings suggestive of HSVE. Specifically, serum and cerebrospinal fluid (CSF) white blood cell counts, red blood cell counts, cerebrospinal glucose and protein, and clinical neurological findings upon admission were evaluated. RESULTS: Two hundred eighteen infants were identified and included in the study. Three infants were identified with polymerase chain reaction-positive HSVE. Only CSF leukocytosis was consistent among HSVE-positive infants. All infants with HSVE exhibited generalized neurological findings. Neither hemorrhagic CSF nor focal neurological findings were indicative of HSVE infection. DISCUSSION: Herpes simplex virus encephalitis has a very low prevalence within this population. Clinically significant neurological findings as well as specific risk factors must be present to consider treatment with empiric acyclovir. Apnea and focal seizures are not specific risk factors for herpetic meningitis in infants. Lack of a CSF leukocytosis is a strong negative predictor for HSVE, and hemorrhagic fluid is not specific for HSVE.

Rachischisis totalis without acrania in a newborn male.

BACKGROUND: Rachischisis totalis is a rare open neural tube defect often associated with lethal acrania. When acrania is not present, mortality remains high and morbidity for survivors is extreme. PATIENT: We describe is a term newborn with in-utero diagnosed rachischisis totalis without acrania and Chiari II malformation with subsequent hydrocephalus. RESULTS: At birth, clinical examination revealed rachischisis totalis without acrania. The spinal defect extended from the mid-cervical to the lower sacral vertebrae. Serial cranial ultrasonography and brain MRI revealed Chiari II malformation with severe hydrocephalus. The spinal defect was closed and a ventriculoperitoneal shunt placed to relieve the hydrocephalus. The clinical course was complicated by significant central apnea, often necessitating aggressive resuscitation and chest compressions. The infant has survived with expected severe developmental delay but has graduated from hospice care. CONCLUSION: This report demonstrates that survival with variable function and quality of life is possible with severe open neural tube defects. A multidisciplinary team approach including family-centered care must be used early to overcome the many ethical challenges that such patients present.