A 2-month-old female infant with progressive macrocephaly and irritability

Michael D. Partington: A 2-month-old female infant was transferred from an outside institution for evaluation and treatment of progressive macrocephal y with irritability. The child was born at full term via cesarean section f or breech position. The pregnancy was otherwise uncomplicated. The child's initial head circumference was 35 cm (50th percentile). The infant did well for 5 weeks, when she began to have feeding intolerance which did not resp ond to changing formulas. The infant also became more irritable and poorly responsive. The mother noted development of distended scalp veins, and the infant was brought in for well child care at 8 weeks of age. At that visit the head circumference was now noted to be 45 cm, with a tense anterior fon tanelle and splitting of the sutures. Imaging studies were obtained at the outside institution, and neurosurgical consultation was also obtained. The child was subsequently transferred to a regional pediatric tertiary care ce nter. There was no family medical history of central nervous system disorde rs. The child's immunizations were current. The child was not on any medica tions. The initial examination revealed an irritable infant who was poorly responsive. The head circumference was 45 cm (above the 95th percentile). T he anterior fontanel was tense, and the sutures were split. Marked meningis m was present. The chest was clear, and the cardiac examination was unremar kable. There was no organomegaly or abdominal masses on examination. The ne urological examination was remarkable for forced downgaze. The pupils were small but reactive. There was papilledema noted on fundoscopy. The infant m oved all extremities with grossly:normal power and normal tone, although th ere was marked head lag. No clonus was present. The outside ultrasound and magnetic resonance imaging (MRI) scans were reviewed. A computed tomography scan was also obtained to search for intracranial calcifications, and none were identified. The infant was transferred to the intensive care unit for placement of a ventricular drain. The airway was well protected, and the c hild was spontaneously hyperventilating. An external ventricular drain was first placed on the right side which resulted in spontaneous drainage of a thick green fluid under pressure which immediately clotted within the colle cting tube. Because of uncertainty about the continued function of this dra in, a second drain was placed on the left side at the same setting. Initial cerebrospinal fluid studies from the right drain revealed 1 white blood ce ll (differential: 20% lymphocytes, 60% monocytes) and 13 red blood cells pe r cubic millimeter, with the left drain producing 120 white cells (74% lymp hocytes, 14% monocytes, 12% macrophages) and 132 red blood cells per cubic millimeter. The glucose values were 33 and 39 mg/dl bilaterally, with a con current serum glucose level of 120 mg/dl. The initial protein measurements were arranged between 2,770 and 3,000 mg/dl, on multiple assessments. Other initial laboratory values included a normal electrolyte panel, the hemoglo bin and hematocrit values were normal, but the platelet count was elevated at 905,000. Coagulation studies were determined to be normal. On the 2nd ho spital day, a single cerebrospinal fluid culture grew out a gram-negative e nteric bacterial strain which was subsequently identified as Citrobacter Th e infant was started on intravenous ceftazidime and gentamicin, Viral and f ungal cultures were initiated. Polymerase-chain reaction for herpes simplex virus was negative. Formal ophthalmologic consultation confirmed the prese nce of papilledema, but no other retinal lesions were identified. Urine sam ples for amino and organic acids were normal. Because of problems with coagulation in the tubing preventing meaningful ou tput, the ventricular drains were removed. The viral and fungal cultures re mained negative. On the 6th hospital day, a diagnostic procedure was perfor med.