Pediatrics case: Hydrocephalus

A 12 week old female infant presents to the emergency department with progressive vomiting, lethargy, and difficulty feeding over the past two days. Her mother reports that the infant has been increasingly irritable in the last week, and does not appear to be herself. She has been less interactive, and her cry has become more high-pitched and weak. She has not been breastfeeding well. Additionally, her mother is concerned because she thinks her infant's head has grown, and the "soft spot" on her head appears more tense. She thinks that the infant has felt "warm", but she has not measured the temperature with a thermometer. The infant has had fewer wet diapers and no bowel movements today.

She reports that the infant was born on time and that there were no prenatal or perinatal complications. The infant was released after a 48 hour stay in the regular newborn nursery, and had follow-up initially with her pediatrician about one week after discharge. She has had no further follow-up. From the previous medical records it is confirmed that the infant was born at term. There was poor prenatal care, but the labor and delivery were unremarkable. Mother's prenatal labs were normal. The infant weighed 2900 grams at birth (25th percentile), measured 47.8 cm in length (10th - 25th percentile), and had a head circumference of 34 cm (25th percentile).

Exam: VS: T 36.5 C, P 165, R 45, BP 98/65. Weight 4.20 kg (5th percentile), length 57 cm (10th - 25th percentile), HC 42.6 cm (95th percentile). In general this is a lethargic infant with a weak, high-pitched cry. Her head is oddly shaped and looks like an inverted pear. Her scalp veins are prominent, and the anterior fontanelle is tense and bulging. Eyes show pupils which are equal and round, but are sluggishly reactive to light. Red reflex is present bilaterally. EOMs are clearly dysconjugate. There is mild tachypnea with slight intercostal retractions. Lung fields are clear to auscultation bilaterally. Her heart exam reveals tachycardia with a regular rhythm and a grade 2/6 systolic ejection murmur at the left sternal border. Capillary refill is 2 seconds. Her spine is straight without protrusions or apparent defects. Her upper extremities show good tone and full range of motion with slightly brisk reflexes. Her lower extremities show increased tone with brisk reflexes bilaterally. There is 4+ clonus bilaterally. On neurologic examination, her eyes show rotated downward gaze bilaterally. There is a poor suck. The startle response is minimally present. The grasp and glabellar reflexes are present. No parachute reflex can be elicited. The Moro is present.

Imaging studies demonstrate hydrocephalus and aqueductal stenosis. A ventriculoperitoneal shunt procedure is performed by a neurosurgeon. Following surgery, the patient's anterior fontanelle is concave and the head circumference has decreased.

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