A 16-month-old girl is brought to clinic by her father for behavior concerns. For the last 6 weeks, the patient has been less interactive with family members and increasingly irritable. Her last checkup was at 12 months, when she met all developmental milestones and was growing appropriately. Since then, the patient has not attained new milestones in speech, gross motor, or fine motor development, which her father attributes to recent social stressors because the parents are undergoing a divorce. Temperature is 37.3 C (99.1 F), blood pressure is 140/90 mm Hg, and pulse is 70/min. The patient has poor eye contact. The anterior fontanelle is closed. Cardiopulmonary examination is unremarkable. The skin is pink and well perfused. Growth charts for head circumference, height, and weight are shown in the exhibits.
Which of the following is the most likely cause of this patient's presentation?

Question

A 16-month-old girl is brought to clinic by her father for behavior concerns.  For the last 6 weeks, the patient has been less interactive with family members and increasingly irritable.  Her last checkup was at 12 months, when she met all developmental milestones and was growing appropriately.  Since then, the patient has not attained new milestones in speech, gross motor, or fine motor development, which her father attributes to recent social stressors because the parents are undergoing a divorce.  Temperature is 37.3 C (99.1 F), blood pressure is 140/90 mm Hg, and pulse is 70/min.  The patient has poor eye contact.  The anterior fontanelle is closed.  Cardiopulmonary examination is unremarkable.  The skin is pink and well perfused.  Growth charts for head circumference, height, and weight are shown in the exhibits.   
Which of the following is the most likely cause of this patient's presentation?

Quizplus · Accepted Answer

11ec25c8_d6f0_b9a5_bc81_53aff9204acf_MD0003_00 This child has a rapidly enlarging head circumference (HC) with irritability, developmental delay, and hypertension. These findings are concerning for increased intracranial pressure due to hydrocephalus, or excessive cerebrospinal fluid (CSF). CSF is produced by the choroid plexus, flows through the ventricular system, and is absorbed into the venous circulation. Any disruption in this process results in abnormal CSF accumulation. The most common cause is obstructive (ie, noncommunicating) hydrocephalus, in which is there is anatomic blockage of CSF flow. Etiologies of obstructive hydrocephalus can include congenital causes (eg, aqueductal stenosis), tumor, and infection. The obstruction results in ventricular dilation and increased intracranial pressure, eventually leading to white matter atrophy. CSF The presentation of hydrocephalus in young children typically includes a rapidly enlarging HC, as seen on this child's growth chart, where HC crosses two major percentiles (ie, 25th, 50th) over a few months. Additional symptoms may include behavior changes (eg, irritability) and/or developmental delay. Children with a closed anterior fontanelle (such as this patient) are more likely to have signs of increased intracranial pressure, including headache, vomiting, hypertension, and papilledema. Pressure on the midbrain may also cause impairment of upward gaze. hydrocephalus in young children Initial evaluation is neuroimaging, including ultrasound of the head in an infant with an open fontanelle or MRI of the brain in this patient with a closed fontanelle. (Choice A) Adjustment disorder is characterized by functional impairment or behavioral symptoms due to an identifiable stressor (eg, divorce). It does not explain a rapidly enlarging HC. (Choices B and D) Congenital cytomegalovirus infection is often asymptomatic at birth and may lead to late complications, including developmental delay and hearing loss, which may affect language acquisition. Hypothyroidism often causes delayed linear growth velocity, constipation, and lethargy. Neither condition would lead to a rapidly enlarging HC associated with hypertension. (Choice E) Rett syndrome is a neurodevelopmental disorder that most commonly presents in toddler girls. Symptoms include irritability, stereotypical hand movements (eg, wringing), and developmental regression after a period of normal development. Unlike in hydrocephalus, in Rett syndrome, slowed head growth and microcephaly are typical. Educational objective: Hydrocephalus (excess cerebrospinal fluid) causes ventricular dilation and increased intracranial pressure. Children may have a rapidly enlarging head circumference, behavior changes, and developmental delay. In patients whose anterior fontanelle has closed, headache, vomiting, hypertension, and papilledema may occur. References: Hydrocephalus in children. (http://www.ncbi.nlm.nih.gov/pubmed/26256071)

A 16-Month-Old Girl Is Brought to Clinic by Her Father