Case 4: Hydrocephalus, Macrothrombocytopenia, Inclusion Bodies, and Nephropathy in a 9-year-old Boy

Tarek A. Belal; Nihal Magdi Al Menabawy

doi:10.1542/pir.2016-0235

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Tarek A. Belal, MD*
Nihal Magdi Al Menabawy, MD^†

^*Pediatrics, Saint Peter's University Hospital, New Brunswick, NJ
^†Kasr Alainy Faculty of Medicine–Pediatrics, Cairo University, Cairo, Egypt

AUTHOR DISCLOSURE
Drs Belal and Al Menabawy have disclosed no financial relationships relevant to this article. This commentary does not contain a discussion of an unapproved/investigative use of a commercial product/device.

Presentation

A previously healthy 9-year-old boy presents to the emergency department with acute-onset headache, vomiting, and blurry vision. Physical examination reveals a blood pressure of 160/100 mm Hg, Glasgow Coma Scale score of 10, bilateral papilledema and splinter hemorrhages, and bilateral abducens palsy. Petechial rash is visible on the trunk and extremities. Brain computed tomography scan shows obstructive hydrocephalus (Fig 1). Laboratory tests show a platelet count of 72 × 10³/μL (72 × 10⁹/L) and normal hemoglobin, white blood cell count, international normalized ratio (INR)/partial thromboplastin time (PTT), and fibrinogen. He is given a platelet transfusion and a ventriculoperitoneal shunt is placed. Normal cerebrospinal fluid analysis and culture results rule out an infectious cause.

Figure 1.

Brain computed tomography scan of the patient showing dilation of both lateral ventricles denoting hydrocephalus.

Postoperatively the boy continues to have high blood pressures (despite antihypertensive medications) that are initially attributed to increased intracranial pressure. However, additional evaluation reveals concentric left ventricular hypertrophy, atrophic right kidney with grade II nephropathy, hypertrophic left kidney, normal renal artery Doppler findings bilaterally, and normal blood urea nitrogen/creatinine. Urinalysis shows 1+ albumin, 15 to 20 red blood cells, and ++ granular casts. Clinicians suggest the presence of long-standing hypertension. He experiences postoperative bleeding from incision sites despite …