History of present illness
A 19-year-old male patient woke up with bilateral blurred vision. Past medical history was positive for ventriculoperitoneal (VP) shunt inserted 11 years earlier due to secondary hydrocephalus caused by traumatic head injury.
Ocular examination findings
On examination, visual acuity was 20/150 in the right eye (OD) and 20/60 in the left eye (OS). Extraocular muscle examination revealed a bilateral abduction deficit. Intraocular pressures and anterior segment examination were unremarkable. Dilated fundus examination demonstrated retinal venous engorgement and tortuosity associated with multilayered retinal hemorrhages, retinal exudates, and optic disc edema in each eye ( Fig. 41.1 ).
Imaging
Spectral-domain optical coherence tomography (OCT) of the nerve demonstrated bilateral optic disc elevation ( Fig. 41.2 A, B). OCT B scan through the macula showed multiple retinal hemorrhages OU, including subretinal, intraretinal, and sub–internal limiting membrane (ILM) hemorrhages ( Fig. 41.2 C, D).
Questions to ask
- ■
Did the patient report any neurological signs (e.g., headache, nausea, weakness)?
- ■
Yes. The patient reported occasional headaches, vomiting with abdominal distention, and lower extremity weakness. Elevated intracranial pressure (ICP) can be an important cause of bilateral optic disc edema and bilateral sixth nerve palsy. , Retinal hemorrhages can be noted adjacent to the nerve in cases of elevated ICP.
- ■
- ■
Did the patient report any history of recent head trauma? Head trauma is one of the most common etiologies of bilateral sixth nerve palsy. ,
- ■
The patient denied any history of recent head trauma.
- ■
- ■
Did the patient endorse any history of high blood pressure? Severe (grade 4) malignant hypertension can cause optic nerve edema and multilayered retinal hemorrhages.
- ■
The patient’s blood pressure was normal by history and examination.
- ■
- ■
Did the patient have any history of blood clotting or hematological disorders? Central retinal vein occlusion (CRVO) can cause venous engorgement and tortuosity with retinal hemorrhages. Simultaneous bilateral CRVO in a young adult should raise the possibility of systemic conditions leading to a hyperviscosity syndrome such as immunogammopathy or leukemia.
- ■
No. The patient denied any personal or familial history of hypercoagulation disorders or hematological diseases.
- ■
Assessment
This is a case of a 19-year-old male patient with history of VP shunt who presented with bilateral vision loss, vomiting, abdominal distention, headaches, and leg weakness. Examination revealed bilateral sixth nerve palsy, optic disc edema, and engorged retinal veins with retinal exudation and multilayered retinal hemorrhages.
Differential diagnosis
- ■
Elevated ICP (possible causes in this case include VP shunt obstruction or infection)
- ■
Hypertensive retinopathy
- ■
Diabetic retinopathy
- ■
Retinitis (infectious, infiltrative)
- ■
Optic neuritis (infectious, infiltrative)
- ■
CRVO
- ■
Hyperviscosity syndrome
Working diagnosis
Hyperviscosity syndrome OU
Systemic investigation
- ■
VP shunt imaging (with computed tomography [CT]/magnetic resonance imaging)
- ■
Imaging was unremarkable and there were no signs of VP shunt catheter malfunction or obstruction
- ■
- ■
Basic laboratory tests
- ■
White blood count (WBC): 697,000/μL
- ■
Hemoglobin: 8.4 g/L
- ■
Platelets: 387,000/μL
- ■
Up to half of the patients with leukemia may demonstrate ocular involvement. CRVO is reported in conjunction with an extremely high WBC blood count causing a hyperviscosity syndrome.
- ■
- ■
- ■
Abdomen CT
- ■
CT showed an enlarged spleen. Leukemic cells can accumulate in the spleen leading to splenic enlargement and abdominal distention.
- ■
New working diagnosis
After ruling out VP shunt obstruction and given the abnormal blood count, a diagnosis of hyperviscosity syndrome secondary to blood dyscrasia was rendered.
Multimodal retinal imaging
