Related Experiment Videos
Kidney transplantation after a severe form of pseudotumor cerebri
C C Mourani1, S G Mallat, M Y Moukarzel
1Department of Pediatrics, Hôtel-Dieu de France Hospital, Beirut, Lebanon.
Insights
Pseudotumor cerebri, a condition of high intracranial pressure, can cause vision loss in children with kidney failure. Early diagnosis and treatment are crucial, though outcomes can be severe despite interventions like optic nerve sheath decompression.
Area of Science:
- Pediatric Nephrology
- Neuro-Ophthalmology
- Intracranial Hypertension Syndromes
Background:
- Pseudotumor cerebri (idiopathic intracranial hypertension) is associated with chronic renal failure and transplantation in pediatric cases.
- Early diagnosis and intervention are vital to prevent vision impairment.
Observation:
- A 7-year-old boy with renal hypoplasia on hemodialysis presented with severe headaches and visual impairment.
- Despite medical management (corticosteroids, acetazolamide) and surgical intervention (lumboperitoneal diversion, optic nerve sheath decompression), vision loss progressed rapidly.
Findings:
- The patient experienced complete vision loss within two weeks, necessitating bilateral optic nerve sheath decompression.
- Renal transplantation was successfully performed, leading to normal renal function and academic performance two years post-surgery.
Implications:
- This case highlights the critical need to suspect pseudotumor cerebri in pediatric patients with renal failure presenting with headaches and papilledema.
- Prompt management is essential, as visual loss can be rapid and irreversible, underscoring the importance of timely renal transplantation in managing associated complications.
Abstract:
Pseudotumor cerebri is a syndrome characterized by intracranial hypertension (intracranial pressure >200 mmH2O) and a normal ventricular system. The diagnosis should be made as early as possible to prevent impairment of vision. Several diseases have been reported in association with pseudotumor cerebri in pediatric patients, and have been occasionally also noted with chronic renal failure, heart and renal transplantation. We report a 7-year-old boy who complained of severe headaches and visual impairment 2 years after hemodialysis for renal hypoplasia. Pseudotumor cerebri was suspected and, despite treatment with corticosteroids, acetazolamide, and lumboperitoneal diversion, visual impairment worsened. Bilateral optic nerve sheath decompression (ONSD) was performed without success and the child completely lost his vision within 2 weeks. He was successfully transplanted 2 months later. Two years post transplantation, the blind child has a normal renal function and school performance. Pseudotumor cerebri must be rapidly suspected in a child with renal failure suffering from headaches and papilledema. Visual loss may progress rapidly and ONSD seems to be the best surgical treatment when medical treatment fails. In this patient renal transplantation was well tolerated, with no deterioration in the neurological status over 2 years of follow-up.