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Renal Involvement in Linear Nevus Sebaceous Syndrome-An Underrecognized Feature
1Department of Paediatrics, Queen Elizabeth Hospital, Hong Kong, China.
Pediatric Reports
|June 2, 2021
Summary
Linear nevus sebaceous syndrome (LNSS) can involve kidney complications, including cystic kidney disease and renal artery stenosis. Early recognition of these renal manifestations is crucial for managing hypertension and improving patient outcomes.
Area of Science:
- Medical Genetics
- Pediatric Nephrology
- Cardiovascular Medicine
Background:
- Linear nevus sebaceous syndrome (LNSS) is a rare congenital neurocutaneous disorder.
- Clinical manifestations typically include nevus sebaceous, ocular anomalies, neurological deficits, and seizures.
- Renal involvement is infrequently reported in LNSS patients.
Observation:
- A 10-year-old girl with LNSS presented with chorioretinal coloboma and nevus sebaceous.
- She had a history of aortic coarctation repaired neonatally and persistent hypertension.
- Magnetic resonance angiography revealed diffuse aortopathy and bilateral renal artery stenosis.
Findings:
- The patient exhibited cystic kidney disease and diffuse narrowing of the aorta and its branches, including the renal arteries.
- Hypertension was managed with oral anti-hypertensive medications due to the inaccessibility of interventional angioplasty.
- This case highlights significant renal and cardiovascular complications in LNSS.
Implications:
- Pediatricians must consider potential renal complications in patients diagnosed with LNSS.
- Awareness of renal involvement is vital for comprehensive management and long-term prognosis.
- This case underscores the importance of thorough cardiovascular and renal evaluations in LNSS.
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