Renal Involvement in Linear Nevus Sebaceous Syndrome—An Underrecognized Feature
Linear nevus sebaceous syndrome (LNSS) is a rare neurocutaneous disorder. It is characterized by the presence of nevus sebaceous, ocular anomalies, neurological deficits, and convulsion. Renal involvement was not commonly reported. We report a 10-year-old girl with LNSS who had concomitant cystic ki...
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MDPI AG
2021-05-01
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| Series: | Pediatric Reports |
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| Online Access: | https://www.mdpi.com/2036-7503/13/2/28 |
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| author | Chonin Kuok Kwaiyu Chan |
| author_facet | Chonin Kuok Kwaiyu Chan |
| author_sort | Chonin Kuok |
| collection | DOAJ |
| description | Linear nevus sebaceous syndrome (LNSS) is a rare neurocutaneous disorder. It is characterized by the presence of nevus sebaceous, ocular anomalies, neurological deficits, and convulsion. Renal involvement was not commonly reported. We report a 10-year-old girl with LNSS who had concomitant cystic kidney disease and diffuse aortopathy with bilateral renal artery stenosis, leading to hypertension requiring oral anti-hypertensive medications. The girl presented with chorioretinal coloboma and multiple nevus sebaceous at birth. She had aortic coarctation and received surgical repair at one week of life. She had persistent hypertension during her follow-up. Further investigations were performed to look for causes of hypertension apart from possible re-coarctation. Her magnetic resonance angiogram revealed diffuse aortopathy, which extended from the aortic arch to the abdominal aorta. Branches of the aorta, including the celiac trunk, superior mesenteric arteries, and renal arteries, were also narrowed. Multiple renal cysts were also identified in her right kidney. Interventional angioplasty over the renal arteries was not feasible due to diffuse narrowing of the aorta, especially at the origins of renal arteries. The blood pressure was controlled with oral anti-hypertensive medications. Our case illustrated that pediatricians should be aware of the possible renal involvements in LNSS, which impose a significant impact on the management and long-term prognosis of these patients. |
| first_indexed | 2024-03-10T11:45:33Z |
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| id | doaj.art-b8da9aefe59a4e56b19bcf162058532c |
| institution | Directory Open Access Journal |
| issn | 2036-7503 |
| language | English |
| last_indexed | 2024-03-10T11:45:33Z |
| publishDate | 2021-05-01 |
| publisher | MDPI AG |
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| series | Pediatric Reports |
| spelling | doaj.art-b8da9aefe59a4e56b19bcf162058532c2023-11-21T18:10:21ZengMDPI AGPediatric Reports2036-75032021-05-0113220320910.3390/pediatric13020028Renal Involvement in Linear Nevus Sebaceous Syndrome—An Underrecognized FeatureChonin Kuok0Kwaiyu Chan1Department of Paediatrics, Queen Elizabeth Hospital, Hong Kong, ChinaDepartment of Paediatrics, Queen Elizabeth Hospital, Hong Kong, ChinaLinear nevus sebaceous syndrome (LNSS) is a rare neurocutaneous disorder. It is characterized by the presence of nevus sebaceous, ocular anomalies, neurological deficits, and convulsion. Renal involvement was not commonly reported. We report a 10-year-old girl with LNSS who had concomitant cystic kidney disease and diffuse aortopathy with bilateral renal artery stenosis, leading to hypertension requiring oral anti-hypertensive medications. The girl presented with chorioretinal coloboma and multiple nevus sebaceous at birth. She had aortic coarctation and received surgical repair at one week of life. She had persistent hypertension during her follow-up. Further investigations were performed to look for causes of hypertension apart from possible re-coarctation. Her magnetic resonance angiogram revealed diffuse aortopathy, which extended from the aortic arch to the abdominal aorta. Branches of the aorta, including the celiac trunk, superior mesenteric arteries, and renal arteries, were also narrowed. Multiple renal cysts were also identified in her right kidney. Interventional angioplasty over the renal arteries was not feasible due to diffuse narrowing of the aorta, especially at the origins of renal arteries. The blood pressure was controlled with oral anti-hypertensive medications. Our case illustrated that pediatricians should be aware of the possible renal involvements in LNSS, which impose a significant impact on the management and long-term prognosis of these patients.https://www.mdpi.com/2036-7503/13/2/28linear nevus sebaceous syndromeepidermal nevus syndromecystic kidney diseaserenal artery stenosishypertension |
| spellingShingle | Chonin Kuok Kwaiyu Chan Renal Involvement in Linear Nevus Sebaceous Syndrome—An Underrecognized Feature Pediatric Reports linear nevus sebaceous syndrome epidermal nevus syndrome cystic kidney disease renal artery stenosis hypertension |
| title | Renal Involvement in Linear Nevus Sebaceous Syndrome—An Underrecognized Feature |
| title_full | Renal Involvement in Linear Nevus Sebaceous Syndrome—An Underrecognized Feature |
| title_fullStr | Renal Involvement in Linear Nevus Sebaceous Syndrome—An Underrecognized Feature |
| title_full_unstemmed | Renal Involvement in Linear Nevus Sebaceous Syndrome—An Underrecognized Feature |
| title_short | Renal Involvement in Linear Nevus Sebaceous Syndrome—An Underrecognized Feature |
| title_sort | renal involvement in linear nevus sebaceous syndrome an underrecognized feature |
| topic | linear nevus sebaceous syndrome epidermal nevus syndrome cystic kidney disease renal artery stenosis hypertension |
| url | https://www.mdpi.com/2036-7503/13/2/28 |
| work_keys_str_mv | AT choninkuok renalinvolvementinlinearnevussebaceoussyndromeanunderrecognizedfeature AT kwaiyuchan renalinvolvementinlinearnevussebaceoussyndromeanunderrecognizedfeature |