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Case Report
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| An unusual association of von Hippel–Lindau disease and posterior nutcracker syndrome: Management of neurological and urological aspects in a young male | ||||||
| Nadhir Karmani1, Faouzi Mallat2, Wissem Hmida2, Khaled Ben Ahmed2, Oussama Karmani3, Sidiya Oueld Chavey3, Amel Ben Abdallah3, Faouzi Mosbah2, Hedi Krifa1 | ||||||
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1Neurosurgery Department, SahloulHospital, Sousse, Tunisia.
2Urology Department, SahloulHospital, Sousse, Tunisia. 3Radiology Department, SahloulHospital, Sousse, Tunisia. | ||||||
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| Karmani N, Mallat F, Hmida W, Ahmed KB, Karmani O, Chavey SO, Abdallah AB, Mosbah F, Krifa H. An unusual association of von Hippel–Lindau disease and posterior nutcracker syndrome: Management of neurological and urological aspects in a young male. Int J Case Rep Images 2015;6(7):396–402. |
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Abstract
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Introduction:
Von Hippel–Lindau (VHL) disease is a complex and systemic entity that can be discovered by neurological complications. Its association with other serious malformative disease like nutcracker syndrome (NCS) was not described, and its complex presentation must not make some other serious problem easily missed or neglected.
Case Report: A 27-year-old male patient with 3 intramedullary hemangioblastoma of six months of insidious evolution complicated by medullary compression. The lesions were completely removed with excellent neurological postoperatory outcome. Thinking of VHL disease, our investigations were expanded and revealed multiple vermian and cerebellar hemangioblastomas, renal masses, and multiple pancreatic cysts. In addition, an incidentally found of posterior NCS during the abdominal computed tomography (CT) done for VHL disease investigations. Our attitude to both neurosurgical and urological problems (the serious presentation of VHL disease with multiple locations of hemangioblastoma and right renal carcinomas) and for the missed symptomatic posterior nutcracker syndrome were discussed. Conclusion: The complex presentation of the VHL disease must not makesome other serious problems easily missed or neglected like renal carcinoma or nutcracker syndrome as seen in our case. | |
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Keywords:
Hemangioblastoma, Nutcracker syndrome, Renal carcinomas, Total removal, Von Hippel–Lindau (VHL) disease
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Author Contributions
Nadhir Karmani – Substantial contributions to conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published Faouzi Mallat – Analysis and interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published Wissem Hmida – Analysis and interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published Khaled Ben Ahmed – Analysis and interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published Oussama Karmani – Analysis and interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published Sidiya Oueld Chavey – Analysis and interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published Amel Ben Abdallah – Analysis and interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published Faouzi Mosbah – Analysis and interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published Hedi Krifa – Analysis and interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published |
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Guarantor of submission
The corresponding author is the guarantor of submission. |
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Source of support
None |
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Conflict of interest
Authors declare no conflict of interest. |
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Copyright
© 2015 Nadhir Karmani et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information. |
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