For Publishers
DiscoveryMetadataPeer reviewHostingPublishing
For Researchers
JoinPublishReviewCollect
Register
Blog
About
Search
Advanced search
My ScienceOpen
Search
For Publishers
For Researchers
Blog
About
33
views
13
references
 Top references
cited by
4
    
0 reviews
 Review
0
comments
 Comment
0
recommends
+1 Recommend
0 collections
    8
    shares
      189
      similar
       All similar
      • Record: found
      • Abstract: found
      • Article: not found

      Bilateral renal artery stenosis and epidermal nevus syndrome in a child

      case-report

      Read this article at

      ScienceOpenPublisherPMC
      Bookmark
          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          Abstract

          Epidermal nevus syndrome is a rare congenital sporadic neuro-ectodermic disorder, characterized by the presence of epidermal nevi in association with various developmental abnormalities of the skin, eyes, nervous, skeletal, cardiovascular and urogenital systems. We describe a 5-year-old boy with conjunctival lipodermoid, cervical and facial sebaceous nevi who presented at 3 years of age with hypertension due to bilateral renal artery stenosis together with multiple vascular anomalies (aorta, celiac trunk, superior mesenteric artery) as shown by magnetic resonance angiography. Systemic arterial hypertension was difficult to control despite combined anti-hypertensive drugs and the surgical repair of the aortic coarctation.

          Related collections

          Most cited references13

          • Record: found
          • Abstract: found
          • Article: not found

          Middle aortic syndrome: from presentation to contemporary open surgical and endovascular treatment.

          K Delis, Peter Gloviczki (2005)
          Middle aortic syndrome (MAS) is a clinical condition generated by segmental narrowing of the abdominal or distal descending thoracic aorta. MAS may be acquired, caused by Takayasu's or temporal arteritis (giant cell arteritides), neurofibromatosis, fibromuscular dysplasia, retroperitoneal fibrosis, mucopolysaccharidosis, and the Williams syndrome, or congenital, ascribed to a developmental anomaly in the fusion and maturation of the paired embryonic dorsal aortas. Segmental aortic stenosis may be located at the suprarenal, inter-renal or infrarenal aorta, with a high propensity for concomitant stenoses in both the renal (63%) and visceral (33%) arteries. Hypertension proximal to the aortic stenosis, and relative hypotension distal to it, are characteristic findings in MAS. Typical manifestations include headache, early fatigue on exertion, and bilateral lower-limb claudication. The severity of hypertension is the primary indication for intervention and the factor determining procedural timing. As a great proportion of patients with MAS are children or teenagers, the clinical benefits of early surgical intervention to reverse refractory hypertension have to be weighed against the repercussions pertaining to the insult of surgery on the developing aorta. Open surgery is the primary treatment of tubular aortic narrowing (MAS) associated with renovascular hypertension and visceral artery stenosis. This entails aortoaortic bypass of the diseased segment or, less often, patch aortoplasty and usually bypass grafting of the stenosed renal and visceral arteries performed with autologous conduits, particularly in the youngest of patients. Endovascular therapy may provide a sound minimally invasive treatment in MAS caused by discrete aortic stenoses that do not encompass the mesenteric and renal arteries. Hypertension is thus improved or cured in more than 70% of patients. Prognosis after uncompromised surgical reconstruction is rewarding in the mid and long term in patients with congenital aortic coarctation but deteriorates in patients with aortoarteritis and recurrent inflammatory activity.
          Article 0 comments Cited 37 times – based on 0 reviews     Review now
            Bookmark
            • Record: found
            • Abstract: not found
            • Article: not found

            Clinical practice. Renal-artery stenosis.

            Lance Dworkin, Christopher Cooper (2009)
            Article 0 comments Cited 24 times – based on 0 reviews     Review now
              Bookmark
              • Record: found
              • Abstract: found
              • Article: not found

              Epidermal nevi and the epidermal nevus syndrome. A review of 131 cases.

              C Commens, I McCrossin, Ian Rogers (1989)
              One hundred thirty-one patients with epidermal nevi are reviewed. The commonest site of involvement was the head and neck, and 13% of patients had widespread lesions. Ages of onset ranged from birth to 14 years. A spread beyond the original distribution was noted in 37% of patients. Many clinical patterns occurred, including eight cases of inflammatory linear verrucous epidermal nevus. A variety of other cutaneous abnormalities were found. Biopsies were performed in 41 cases. Three patients developed secondary tumors. One hundred nineteen of the patients were assessed for the presence of abnormalities in other organ systems. One or more abnormalities were demonstrated in 33% of patients, and in 5%, five or more abnormalities were detected. This study indicates that patients with epidermal nevi are at significant risk of having other abnormalities and warrant detailed initial assessment and close follow-up.
              Article 0 comments Cited 21 times – based on 0 reviews     Review now
                Bookmark
                All references

                Author and article information

                Contributors
                : alsohim-md@hotmail.com
                Journal
                Journal ID (nlm-ta): Pediatr Nephrol
                Title: Pediatric Nephrology (Berlin, Germany)
                Publisher: Springer-Verlag (Berlin/Heidelberg )
                ISSN (Print): 0931-041X
                ISSN (Electronic): 1432-198X
                Publication date (Electronic): 1 July 2011
                Publication date PMC-release: 1 July 2011
                Publication date (Print): November 2011
                Volume: 26
                Issue: 11
                Pages: 2081-2084
                Affiliations
                [1 ]Service de néphrologie, rhumatologie et dermatologie pédiatriques, Hôpital Femme Mère Enfant, centre de référence des maladies rénales rares, Hospices Civils de Lyon, 59 boulevard Pinel, 69500 Bron, France
                [2 ]Service de chirurgie cardio-vasculaire, Hôpital Louis Pradel, groupement hospitalier Est, Bron, France
                [3 ]Service de radiologie, Hôpital Femme Mère Enfant, Bron, France
                [4 ]Université de Lyon, Lyon, France
                [5 ]King Saud university, Riyadh, Kingdom of Saudi Arabia
                Article
                Publisher ID: 1949
                DOI: 10.1007/s00467-011-1949-z
                PMC ID: 3178020
                PubMed ID: 21720804
                SO-VID: 67f79ca0-78f8-4201-a5cc-698c262a0ecc
                Copyright © © The Author(s) 2011
                History
                Date received : 14 December 2010
                Date revision received : 25 May 2011
                Date accepted : 31 May 2011
                Categories
                Subject: Brief Report
                Custom metadata
                issue-copyright-statement © IPNA 2011

                ScienceOpen disciplines: Nephrology
                Keywords: hypertension,renal artery stenosis,epidermal nevus syndrome,aortic stenosis
                Data availability:
                ScienceOpen disciplines: Nephrology
                Keywords: hypertension, renal artery stenosis, epidermal nevus syndrome, aortic stenosis

                Comments

                Comment on this article

                scite_

                Similar content189

                See all similar

                Cited by4

                See all cited by

                Most referenced authors79

                See all reference authors