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European Surgery
Erschienen in:

01.12.2014 | Case Report

Buried penis accompanied by undescended testicles and megameatus hypospadias as a severe genital manifestation of Robinow Syndrome

verfasst von: Assist. Prof. Burak Ersoy, MD, Tayfun Aköz, MD, Sinan Ekici, MD

Erschienen in: European Surgery | Ausgabe 6/2014

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Excerpt

Robinow Syndrome was described in 1969 as a new variant of dwarfing syndrome with mesomelic limb shortening, hemivertebrae and genital hypoplasia [1]. Both autosomal dominant and recessive inheritance have been reported, of which the former is more common [2]. Clusters of the autosomal recessive form have been particularly frequently observed in Turkey, Oman, and Czech Republic, demonstrating a high degree of consanguineous marriages in these populations [2‐6]. …
Vorheriger Artikel Xanthogranulomatous cholecystitis: an analysis of 55 cases
Literatur
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Robinow M, Silverman FN, Smith HD. A newly recognized dwarfing syndrome. Am J Dis Child. 1969;117:645–51.PubMed
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Van Bokhoven H, Celli, J, Kayserili H, et al. Mutation of the gene encoding the ROR2 tyrosine kinase causes autosomal recessive Robinow syndrome. Nat Genet. 2000;25(4):423–6.PubMedCrossRef
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Maizels M, Zaontz M, Donovan J, Bushnick PN, Firlit CF. Surgical correction of the buried penis: description of a classification system and a technique to correct the disorder. J Urol. 1986;136(1 Pt 2):268–71.PubMed
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Donahoe PK, Keating MA. Preputial unfurling to correct the buried penis. J Pediatr Surg. 1986;21:1055–7.PubMedCrossRef
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Snodgrass W. Tubularized, incised plate urethroplasty for distal hypospadias. J Urol. 1994;151:464–5.PubMed
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Sanal M, Karadag E, Konca Y, Kocabasoglu U. Megameatus and intact prepuce (MIP) associated with meatal web—a case report. Eur Surg. 1999;32(1):35–6.CrossRef
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Bar-Yosef Y, Binyamini J, Mullerad M, Matzkin H, Ben-Chaim J. Megameatus intact prepuce hypospadias variant: application of tubularized incised plate urethroplasty. Urology. 2005;66(4):861–4.PubMedCrossRef
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Docimo SG. Subcutaneous frenulum flap (SCUFF) for iatrogenic or primary megameatus and reoperative hypospadias repair. Urology. 2001;58(2):271–3.PubMedCrossRef
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Hill GA, Wacksman J, Lewis AG, Sheldon CA. The modified pyramid hypospadias procedure: repair of megameatus and deep glanular groove variants. J Urol. 1993;150(4):1208–11.PubMed
Metadaten
Titel
Buried penis accompanied by undescended testicles and megameatus hypospadias as a severe genital manifestation of Robinow Syndrome
verfasst von
Assist. Prof. Burak Ersoy, MD
Tayfun Aköz, MD
Sinan Ekici, MD
Publikationsdatum
01.12.2014
Verlag
Springer Vienna
Erschienen in
European Surgery / Ausgabe 6/2014
Print ISSN: 1682-8631
Elektronische ISSN: 1682-4016
DOI
https://doi.org/10.1007/s10353-014-0282-8

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