Congenital Dermal Sinus: case series and the consequences of late diagnosis and treatment

Introduction:Congenital Dermal Sinuses (CDS) are rare closed dysraphisms that can present throughout the extent of the neuroaxis. They occur due to a failure of the disjunction of the neuroectoderm and cutaneous ectoderm in a focal point during 3-4 week of embryogenic development. The prevalence of...

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Autores principales:	Alick Durão Moreira, Antonio Bellas, Marcelo Sampaio Poousa, Rafaeldos Santos Mitraud Mitraud, Tatiana Protzenko
Formato:	article
Lenguaje:	EN
Publicado:	Brazilian Society for Pediatric Neurosurgery 2020
Materias:	congenital dermal sinus dermal sinus dermal sinus tract occult dysraphism cutaneous stigma Pediatrics RJ1-570 Surgery RD1-811
Acceso en línea:	https://doaj.org/article/afb1d6d2e83f4254865eefbe4f145d22
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spelling	oai:doaj.org-article:afb1d6d2e83f4254865eefbe4f145d222021-11-23T21:46:36ZCongenital Dermal Sinus: case series and the consequences of late diagnosis and treatment2675-362610.46900/apn.v2i3(September-December).62https://doaj.org/article/afb1d6d2e83f4254865eefbe4f145d222020-12-01T00:00:00Zhttps://www.archpedneurosurg.com.br/pkp/index.php/sbnped2019/article/view/62https://doaj.org/toc/2675-3626Introduction:Congenital Dermal Sinuses (CDS) are rare closed dysraphisms that can present throughout the extent of the neuroaxis. They occur due to a failure of the disjunction of the neuroectoderm and cutaneous ectoderm in a focal point during 3-4 week of embryogenic development. The prevalence of CDS of all types has been estimated to be 1 in 2,500 live births, most commonly localized in the lumbar region. More than half of the cases are associated with dermoid or epidermoid tumors. Clinical presentation of CDS usually consists in cutaneous stigmas like dimples, which has the potential to be diagnosed at birth. However, the majority of patients are diagnosed older and after complications such as meningitis, abscess, osteomyelitis, rupture of an associated epi/dermoid cyst. Once suspected the patient should be submitted to an image study with CT scan and/or MRI, and surgical consultation. Complete exeresis is the definitive treatment. Case report: we present 3 cases of CDS, including an extremely rare case of frontonasal location, to illustrate the extent of the disease and the importance of early diagnosis and treatment. All of the 3 cases presented with complications, requiring surgical treatment and long term antibiotic therapy. Conclusion: Although well reported in the literature, CDS are usually diagnosed after complications. The knowledge of clinical presentation, early diagnosis and treatment are essential to prevent its life threatening complications.Alick Durão MoreiraAntonio BellasMarcelo Sampaio PoousaRafaeldos Santos Mitraud MitraudTatiana ProtzenkoBrazilian Society for Pediatric Neurosurgeryarticlecongenital dermal sinusdermal sinusdermal sinus tractoccult dysraphismcutaneous stigmaPediatricsRJ1-570SurgeryRD1-811ENArchives of Pediatric Neurosurgery, Vol 2, Iss 3(September-December), Pp e622020-e622020 (2020)
institution	DOAJ
collection	DOAJ
language	EN
topic	congenital dermal sinus dermal sinus dermal sinus tract occult dysraphism cutaneous stigma Pediatrics RJ1-570 Surgery RD1-811
spellingShingle	congenital dermal sinus dermal sinus dermal sinus tract occult dysraphism cutaneous stigma Pediatrics RJ1-570 Surgery RD1-811 Alick Durão Moreira Antonio Bellas Marcelo Sampaio Poousa Rafaeldos Santos Mitraud Mitraud Tatiana Protzenko Congenital Dermal Sinus: case series and the consequences of late diagnosis and treatment
description	Introduction:Congenital Dermal Sinuses (CDS) are rare closed dysraphisms that can present throughout the extent of the neuroaxis. They occur due to a failure of the disjunction of the neuroectoderm and cutaneous ectoderm in a focal point during 3-4 week of embryogenic development. The prevalence of CDS of all types has been estimated to be 1 in 2,500 live births, most commonly localized in the lumbar region. More than half of the cases are associated with dermoid or epidermoid tumors. Clinical presentation of CDS usually consists in cutaneous stigmas like dimples, which has the potential to be diagnosed at birth. However, the majority of patients are diagnosed older and after complications such as meningitis, abscess, osteomyelitis, rupture of an associated epi/dermoid cyst. Once suspected the patient should be submitted to an image study with CT scan and/or MRI, and surgical consultation. Complete exeresis is the definitive treatment. Case report: we present 3 cases of CDS, including an extremely rare case of frontonasal location, to illustrate the extent of the disease and the importance of early diagnosis and treatment. All of the 3 cases presented with complications, requiring surgical treatment and long term antibiotic therapy. Conclusion: Although well reported in the literature, CDS are usually diagnosed after complications. The knowledge of clinical presentation, early diagnosis and treatment are essential to prevent its life threatening complications.
format	article
author	Alick Durão Moreira Antonio Bellas Marcelo Sampaio Poousa Rafaeldos Santos Mitraud Mitraud Tatiana Protzenko
author_facet	Alick Durão Moreira Antonio Bellas Marcelo Sampaio Poousa Rafaeldos Santos Mitraud Mitraud Tatiana Protzenko
author_sort	Alick Durão Moreira
title	Congenital Dermal Sinus: case series and the consequences of late diagnosis and treatment
title_short	Congenital Dermal Sinus: case series and the consequences of late diagnosis and treatment
title_full	Congenital Dermal Sinus: case series and the consequences of late diagnosis and treatment
title_fullStr	Congenital Dermal Sinus: case series and the consequences of late diagnosis and treatment
title_full_unstemmed	Congenital Dermal Sinus: case series and the consequences of late diagnosis and treatment
title_sort	congenital dermal sinus: case series and the consequences of late diagnosis and treatment
publisher	Brazilian Society for Pediatric Neurosurgery
publishDate	2020
url	https://doaj.org/article/afb1d6d2e83f4254865eefbe4f145d22
work_keys_str_mv	AT alickduraomoreira congenitaldermalsinuscaseseriesandtheconsequencesoflatediagnosisandtreatment AT antoniobellas congenitaldermalsinuscaseseriesandtheconsequencesoflatediagnosisandtreatment AT marcelosampaiopoousa congenitaldermalsinuscaseseriesandtheconsequencesoflatediagnosisandtreatment AT rafaeldossantosmitraudmitraud congenitaldermalsinuscaseseriesandtheconsequencesoflatediagnosisandtreatment AT tatianaprotzenko congenitaldermalsinuscaseseriesandtheconsequencesoflatediagnosisandtreatment
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Congenital Dermal Sinus: case series and the consequences of late diagnosis and treatment

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