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An Interdisciplinary Approach to Müllerian Outflow Tract Obstruction Associated with Cloacal Malformation and Cloacal Exstrophy

People with cloacal malformation and 46,XX cloacal exstrophy are at risk of developing Müllerian outflow tract obstruction (OTO). Management of OTO requires expertise of many medical and surgical specialties. The primary presenting symptom associated with OTO is cyclical and later continuous pain an...

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Autores principales: Sack, Bryan S., Speck, K. Elizabeth, Hryhorczuk, Anastasia L., Sandberg, David E., Kraft, Kate H., Ralls, Matthew W., Keegan, Catherine E., Quint, Elisabeth H., Dendrinos, Melina L.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9369038/
https://www.ncbi.nlm.nih.gov/pubmed/35956025
http://dx.doi.org/10.3390/jcm11154408
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author Sack, Bryan S.
Speck, K. Elizabeth
Hryhorczuk, Anastasia L.
Sandberg, David E.
Kraft, Kate H.
Ralls, Matthew W.
Keegan, Catherine E.
Quint, Elisabeth H.
Dendrinos, Melina L.
author_facet Sack, Bryan S.
Speck, K. Elizabeth
Hryhorczuk, Anastasia L.
Sandberg, David E.
Kraft, Kate H.
Ralls, Matthew W.
Keegan, Catherine E.
Quint, Elisabeth H.
Dendrinos, Melina L.
author_sort Sack, Bryan S.
collection PubMed
description People with cloacal malformation and 46,XX cloacal exstrophy are at risk of developing Müllerian outflow tract obstruction (OTO). Management of OTO requires expertise of many medical and surgical specialties. The primary presenting symptom associated with OTO is cyclical and later continuous pain and can be initially quelled with hormonal suppression as a temporizing measure to allow for patient maturation. The decision for timing and method of definitive treatment to establish a patent outflow tract that can also be used for penetrative sexual activity and potential fertility is a complicated one and incredibly variable based on patient age alone. To understand the management approach to OTO, we put forth five phases with associated recommendations: (1) caregiver and patient education and evaluation before obstruction; (2) presentation, diagnosis, and symptom temporization; (3) readiness assessment; (4) peri-procedural management; (5) long-term surveillance. This review will emphasize the importance of interdisciplinary team management of the complex shared medical, surgical, and psychological decision making required to successfully guide developing patients with outflow obstruction secondary to cloacal malformations and cloacal exstrophy through adolescence.
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spelling pubmed-93690382022-08-12 An Interdisciplinary Approach to Müllerian Outflow Tract Obstruction Associated with Cloacal Malformation and Cloacal Exstrophy Sack, Bryan S. Speck, K. Elizabeth Hryhorczuk, Anastasia L. Sandberg, David E. Kraft, Kate H. Ralls, Matthew W. Keegan, Catherine E. Quint, Elisabeth H. Dendrinos, Melina L. J Clin Med Review People with cloacal malformation and 46,XX cloacal exstrophy are at risk of developing Müllerian outflow tract obstruction (OTO). Management of OTO requires expertise of many medical and surgical specialties. The primary presenting symptom associated with OTO is cyclical and later continuous pain and can be initially quelled with hormonal suppression as a temporizing measure to allow for patient maturation. The decision for timing and method of definitive treatment to establish a patent outflow tract that can also be used for penetrative sexual activity and potential fertility is a complicated one and incredibly variable based on patient age alone. To understand the management approach to OTO, we put forth five phases with associated recommendations: (1) caregiver and patient education and evaluation before obstruction; (2) presentation, diagnosis, and symptom temporization; (3) readiness assessment; (4) peri-procedural management; (5) long-term surveillance. This review will emphasize the importance of interdisciplinary team management of the complex shared medical, surgical, and psychological decision making required to successfully guide developing patients with outflow obstruction secondary to cloacal malformations and cloacal exstrophy through adolescence. MDPI 2022-07-28 /pmc/articles/PMC9369038/ /pubmed/35956025 http://dx.doi.org/10.3390/jcm11154408 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Sack, Bryan S.
Speck, K. Elizabeth
Hryhorczuk, Anastasia L.
Sandberg, David E.
Kraft, Kate H.
Ralls, Matthew W.
Keegan, Catherine E.
Quint, Elisabeth H.
Dendrinos, Melina L.
An Interdisciplinary Approach to Müllerian Outflow Tract Obstruction Associated with Cloacal Malformation and Cloacal Exstrophy
title An Interdisciplinary Approach to Müllerian Outflow Tract Obstruction Associated with Cloacal Malformation and Cloacal Exstrophy
title_full An Interdisciplinary Approach to Müllerian Outflow Tract Obstruction Associated with Cloacal Malformation and Cloacal Exstrophy
title_fullStr An Interdisciplinary Approach to Müllerian Outflow Tract Obstruction Associated with Cloacal Malformation and Cloacal Exstrophy
title_full_unstemmed An Interdisciplinary Approach to Müllerian Outflow Tract Obstruction Associated with Cloacal Malformation and Cloacal Exstrophy
title_short An Interdisciplinary Approach to Müllerian Outflow Tract Obstruction Associated with Cloacal Malformation and Cloacal Exstrophy
title_sort interdisciplinary approach to müllerian outflow tract obstruction associated with cloacal malformation and cloacal exstrophy
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9369038/
https://www.ncbi.nlm.nih.gov/pubmed/35956025
http://dx.doi.org/10.3390/jcm11154408
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