A rare case of Watson syndrome

Watson for the first time reported a case series of children in a family that presented with pulmonary valve stenosis, mental retardation, short stature, and small brown color skin lesions that are known as cafe-au-lait spots. We present a rare new variant of the syndrome in an adult patient with se...

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Main Authors: Feridoun Sabzi, Aghigh Heydari, Reza Heidari Moghaddam, Mohammad Rouzbahani, Atefeh Asadmobini
Format: Article
Language:English
Published: Pensoft Publishers 2022-08-01
Series:Folia Medica
Subjects:
Online Access:https://foliamedica.bg/article/63559/download/pdf/
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author Feridoun Sabzi
Aghigh Heydari
Reza Heidari Moghaddam
Mohammad Rouzbahani
Atefeh Asadmobini
author_facet Feridoun Sabzi
Aghigh Heydari
Reza Heidari Moghaddam
Mohammad Rouzbahani
Atefeh Asadmobini
author_sort Feridoun Sabzi
collection DOAJ
description Watson for the first time reported a case series of children in a family that presented with pulmonary valve stenosis, mental retardation, short stature, and small brown color skin lesions that are known as cafe-au-lait spots. We present a rare new variant of the syndrome in an adult patient with severe pulmonary valve stenosis, main, left, and right pulmonary artery aneurysm, short stature, mental retardation, coronary artery disease, and skin lesions. The patient underwent open cardiac surgery with pulmonary valvotomy and aneurysmorrhaphy of the main pulmonary artery and its right and left branches. The postoperative course was uneventful and the six-month follow-up with transthoracic echocardiography revealed no recurrence of aneurysm of repairing pulmonary arteries and good clinical outcome of the patient. Our patient had a unique characteristic of aneurysm of the main pulmonary artery and its both branches that has rarely been reported previously in the medical literature.
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spelling doaj.art-0f5e5497e64a4e34aa2f0edabe1f79fe2022-12-22T03:15:31ZengPensoft PublishersFolia Medica1314-21432022-08-0164467267510.3897/folmed.64.e6355963559A rare case of Watson syndromeFeridoun Sabzi0Aghigh Heydari1Reza Heidari Moghaddam2Mohammad Rouzbahani3Atefeh Asadmobini4Kermanshah University of Medical SciencesKermanshah University of Medical SciencesKermanshah University of Medical SciencesKermanshah University of Medical SciencesKermanshah University of Medical SciencesWatson for the first time reported a case series of children in a family that presented with pulmonary valve stenosis, mental retardation, short stature, and small brown color skin lesions that are known as cafe-au-lait spots. We present a rare new variant of the syndrome in an adult patient with severe pulmonary valve stenosis, main, left, and right pulmonary artery aneurysm, short stature, mental retardation, coronary artery disease, and skin lesions. The patient underwent open cardiac surgery with pulmonary valvotomy and aneurysmorrhaphy of the main pulmonary artery and its right and left branches. The postoperative course was uneventful and the six-month follow-up with transthoracic echocardiography revealed no recurrence of aneurysm of repairing pulmonary arteries and good clinical outcome of the patient. Our patient had a unique characteristic of aneurysm of the main pulmonary artery and its both branches that has rarely been reported previously in the medical literature.https://foliamedica.bg/article/63559/download/pdf/adultcongenital heart diseaseneurofibromatosis
spellingShingle Feridoun Sabzi
Aghigh Heydari
Reza Heidari Moghaddam
Mohammad Rouzbahani
Atefeh Asadmobini
A rare case of Watson syndrome
Folia Medica
adult
congenital heart disease
neurofibromatosis
title A rare case of Watson syndrome
title_full A rare case of Watson syndrome
title_fullStr A rare case of Watson syndrome
title_full_unstemmed A rare case of Watson syndrome
title_short A rare case of Watson syndrome
title_sort rare case of watson syndrome
topic adult
congenital heart disease
neurofibromatosis
url https://foliamedica.bg/article/63559/download/pdf/
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