Pai Syndrome: An Adult Patient with Bifid Nose and Frontal Hairline Marker

Author:

Coban Y. Kenan1,Boran Cetin2,Omeroglu S. Alp3,Okur Erdogan4

Affiliation:

1. Department of Plastic and Reconstructive Surgery, School of Medicine, Kahramanmaras Sutcu Imam University, Kahramanmaras, Turkey

2. Department of Pathology, School of Medicine, Kahramanmaras Sutcu Imam University, Kahramanmaras, Turkey

3. Department of Radiology, School of Medicine, Kahramanmaras Sutcu Imam University, Kahramanmaras, Turkey

4. Department of Otorhinolaryngology, School of Medicine, Kahramanmaras Sutcu Imam University, Kahramanmaras, Turkey

Abstract

Objective A 17-year-old previously unreported patient with Pai syndrome is described. The boy had median cleft of upper lip, a polypoid skin mass over the columella, a minimal cleft of the upper central incisors, frontal alopecia of the anterior hairline, and bifid nose. Magnetic resonance imaging showed pericallosal lipoma. No mental retardation was present, and a chromosomal study showed normal male 46, XY karyotype.

Publisher

SAGE Publications

Subject

Otorhinolaryngology,Oral Surgery

Cited by 11 articles. 订阅此论文施引文献 订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献

1. Cleft 0/14;Facial Reconstruction of Unusual Facial Clefts;2023

2. Accessory columellas: A case series on surgical method and short-term postoperative course;Journal of Plastic, Reconstructive & Aesthetic Surgery;2022-12

3. Toward clinical and molecular dissection of frontonasal dysplasia with facial skin polyps: From Pai syndrome to differential diagnosis through a series of 27 patients;American Journal of Medical Genetics Part A;2022-04-21

4. Pai syndrome: a review;Child's Nervous System;2020-07-10

5. First Report of Pai Syndrome With Upper Alveolar Cavernous Hemangioma: Diagnosis and Surgical Treatment;Journal of Craniofacial Surgery;2020-05-10

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