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Orthognathic surgery in Melnick-Needles syndrome: a review of the literature and report of two siblings.

International journal of oral and maxillofacial surgery 2018 Vol.47(6) p. 738-742

O'Connell JE, Bourke B, Kearns GJ

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Abstract

Melnick-Needles syndrome (MNS) is a rare congenital X-linked dominant skeletal dysplasia, characterized by exophthalmos, a prominent forehead, and mandibular hypoplasia and retrognathism. Dental features may include anodontia, hypodontia, or oligodontia. Increased collagen content, unpredictable collagen synthesis, and abnormal bony architecture have raised concerns regarding bone splitting intraoperatively and bone healing postoperatively. This report describes the cases of two sisters with MNS, who successfully underwent orthognathic surgery consisting of bilateral mandibular ramus osteotomies combined with advancement genioplasty and iliac crest bone grafting, to correct the classical MNS facial deformity of mandibular retrognathia.

추출된 의학 개체 (NER)

유형영어 표현한국어 / 풀이UMLS CUI출처등장
시술 orthognathic surgery 안면윤곽술 dict 2
시술 genioplasty 턱끝성형술 dict 1
해부 mandibular scispacy 1
해부 retrognathism scispacy 1
해부 bone scispacy 1
해부 iliac crest bone scispacy 1
합병증 skeletal dysplasia scispacy 1
합병증 forehead scispacy 1
합병증 MNS facial scispacy 1
합병증 mandibular retrognathia scispacy 1
질환 Melnick-Needles syndrome C0025237
Melnick-Needles Syndrome
scispacy 1
질환 congenital X-linked dominant skeletal dysplasia scispacy 1
질환 exophthalmos C0015300
Exophthalmos
scispacy 1
질환 mandibular hypoplasia C0025990
Micrognathism
scispacy 1
질환 anodontia C0399352
Developmental absence of tooth
scispacy 1
질환 hypodontia C0020608
Hypodontia
scispacy 1
질환 oligodontia C0020608
Hypodontia
scispacy 1
질환 mandibular retrognathia C0025990
Micrognathism
scispacy 1
질환 MNS → Melnick-Needles syndrome scispacy 1
기타 collagen scispacy 1
기타 bilateral mandibular ramus osteotomies scispacy 1

MeSH Terms

Adult; Female; Humans; Orthognathic Surgical Procedures; Osteochondrodysplasias; Siblings

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