Binder syndrome: Clinical findings and surgical treatment of 18 patients at the Department of Plastic Surgery in Polanica Zdrój.

Advances in clinical and experimental medicine : official organ Wroclaw Medical University 2017 Vol.26(3) p. 427-437

Drozdowski PH, Łątkowski I, Zachara MG, Wójcicki P

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Abstract

[BACKGROUND] Binder syndrome (BS) is an uncommon congenital underdevelopment of the maxilla and nasal skeleton. Other clinical features include a hypoplastic or absent anterior nasal spine; a short, flat nose with short columella; an acute nasolabial angle; a convex upper lip and class III malocclusion.

[OBJECTIVES] The aim of the study was to outline the major characteristics of BS and to present a variety of surgical treatment methods.

[MATERIAL AND METHODS] The study included 18 patients treated in the authors' department from 1989 to 2013.

[RESULTS] The patients were predominantly women, aged 6 months to 34 years. Nine patients did not present any co-morbidities, but in the other 9 the most common co-morbidities were a unilateral cleft lip and palate, followed by a cleft palate, a bilateral cleft lip and palate, a cleft lip, GERD, gluten intolerance, oligophrenia, goiter and foot malformation. Most of the patients had not been operated on previously. The most common procedure carried out was an iliac crest bone graft. In 4 patients, no procedures other than cleft lip and palate repair were undertaken. In 3 cases a Le Fort I osteotomy was performed to correct the patients' orthognathic status. No major or life-threating complications were noted. In 2 cases, due to a high degree of resorption of bone grafts, multiple secondary grafting of bone, cartilage and deepithelialized skin was necessary to obtain satisfactory results.

[CONCLUSIONS] In BS surgical treatment is the treatment of choice. It results in adequate correction of facial retrusion. However, due to various degrees of bone resorption, the results are not lifelong. No unequivocally superior surgical strategy in BS has been presented so far. Most disagreement among authors is related to the need for and timing of maxillary osteotomy, the choice between bone and cartilage grafting in nose reconstruction and columella lengthening. Although alloplastic materials offer the tempting advantage of fast and simultaneous augmentation of deficient tissues, their use may risk prolonged infections and extrusion, resulting in exacerbations of deformities.

추출된 의학 개체 (NER)

유형영어 표현한국어 / 풀이UMLS CUI출처등장
해부 columella scispacy 1
해부 palate scispacy 1
해부 bone scispacy 1
해부 cartilage scispacy 1
해부 skin scispacy 1
해부 nose scispacy 1
해부 tissues scispacy 1
해부 maxilla 상악골 dict 1
합병증 nasolabial scispacy 1
약물 [BACKGROUND] Binder scispacy 1
약물 [OBJECTIVES] scispacy 1
약물 [MATERIAL AND scispacy 1
약물 Le Fort I scispacy 1
약물 [CONCLUSIONS] In scispacy 1
질환 cleft palate C0008925
Cleft Palate
scispacy 1
질환 GERD C0017168
Gastroesophageal reflux disease
scispacy 1
질환 gluten intolerance C0007570
Celiac Disease
scispacy 1
질환 oligophrenia C0549491
Oligophrenia
scispacy 1
질환 goiter C0018021
Goiter
scispacy 1
질환 infections C0851162
Infections of musculoskeletal system
scispacy 1
질환 exacerbations of deformities scispacy 1
질환 lip scispacy 1
질환 Binder syndrome C0220692
Maxillonasal dysplasia, Binder type
scispacy 1
질환 hypoplastic or absent anterior nasal spine scispacy 1
질환 malocclusion C0024636
Malocclusion
scispacy 1
질환 cleft lip C0008924
Cleft upper lip
scispacy 1
질환 palate C0700374
Palate
scispacy 1
기타 patients scispacy 1
기타 nasal scispacy 1
기타 anterior nasal scispacy 1
기타 class III scispacy 1
기타 women scispacy 1
기타 GERD scispacy 1
기타 iliac crest bone graft scispacy 1
기타 bone grafts scispacy 1
기타 maxillary scispacy 1

MeSH Terms

Adolescent; Adult; Bone Transplantation; Child; Child, Preschool; Cleft Lip; Cleft Palate; Female; Humans; Infant; Male; Malocclusion, Angle Class III; Maxilla; Maxillofacial Abnormalities; Nose; Osteotomy, Le Fort; Plastic Surgery Procedures; Surgery, Plastic; Young Adult

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