Advances in Clinical and Experimental Medicine

Title abbreviation: Adv Clin Exp Med
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ISSN 1899–5276 (print)
ISSN 2451-2680 (online)
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Advances in Clinical and Experimental Medicine

2017, vol. 26, nr 3, May-June, p. 427–437

doi: 10.17219/acem/62123

Publication type: original article

Language: English

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

Piotr H. Drozdowski1,A,D, Ireneusz Łątkowski1,2,C, Mateusz G. Zachara1,B,E, Piotr Wójcicki1,2,F

1 Plastic Surgery Unit, Specialist Medical Centre, Polanica Zdrój, Poland

2 Plastic Surgery Clinic, Wroclaw Medical University, Specialist Medical Center, Polanica Zdrój, Poland

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.
Conclusion. 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.

Key words

Binder syndrome, maxillary underdevelopment, bone graft, cartilage graft, resorption

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