Surgical treatment of trigonocephaly

Özet

Sendromik olmayan sinostosisin nadir formu olan metopik sinostozisin anterior kalvariumda trigonosefaliye sebep olur. Trigonosefalide, bilateral frontotemporal daralmayla beraber kompansetuar biparietal ekspansiyon, supraorbital ve lateral retrüzyon ve hipotelorismle karakterize keel gemi şeklinde kafa mevcuttur. Bu çalışmada son 5 yıldır, 2005 ile 2010 yılları arasında, Plastik Cerrahi ve Beyin Cerrahi ekipleri tarafından trigonosefali tanısı alarak ameliyat edilmiş 9 hasta retrospektif olarak incelendi. Hastaların klinik özellikleri, yapılan cerrahi müdahaleler, komplikasyonlar ve takip periodları, ameliyat öncesi ve sonrası tomografileri incelendi. Cerrahi teknikte fronto-orbital barın orta hatta konan interpozisyonel kemik grefti ile beraber şekillendirilmesi ile frontal açılanma düzeltildi, frontal kemiğe yapılan multiple osteotomiler ve parietal kemiğe fıçı tahtası şeklindeki osteotomiler yapılarak anterior kalvarium şekillendirildi. Ameliyat edilen hastaların yaş ortalaması 11 aydı. İnterorbital mesafe 18.8mmden 20,9mme, biokuler mesafe 67.56mmden 74.39mme, interorbital mesafe 13.44mmden 15.65mme, intertemporal mesafe ise 58.22mmden 64.16mme genişledi. Ortalama takip süresi 34 ay olan hastalarda ciddi bir komplikasyon görülmedi. Trigonosefalinin cerrahi tedavisinde fronto-orbital ilerletme ve frontal ve parietal osteotomilerle kalvarium anterior 2/3ü şekillendirilir. Bu cerrahi teknikte yapılan osteotomiler sonrası oluşan kemik defekti minimaldir. Cerrahi sonuçlar genellikle hasta ebeveynlerinde ve hastalarda kozmetik olarak kafa şeklindeki düzelmeden dolayı memnuniyet vericidir.

Abstract

Metopic synostosis is a relatively rare form of nonsyndromic synostosis. Premature closure of the metopic suture results in deformation of the anterior calvarium, in the phenotypic features of trigonocephaly Trigonocephalic deformities are recognized because of a pathognomickeel-shaped' deformity of the forehead with a prominent midline ridge, bilateral frontotemporal constriction with compensatory biparietal expansion, supraorbital and lateral orbital retrusion and hypotelorism. 1 This article presents a surgical technique used over 5 years to treat trigonocephalic children. Retrospective analyses were performed on 9 patients with metopic synostosis treated during 2005-2010. The study included reviews of preoperative and postoperative computed tomography scans, operative techniques, clinical outcomes and complications. Operative techniques included fronto-orbital bandeau tilting after expansion with a midline interpositional -bone graft, detriangulation of foreheads with an inward bending at lateral orbital wall, multiple zigzag osteotomy of frontal bone, barrel-stave osteotomy of parietal bone. The average age of the patients at time of surgery was 11 months. Interorbital distances widened from 18.8 mm to 20.9 mm, biocular distance widened from 67.56 mm to 74.39mm, interorbital distance widened from 13.44mm to 15.65mm, intertemporal distance widened from 58.22mm to 64.16mm. Mean follow-up was 34 months, and no neurological sequelae or other significant complications were encountered. Trigonocephaly requires surgical correction involving anterior two-thirds calvarial remodeling with fronto-orbital advancement and frontal-bone remodeling. The described operative approach minimizes bone defects by adopting multiple zigzag osteotomy of the frontal bone. This modality results in significant improvements in skull form and high patient/parent satisfaction.