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Neurological Surgery No Shinkei Geka Volume 37, Issue 1 （January 2009）

Neurological Surgery 脳神経外科 37巻1号（2009年1月発行）

Japanese English

研究

重症のsyndromic craniosynostosisに対する集学的治療 Multidisciplinary Treatment for Severe Syndromic Craniosynostosis 鵜山淳 ¹ , 河村淳史 ¹ , 山元一樹 ¹ , 長嶋達也 ¹ , 西本操 ⁷ , 大山知樹 ² , 西島栄治 ³ , 佐藤志以樹 ³ , 中尾秀人 ⁴ , 野村耕治 ⁵ , 大津雅秀 ⁶ , 阪本浩一 ⁶ Atsushi UYAMA ¹ , Atsufumi KAWAMURA ¹ , Kazuki YAMAMOTO ¹ , Tatsuya NAGASHIMA ¹ , Soh NISHIMOTO ⁷ , Tomoki OYAMA ² , Eiji NISHIJIMA ³ , Shiiki SATOH ³ , Hideto NAKAO ⁴ , Koji NOMURA ⁵ , Masahide OTSU ⁶ , Koichi SAKAMOTO ⁶ ¹兵庫県立こども病院脳神経外科 ²兵庫県立こども病院形成外科 ³兵庫県立こども病院小児外科 ⁴兵庫県立こども病院新生児科 ⁵兵庫県立こども病院眼科 ⁶兵庫県立こども病院耳鼻科 ⁷兵庫医科大学形成外科 ¹Department of Neurosurgery,Hyogo Prefectural Kobe Children's Hospital ²Department of Plastic Surgery,Hyogo Prefectural Kobe Children's Hospital ³Department of Pediatric Surgery,Hyogo Prefectural Kobe Children's Hospital ⁴Department of Neonatology,Hyogo Prefectural Kobe Children's Hospital ⁵Department of Ophthalmology,Hyogo Prefectural Kobe Children's Hospital ⁶Department of Otorhinolaryngology,Hyogo Prefectural Kobe Children's Hospital ⁷Department of Plastic surgery,Hyogo College of Medicine キーワード： syndromic craniosynostosis , fronto-orbital advancement by gradual distraction , posterior cranial remodeling , ventriculoperitoneal shunt , tracheotomy Keyword: syndromic craniosynostosis , fronto-orbital advancement by gradual distraction , posterior cranial remodeling , ventriculoperitoneal shunt , tracheotomy pp.25-34

発行日 2009年1月10日 Published Date 2009/1/10

DOI https://doi.org/10.11477/mf.1436100868

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Ⅰ．緒言

　頭蓋縫合早期癒合症は，1万人に1～5人に発生する^44,45)．頭蓋縫合早期癒合症のうち頭蓋冠および顔面骨に広範な形態異常を合併するものはsyndromic craniosynostosisに分類され⁷⁾，重症例では頭蓋顔面奇形に対する乳児期からの多段階的手術や水頭症の管理，気管形成不全に対する呼吸管理などの集学的治療が必要である．今回，当院で経験した乳児期の頭蓋形成術，水頭症の管理，気管切開と人工呼吸器管理を要した最重症のsyndromic craniosynostosis 6例の治療と問題点につき報告する．

　We describe the treatment of patients having syndromic craniosynostosis with severe craniofacial abnormality. One patient had Cruzon's syndrome, one had Beare-Stevenson cutis gyrata syndrome, and four had Pfeiffer's syndrome. Anterior cranial deformity in all patients was treated using fronto-orbital advancement (FOA) by gradual distraction. Initially, the frontal and supraorbital bones were removed and remodeled. Next, the frontal bones were fixed loosely to the supraorbital bones with absorbable threads. Then, the supraorbital and temporal bones were connected using distraction devices on both sides. The temporal bones were thereafter reinforced with titanium plates. Distraction was started one week postoperation, and the mean amount of elongation was 28.9mm. Distraction devices were removed one to five months after the operation. One case required FOA by the traditional method ten months after the initial operation. Local infection was observed in three cases, but there were no major complications. Posterior cranial remodelings were performed in five cases, with one requiring a second operation. We chose the appropriate procedure according to the degree of cranial deformity and operative findings. We performed decompression of the foramen magnum in five cases and laminectomy of the atlas in two cases. Ventriculoperitoneal shunt for hydrocephalus and tracheotomy for airway obstruction were performed in all cases. Cranial remodeling for treating severe craniofacial abnormality requires careful inspection of abnormalities, proper timing, close attention to the procedure and adequate perioperative care. Multidisciplinary therapy is essential for treating severe syndromic craniosynostosis due to systematic osseocartilaginous aplasia.