Gandhoke Charandeep S, Syal Simran K, Sharma Ajay, Srivastava Arvind K, Singh Daljit
Department of Neurosurgery, Maulana Azad Medical College, Lok Nayak Jai Prakash Narayan Hospital, Guru Nanak Eye Centre and Govind Ballabh Pant Institute of Postgraduate Medical Education and Research (GIPMER), New Delhi, India.
Department of Paediatrics, Sri Guru Ram Das Institute of Medical Sciences and Research, Amritsar, Punjab, India.
J Pediatr Neurosci. 2020 Apr-Jun;15(2):72-80. doi: 10.4103/jpn.JPN_101_18. Epub 2020 Jun 27.
This study aimed to analyze the spectrum and surgical outcome of cases of craniosynostosis operated at a tertiary referral institute in India.
This was a cross-sectional study.
We retrospectively examined 60 cases of craniosynostosis operated at our institute from 2008 to 2014 (with a minimum follow-up of 2 years). Data was collected including name, age, gender, involved sutures, other medical conditions, whether syndromic craniosynostosis or not, whether symptoms and signs of intracranial hypertension were present or not, associated findings on magnetic resonance imaging of brain and cervico-medullary junction, type of surgery performed, age at which surgery was performed, perioperative complications (if any), and findings on follow-up. To be able to analyze the surgical results, we used the seven category classification system used by Sloan
Craniosynostosis affected more men than women. The incidence of syndromic craniosynostosis was 11.67%. Mean age at first surgery was 3.85 years. Chiari malformation was present in 80% of the Crouzon's syndrome cases, 62.5% of the oxycephaly cases, and 4.44% of the non-syndromic, non-oxycephaly cases. Intracranial hypertension was present in 80% of the Crouzon's syndrome cases, 75% of the oxycephaly cases, and 6.67% of the non-syndromic, non-oxycephaly cases. Perioperative complications were present in 42.86% of the syndromic craniosynostosis cases, 50% of the oxycephaly cases, and 15.56% of the non-syndromic, non-oxycephaly cases. Compromised overall correction was present in 4 of 7 cases of syndromic craniosynostosis, 3 of 8 cases of oxycephaly, and 2 of 45 cases of non-syndromic, non-oxycephaly group.
The study highlights the importance of educating the masses so that cases of craniosynostosis present early. The incidence of Chiari malformation, intracranial hypertension, and perioperative complications was significantly higher in the syndromic craniosynostosis and oxycephaly groups than in single-suture craniosynostosis. The best surgical outcome and the least perioperative complications were seen in the trigonocephaly group. Compromised overall correction and reoperations were more common in the syndromic and complex craniosynostosis groups than in single-suture craniosynostosis.
本研究旨在分析在印度一家三级转诊机构接受手术治疗的颅缝早闭病例的谱系及手术结果。
这是一项横断面研究。
我们回顾性研究了2008年至2014年在我院接受手术治疗的60例颅缝早闭病例（最小随访期为2年）。收集的数据包括姓名、年龄、性别、受累缝线、其他疾病情况、是否为综合征性颅缝早闭、是否存在颅内高压的症状和体征、脑及颈髓交界处磁共振成像的相关发现、所施行的手术类型、手术时的年龄、围手术期并发症（如有）以及随访结果。为了能够分析手术结果，我们采用了斯隆使用的七类分类系统。
颅缝早闭在男性中的发病率高于女性。综合征性颅缝早闭的发病率为11.67%。首次手术的平均年龄为3.85岁。在克鲁宗综合征病例中，80%存在小脑扁桃体下疝畸形；在尖头畸形病例中，62.5%存在小脑扁桃体下疝畸形；在非综合征性、非尖头畸形病例中，4.44%存在小脑扁桃体下疝畸形。在克鲁宗综合征病例中，80%存在颅内高压；在尖头畸形病例中，75%存在颅内高压；在非综合征性、非尖头畸形病例中，6.67%存在颅内高压。综合征性颅缝早闭病例中42.86%出现围手术期并发症；尖头畸形病例中50%出现围手术期并发症；非综合征性、非尖头畸形病例中15.56%出现围手术期并发症。在综合征性颅缝早闭的7例病例中有4例出现整体矫正不足；在尖头畸形的8例病例中有3例出现整体矫正不足；在非综合征性、非尖头畸形组的45例病例中有2例出现整体矫正不足。
该研究强调了对公众进行教育以使颅缝早闭病例能早期就诊的重要性。综合征性颅缝早闭和尖头畸形组中，小脑扁桃体下疝畸形、颅内高压及围手术期并发症的发生率显著高于单缝颅缝早闭组。三角头畸形组的手术效果最佳，围手术期并发症最少。综合征性和复杂性颅缝早闭组中整体矫正不足及再次手术的情况比单缝颅缝早闭组更为常见。
