Medical College, Aga Khan University, Karachi, 74800, Pakistan.
Department of Oncology, Aga Khan University Hospital, Karachi, 74800, Pakistan.
Childs Nerv Syst. 2024 Nov;40(11):3537-3544. doi: 10.1007/s00381-024-06637-9. Epub 2024 Sep 30.
Diffuse intrinsic pontine glioma (DIPG) primarily affects pediatric patients. Data on the global incidence of DIPG remain sparse, especially in South Asia and low-middle-income countries like Pakistan.
After exemption from the Ethics Review Committee, a retrospective study was conducted. Records of patients with DIPG at the Aga Khan Hospital in Karachi, from January 2010 to December 2022, were reviewed.
A total of 35 pediatric patients were managed for DIPG. The median age of the patients was 9, with 19 (54.3%) males and 16 (45.7%) females. Cranial nerve palsies were the most common complaint and were present in 19 (54.3%) patients, followed by headaches in 18 (51.4%), long tract signs in 14 (40%), ataxia/cerebellar symptoms in 14 (40%), and seizures in 5 (14.3%). MRI was the primary diagnostic tool, used alone or with CT in 32 (94.1%) patients; CT alone was used in only 2 (5.7%) patients. Biopsy was performed in 10 (28.6%) patients. Primary radiation therapy was administered to 14 (40%) patients with 5400 cGy in 30 fractions. All these patients received steroids while none of them received reirradiation. VP shunt surgery for hydrocephalus was performed in 9 (25.7%) patients. Over half (54.3%) refused treatment post-diagnosis, and 71.4% were lost to follow-up.
Providing timely, quality multi-disciplinary care to DIPG patients within resource constraints remains challenging in Pakistan. However, recent developments show promise for improving DIPG care in the country.
弥漫性内在脑桥胶质瘤(DIPG)主要影响儿科患者。关于 DIPG 的全球发病率数据仍然很少,特别是在南亚和巴基斯坦等中低收入国家。
在获得伦理审查委员会豁免后,进行了一项回顾性研究。回顾了 2010 年 1 月至 2022 年 12 月在卡拉奇 Aga Khan 医院就诊的 DIPG 患者的记录。
共管理了 35 名儿科患者的 DIPG。患者的中位年龄为 9 岁,其中 19 名(54.3%)为男性,16 名(45.7%)为女性。颅神经麻痹是最常见的主诉,19 例(54.3%)患者存在颅神经麻痹,其次是头痛 18 例(51.4%)、长束征 14 例(40%)、共济失调/小脑症状 14 例(40%)和癫痫发作 5 例(14.3%)。磁共振成像(MRI)是主要的诊断工具,单独使用或与 CT 一起使用 32 例(94.1%)患者;仅单独使用 CT 的患者有 2 例(5.7%)。10 例(28.6%)患者进行了活检。14 例(40%)患者接受了原发性放射治疗,剂量为 5400cGy,分 30 次进行。所有这些患者都接受了类固醇治疗,而没有人接受再放疗。9 例(25.7%)脑积水患者接受了脑室-心房分流术。超过一半(54.3%)患者在诊断后拒绝治疗,71.4%的患者失访。
在资源有限的情况下,向 DIPG 患者提供及时、高质量的多学科护理仍然具有挑战性。然而,最近的发展表明,有希望改善该国的 DIPG 护理。