The trends in diagnosis, management, and care of patients with diffuse intrinsic pontine gliomas: Perspectives from a tertiary care hospital of pakistan

Childs Nerv Syst. 2024 Nov;40(11):3537-3544. doi: 10.1007/s00381-024-06637-9. Epub 2024 Sep 30.

Abstract

Background: 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.

Methods: 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.

Results: 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.

Conclusion: 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.

Keywords: Brainstem; Glioma; Low- and middle-income countries (LMIC); Pakistan; Pontine.

MeSH terms

  • Adolescent
  • Brain Stem Neoplasms* / diagnosis
  • Brain Stem Neoplasms* / epidemiology
  • Brain Stem Neoplasms* / therapy
  • Child
  • Child, Preschool
  • Diffuse Intrinsic Pontine Glioma* / diagnosis
  • Diffuse Intrinsic Pontine Glioma* / epidemiology
  • Diffuse Intrinsic Pontine Glioma* / therapy
  • Female
  • Humans
  • Infant
  • Male
  • Pakistan / epidemiology
  • Retrospective Studies
  • Tertiary Care Centers* / trends