Techlemetzi Nikolitsa, Katsoudas Sokratis, Tsitsekli Evangelia, Pichlinski Ioannis, Polychroni Ioulia, Zosi Paraskevi
Pediatrics, General Hospital of Nikaia Peiraia Agios Panteleimon, Nikaia, GRC.
Pediatric Endocrinology, Private Unit, Athens, GRC.
Cureus. 2025 Aug 24;17(8):e90868. doi: 10.7759/cureus.90868. eCollection 2025 Aug.
Chiari I malformation (CIM) and idiopathic growth hormone deficiency (GHD) are distinct, yet occasionally coexisting conditions in pediatric patients. The underlying pathophysiological link between the two entities remains controversial. In this case series, we present five pediatric patients with concurrent CIM and idiopathic GHD, detailing their clinical presentations, diagnostic workups, and responses to recombinant human growth hormone (rhGH) therapy. Notably, three of the cases were asymptomatic for CIM, with the malformation incidentally detected during evaluations for short stature. All patients demonstrated a positive growth response to rhGH treatment, with no evidence of CIM progression over the monitoring period. Our literature review highlights several proposed mechanisms linking CIM and GHD, including the potential effects of breech delivery, congenital midline developmental anomalies, and an underdeveloped posterior cranial fossa. Despite concerns in some reports about the possible exacerbation of CIM symptoms with rhGH therapy, such as persistent headaches, cerebellar ataxia, or spinal cord-related symptoms, our findings suggest that, when administered under careful multidisciplinary supervision, rhGH can effectively promote growth without adversely affecting CIM. To conclude, we highlight the importance of close collaboration between endocrinologists and neurologists in managing patients with these coexisting conditions.
Chiari I型畸形(CIM)和特发性生长激素缺乏症(GHD)是不同的,但在儿科患者中偶尔会同时存在。这两种疾病之间潜在的病理生理联系仍存在争议。在这个病例系列中,我们介绍了五名同时患有CIM和特发性GHD的儿科患者,详细描述了他们的临床表现、诊断检查以及对重组人生长激素(rhGH)治疗的反应。值得注意的是,其中三例CIM无症状,在对身材矮小进行评估时偶然发现了这种畸形。所有患者对rhGH治疗均表现出积极的生长反应,在监测期内没有CIM进展的证据。我们的文献综述强调了几种将CIM和GHD联系起来的机制,包括臀位分娩的潜在影响、先天性中线发育异常以及后颅窝发育不全。尽管一些报告担心rhGH治疗可能会加重CIM症状,如持续性头痛、小脑共济失调或脊髓相关症状,但我们的研究结果表明,在多学科的仔细监督下给药时,rhGH可以有效促进生长而不会对CIM产生不利影响。总之,我们强调内分泌学家和神经学家在管理这些并存疾病患者方面密切合作的重要性。