Growth hormone deficiency: a window to CNS malformations
DOI:
https://doi.org/10.18203/2349-3291.ijcp20252229Keywords:
Growth hormone deficiency, Chiari malformations, Pituitary hormonesAbstract
Chiari malformation denotes a pathological caudal ectopy of the cerebellar tonsils below the level of the foramen magnum. Several types of the condition exist, of which Type 1 is the most common. It often results in few symptoms, and in many cases is detected as an incidental finding when an MRI is performed. Chiari type 1 has been hypothesized to be associated with Growth Hormone deficiency. A 6-year-old female child, 2nd by birth order born of non- Consanguineous marriage presented with short stature (83cm, below 3rd SD) with normal head size, normal US:LS ratio with no motor developmental delay and the child's height was below the mid parental height percentile. Work up for proportional short stature was done and child's basal Growth Hormone levels were low -0.153ng/ml (2-5 ng/ml) and IGF-1 levels were low 1736 ng/ml (2188-4996 ng/ml). The thyroid function test, cortisol levels, calcium, phosphorus, ALP were normal. Growth Hormone stimulation with clonidine revealed low post stimulation GH levels being 0.177, 0.86, 1.1, 0.6, 0.66 respectively at 0, 30, 60, 90 mins and GH stimulation with Glucagon revealed low post stimulation GH levels being 0.19, 0.27, 0.18, 0.74, 0.32, 0.32 respectively at 0, 30, 60 ,90, 120 mins. Work up for other pituitary hormones were normal. MRI brain of the child was done which showed cerebellar tonsillar herniation with likely syrinx in visualized section of cervical spinal canal cord suggestive of Chiari malformation type 1.
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Kahn EN, Muraszko KM, Maher CO. Prevalence of Chiari I malformation and syringomyelia. Neurosurg Clin N Am. 2015;26(4):501–7. DOI: https://doi.org/10.1016/j.nec.2015.06.006
Shamji MF, Ventureyra ECG, Baronia B, Nzau M, Vassilyadi M. Classification of symptomatic Chiari I malformation to guide surgical strategy. Can J Neurol Sci. 2010;37(4):482–7. DOI: https://doi.org/10.1017/S0317167100010507
Aydin S, Hanimoglu H, Tanriverdi T, Yentur E, Kaynar MY. Chiari type I malformations in adults: a morphometric analysis of the posterior cranial fossa. Surg Neurol. 2005;64(3):237–41. DOI: https://doi.org/10.1016/j.surneu.2005.02.021
Dagtekin A, Avci E, Kara E, Uzmansel D, Dagtekin O, Koseoglu A, et al. Posterior cranial fossa morphometry in symptomatic adult Chiari I malformation patients: comparative clinical and anatomical study. Clin Neurol Neurosurg. 2011;113(5):399–403. DOI: https://doi.org/10.1016/j.clineuro.2010.12.020
Tubbs RS, Beckman J, Naftel RP, Chern JJ, Wellons JC, Rozzelle CJ, et al. Institutional experience with 500 cases of surgically treated pediatric Chiari malformation Type I. J Neurosurg Pediatr. 2011;7(3):248–56. DOI: https://doi.org/10.3171/2010.12.PEDS10379
Chinoy A, Murray PG. Diagnosis of growth hormone deficiency in the paediatric and transitional age. Best Pract Res Clin Endocrinol Metab. 2016;30(6):737–47. DOI: https://doi.org/10.1016/j.beem.2016.11.002
Gupta A, Vitali AM, Rothstein R, Cochrane DD. Resolution of syringomyelia and Chiari malformation after growth hormone therapy. Childs Nerv Syst. 2008;24(11):1345–8. DOI: https://doi.org/10.1007/s00381-008-0675-8
Avellino AM, Kim DK, Weinberger E, Roberts TS. Resolution of spinal syringes and Chiari I malformation in a child. Case illustration. J Neurosurg. 1996;84(4):708. DOI: https://doi.org/10.3171/jns.1996.84.4.0708
Ellenbogen RG, Roberts TS. Spontaneous resolution of a cardiothoracic syrinx in a child. Case report and review of the literature. Pediatr Neurosurg. 1999;30(1):43–6. DOI: https://doi.org/10.1159/000028760
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