Objectives: Syringomyelia is a dilatation of the central canal of the cord frequently associated with congenital malformation of the cranial-cervical junction and Chiari I malformation. GH deficiency is also reported in these subjects and replacement therapy with GH seems not only to increase growth velocity but also to improve Chiari I malformation and syringomyelia. Methods: We report the case of a 4 year old child who undertook a RMN for a venous facial angioma which showed incidentally Chiari I malformation and cervical syringomyelia. Following neurosurgical decompression, he required surgical revision for cerebral spinal fluid fistula and recurrence of syringomyelia. He was then referred to our clinic for short stature (Height-3SDS; Growth velocity -1.83SDS). The main organic causes of short stature, such as coeliac disease, hypothyroidism and GH deficiency were excluded (GH arginine stimulation test peak value 12.7 ng/ml). GH therapy (0.5 mg/day) was started on the basis of previous studies reported in the literature showing an improvement of the syrinxes during GH treatment in absence of significant side effects. Since the response to treatment was unpredictable, we performed a close clinical follow-up with quarterly auxological examinations and annually RMN imaging. Results: The child showed during 3 years of GH treatment a significant increase of stature (from -3 to -1,7SDS) and Growth Velocity (from -1.83SDS to 3.67SDS). Furthermore the RMN showed a stabilization of the cranial-cervical junction and partial reduction of the syrinx cavity. Conclusions: Our case suggests the possible efficacy of GH therapy in children with short stature and syringomyelia regardless of the presence of GH deficiency.

GH THERAPY IN A NON-GHD SHORT CHILD WITH SYRINGOMYELIA AND CHIARI I MALFORMATION

Moretti A
Writing – Original Draft Preparation
;
SIMONCINI, DANIELA
Investigation
;
Cianci P
Investigation
;
Salvatoni A
Project Administration
2017

Abstract

Objectives: Syringomyelia is a dilatation of the central canal of the cord frequently associated with congenital malformation of the cranial-cervical junction and Chiari I malformation. GH deficiency is also reported in these subjects and replacement therapy with GH seems not only to increase growth velocity but also to improve Chiari I malformation and syringomyelia. Methods: We report the case of a 4 year old child who undertook a RMN for a venous facial angioma which showed incidentally Chiari I malformation and cervical syringomyelia. Following neurosurgical decompression, he required surgical revision for cerebral spinal fluid fistula and recurrence of syringomyelia. He was then referred to our clinic for short stature (Height-3SDS; Growth velocity -1.83SDS). The main organic causes of short stature, such as coeliac disease, hypothyroidism and GH deficiency were excluded (GH arginine stimulation test peak value 12.7 ng/ml). GH therapy (0.5 mg/day) was started on the basis of previous studies reported in the literature showing an improvement of the syrinxes during GH treatment in absence of significant side effects. Since the response to treatment was unpredictable, we performed a close clinical follow-up with quarterly auxological examinations and annually RMN imaging. Results: The child showed during 3 years of GH treatment a significant increase of stature (from -3 to -1,7SDS) and Growth Velocity (from -1.83SDS to 3.67SDS). Furthermore the RMN showed a stabilization of the cranial-cervical junction and partial reduction of the syrinx cavity. Conclusions: Our case suggests the possible efficacy of GH therapy in children with short stature and syringomyelia regardless of the presence of GH deficiency.
Moretti, A; Simoncini, Daniela; Md, ; Cianci, P; Salvatoni, A
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/11383/2071677
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