Cricopharyngeal Achalasia Associated with Arnold-Chiari Malformation in Childhood

Abstract

Six patients with myelomeningocele and the Arnold-Chiari malformation developed cricopharyngeal achalasia and lower cranial nerve deficits. Diagnosis is established by cine-esophagram. Distortion of the brain stem or cranial nerves secondary to the Arnold-Chiari malformation may produce the autonomic imbalance necessary for cricopharyngeal achalasia. Treatment is supportive and includes verification of cerebral spinal fluid shunt function. Suboccipital craniectomy may reverse progressive lower cranial nerve deficits and reduce cricopharyngeus spasm. Cricopharyngeal myotomy may be considered when the cranial nerve deficits and cricopharyngeal achalasia are fixed, irreversible, and continue to cause disability.

Intraoperative Autonomic Dysfunction Associated with Arnold-Chiari Malformation

“…They may develop apnea if they are breathing [9] spontaneously and may show signs of cardiovascular instability, as evidenced in our cases.…”

Derangement of swallowing in children with myelomeningocele

“…Swallowing abnormalities in children with myelomeningocele and Chiari malformation have been reported and partially described earlier [11][12][13]. Reichert et al reported that congenital cricopharyngeal achalasia in four children with myelomeningocele abated after ventricular shunt revision (2/4), posterior fossa decompression (1/4), and myotomy and gastrostomy (1/4).…”

“…Reichert et al reported that congenital cricopharyngeal achalasia in four children with myelomeningocele abated after ventricular shunt revision (2/4), posterior fossa decompression (1/4), and myotomy and gastrostomy (1/4). Shunt revision was performed in 4 of the 6 myelomeningocele patients with cricopharyngeal achalasia studied by Gendell et al [11]. Only two improved after ventricular shunt alone.…”

“…McLone: Swallowing disorders with myelomeningocele tion or pressure on the cranial nerves is the most likely cause of the respiratory and swallowing problems. Both ventricular shunting and posterior fossa decompression are known to produce a rapid, if sometimes temporary, amelioration of symptoms [11][12][13]. Such procedures would have little effect if the abnormalities of swallowing and respiration were due to immutable destruction of tissue by hemorrhage or congenital malformation of brainstem nuclei.…”

“…Well-documented congenital or genetic diseases associated with abnormal deglutition during childhood include: Mobius syndrome, Friedreich's ataxia, familial dysautonomia, and cerebral palsy [5][6][7][8][9][10]. Children with myelomeningocele may also experience disordered swallowing [11][12][13]. We herein report the swallowing abnormalities observed in 13 children with myelomeningocele and discuss possible relationship to the Chiari malformation of the brain present in these patients.…”