Anesthesia for a patient of acromesomelic dysplasia with associated hydrocephalus, Arnold Chiari malformation and syringomyelia

Abstract

Acromesomelic dysplasias are autosomal recessive osteochondrodysplasias. Acromesomelic dysplasia Maroteaux-type (AMDM), also known as St Helena dysplasia, is of two types: The classical and the mild variety. About 50 cases of AMDM have been reported till date, most of them being the classical variety. There is scarcity of literature on anesthesia for such patients. We are reporting a case of general anesthetic management of AMDM, associated with hydrocephalus, Arnold Chiari malformation type-1 and syringomyelia. The patient was a 10-year-old short-statured boy who presented with symptomatic thoracic kyphoscoliosis, gibbus deformity and back pain. On examination, there was no neurological deficit. Radiology revealed thoracic kyphoscoliosis, mild ventriculomegaly and upper cervical syringomyelia. The patient underwent posterior fossa decompression in the prone position under general anesthesia. We will discuss the anesthetic considerations for such patients and review the pertinent literature.

A case report of Arnold Chiari type 1 malformation in acromesomelic dwarf infant

“…Haldar et al . described about the anesthesia concerns involved in patients with Acromesomelic dysplasia with associated hydrocephalus, Arnold Chiari malformation and syringomyelia [ 5 ]. Arnold Chiari malformation is almost always associated with neural tube defects [ 6 ].…”

Anesthesia Experience Of A Pediatric Patient With Arnold Chiari Type I Malformation

“…In these patients kyphosis poses significant problems on airway safety. Both induction with inhalation agents leading to hypercarbia and increased intracranial pressures; and the probability of respiratory depression and apnea caused by intravenous induction agents due to difficult airway, demands greater concern on anesthesia safety on patients with this diagnosis (6). A retrospective study of pregnant women with syringomyelia reports that general and neuraxial anesthesia can be successfully performed without major long-term complications (7).…”

Title: A Dental Perspective on the Rare Disorder of Acromesomelic Dysplasia - A Case Report.

“…2 About 50 cases of AMDM have been reported till date, most of them being the classical variety. 3 This is an autosomal recessive skeletal dysplasia with a prevalence of ~1/1,000,000. 4 A diagnosis of AMDM type is usually made on the basis of combined clinical, genetic and radiographic findings.…”