///2010 Abstract Details
2010 Abstract Details2019-08-03T15:49:10-05:00


Abstract Number: 176
Abstract Type: Case Report/Case Series

Krzysztof M. Kuczkowski M.D.1 ; Claudia L. Fernndez M.D.2; Leon Drobnik M.D.3; Susilo Chandra M.D.4

Introduction: The use of central neuraxial blockade in patients with preexisting neurological disorders is controversial (theoretical risks of worsening of neurological symptoms following regional anesthesia). Charcot-Marie-Tooth (CMT) disease is a heterogeneous group of genetic disorders presenting with the phenotype of a chronic progressive neuropathy affecting both the sensory and motor nerves (1). We herein report a case of a parturient with CMT disease who received an uneventful spinal anesthesia for cesarean delivery.

Report of case: A 26-year-old G1P0 female at 37 weeks of gestation and coexisting CMT disease was admitted to Labor & Delivery suite with spontaneous ruptures of membranes. Typical signs of CMT disease (peroneal weakness, sensory deficits at the feet and impaired tendon reflexes) were noted on preanesthetic neurological examination. Single dose spinal anesthesia with 12 mg of 0.75% bupivacaine, 10 mcg of fentanyl and 0.2 mg of morphine at the L3-L4 interspace was used for cesarean section. No worsening of CMT disease was reported in the immediate postoperative period and during a follow up visit 2 weeks after surgery.

Discussion: CMT disease or hereditary motor and sensory neuropathy (HMSN) is a genetically heterogeneous group of conditions that affect the peripheral nervous system. The disease is characterized by degeneration or abnormal development of peripheral nerves and exhibits a range of patterns of genetic transmission (1). The classification of CMT is complex and undergoes constant revisions as new genes and mutations are discovered. Disease course is variable because of genotypic and phenotypic heterogeneity. Typical patients present with distally accentuated motor weakness, muscle wasting, and sensory loss leading to significant and progressive clinical morbidity and impaired quality of life. At present, there is no drug therapy for CMT disease, and rehabilitation therapy and surgical procedures for skeletal deformities are the only available treatments, although best approach has not been defined. Only sparse information is available concerning the relationship between pregnancy and CMT. CMT disease increases the risk for complications during labor and delivery, which is linked to a higher occurrence of emergency interventions during birth. Opinions regarding safety of regional anesthesia in patients with CMT are often contradictory and based on theory rather than evidence based medicine. Our report confirms the developing consensus that regional anesthesia is a safe alternative to general anesthesia in parturients with CMT.

Conclusion: Given the fact that CMT is the most common inherited neuromuscular disorder, it is imperative to develop an evidence based approach to the peripartum (obstetric and anesthetic) management of parturients with coexisting CME disease.

References: 1. Neuromuscul Disord 2009;19:675-9.

SOAP 2010