Pregnancies and deliveries in patients with Charcot-Marie-Tooth disease

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Acta Med Croatica. 2010 Jul;64(3):215-20.

Pregnancies and deliveries in patients with Charcot-Marie-Tooth disease

Herman M, Delmis J, Ivanisevi & #263; M, Zupi & #263; T.

University Department of Gynecology and Obstetrics, Zagreb University Hospital

Center, Zagreb, Croatia.

Abstract

Hereditary motor sensory neuropathy (HMSN), also known as Charcot-Marie-Tooth

(CMT) disease, is a spectrum of disorders caused by a specific mutation in one

of several myelin genes, which results in defects in myelin structure,

maintenance and formation. Affected individuals show progressive distal limb

atrophy and weakness, often with gait disturbance and deformity of feet and

hands.

There have been few studies on how CMT disease can affect pregnancy, birth and

the newborn. CMT is an independent risk factor for complications during

pregnancy and delivery. Patients with CMT have more operative deliveries,

malpresentations and postpartum bleeding than the general obstetric population.

It is not clear whether the increased prevalence of malpresentation is related

to fetal disease, although the disorder typically does not present until later

in childhood.

Postpartum bleeding from atony may be related to the disease effect on uterine

adrenergic nerves. Exacerbation of CMT disease can occur in pregnancy, an effect

that may be mediated by increased plasma progesterone level.

Observations in an animal model were consistent with these findings as the

administration of progesterone resulted in a more progressive neuropathy, while

a progesterone antagonist slowed the disease progression.

We treated two patients with CMT (type 5 and type X1) at our Department. Both of

them had normal course of pregnancy until delivery. Emergency cesarean section

was performed in both cases; in one because of malpresentation, contracted

pelvis and signs of impending fetal asphyxiation during the second stage of

delivery, and in the other one based on neurologist indication.

In the latter, uterine atony with profuse postpartum bleeding occurred

immediately after cesarean section and emergency hysterectomy was performed

according to clinical status.