Case report
Anesthetic management for patients with arthrogryposis multiplex congenita and severe micrognathia: case reports

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Abstract

Arthrogryposis multiplex congenita (AMC) is a spectrum syndrome of multiple persistent limb contractures often accompanied by associated anomalies, including cleft palate, genitourinary defects, gastroschisis, and cardiac defects. Pediatric patients with AMC frequently present for multiple surgeries requiring general endotracheal anesthesia. We describe our anesthetic experience with the laryngeal mask airway and endotracheal tube in two neonates with AMC and severe micrognathia. We discuss AMC and outline the problems encountered in difficult airway management.

Introduction

There are few documented cases in the anesthesia literature of anesthetic management for patients with arthrogryposis multiplex congenita (AMC). This is a spectrum syndrome of multiple, persistent limb contractures often with other unrelated associated anomalies, such as cleft palate, genitourinary defects, gastroschisis, and cardiac defects.1 Children diagnosed with AMC often present for multiple surgeries requiring general endotracheal anesthesia. Anticipated problems include difficult airway management because of micrognathia, difficult venous accesses caused by the contractures, and an increased potential for hyperpyrexia.2 The anesthetic management of two neonates with AMC and severe micrognathia are presented.

Section snippets

Case 1

A 3-month-old, 4-kg male infant presented for laparoscopic gastrostomy tube placement and bilateral inguinal hernia exploration. The birth history revealed that the infant was born at term, with a birth weight of 3.03 kg. Because of multiple, severe limb contractures, a diagnosis of AMC was made at birth. He had undergone no previous surgeries. Physical examination revealed an awake infant with severe, multiple joint contractures involving all limbs and marked micrognathia (Figure 1).

Discussion

AMC was originally described by Otto in 1841 as a congenital myodystrophy. The etiology of this condition is widely debated. Many authors concluded that AMC is not a disease, but a syndrome complex that may be a combination of central nervous system disorders, muscular disorders, or both.5 Oberoi et al.6 have written an extensive summary of this syndrome and a case report of anesthetic management for surgery in an 11-year-old child with AMC who appeared to have no airway difficulties.

The

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    Citation Excerpt :

    Trauma commonly compromises the airway in all patients and amplifies the difficulty in this subset of patients; the problem is further compounded in emergency situations. There have been many case reports describing the anesthetic management for elective operative procedures in patients with AMC,6–9 but a situation of “cannot ventilate cannot intubate” in a trauma setting has not been discussed previously. A difficult or rather impossible laryngoscopy and/or intubation can be anticipated in up to 25% of AMC patients.

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Dr. Mayhew’s email address is [email protected]

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