Difficult airway in a child with spinal muscular atrophy type I

Abstract

Spinal muscular atrophy (SMA) type I is a relatively common inherited neuromuscular disease of hypotonic newborns, but is not associated with craniofacial abnormalities. There is nothing in the literature about difficult intubation in patients affected by this disease. We report a case of 34-month-old girl with SMA type I who was scheduled for emergency endoscopic laser treatment of tracheal stenosis caused by granulations. Tracheostomy was performed at 17 months of age and before this, the orotracheal tube was changed periodically without difficulty. For this laser treatment, orotracheal intubation was required. Preoperative physical examination revealed micrognathia and class II malocclusion. Opening her mouth was not difficult. Although difficult orotracheal intubation was predictable, we attempted to intubate her trachea as usual, but could not visualize the epiglottis. We decided to proceed with retrograde intubation, one of the standard techniques employed in a child with a difficult airway, via the tracheostome. A feeding nasogastric catheter was used as a guide catheter, and our strategy was successful. In this study we report a case of difficult airway in a child with SMA type I. The relationship between SMA type I with a tracheostome and difficult airway are discussed.

Anesthesia and perioperative medical management of children with spinal muscular atrophy

“…Our series demonstrated no adverse anesthetic effects secondary to the use of nondepolarizing muscle relaxants, opioids, sedative/hypnotics, inhaled anesthetics, and local anesthetics. This is commensurate with prior reports of safe delivery of both balanced anesthesia of inhalation agents and intravenous opioids or safe application of neural axis blockade and TIVA in patients with SMA (5,8,18). No complications were reported in the one child who received succinylcholine.…”

“…The choice of airway safeguard and devices (e.g., face mask, laryngeal mask airway [LMA], endotracheal tube) is dictated by the type of surgery. However, direct laryngeal intubation may become progressively difficult with severe or advanced muscle atrophy, particularly in SMA type I, because of atrophy of masseter and other muscles of mastication as was noted unexpectedly in one patient in our series (18,23). Anesthetic technique of spontaneous breathing via face mask or LMA may be indicated for short and minimally invasive procedure such as muscle biopsy, bearing in mind the potential for laryngospasm and need for administering nondepolarizing muscles relaxant that may prolong emergence or need for prolonged ventilatory support.…”

A retrospective cohort study of children with spinal muscular atrophy type 2 receiving anesthesia for intrathecal administration of nusinersen

“…Airway management of patients with SMA can be hampered by limited cervical spine mobility and ankyloses of the mandibular joint which is found in 30% of teenagers with SMA and in 80% of patients over the age of 30 . Restrictive neuromuscular pulmonary disease is a hallmark of SMA with pulmonary insult related to the severity of the disease.…”

“…Airway management of patients with SMA can be hampered by limited cervical spine mobility and ankyloses of the mandibular joint which is found in 30% of teenagers with SMA and in 80% of patients over the age of 30. [11][12][13] Restrictive neuromuscular pul- | 1107 involvement in SMA is related both to bulbar dysfunction and gastrointestinal motility problems. Bulbar dysfunction can lead to dysphagia and aspiration, often necessitating gastrostomy tube placement.…”

Using General Anesthesia plus Muscle Relaxant in a Patient with Spinal Muscular Atrophy Type IV: A Case Report

“…Dose requirements for spinal anesthesia were difficult to predict, increasing the risk of either a failed or high block. CSE or continuous spinal technique may allow the block height to be titrated more carefully and should be considered ideal [11–15]. …”