Anesth Analg 2000;91:858-859
© 2000 International Anesthesia Research Society
PEDIATRIC ANESTHESIA
Spinal Anesthesia For Gastrostomy in an Infant with Nemaline Myopathy
Ze’ev Shenkman, MD*,
Offer Sheffer, MD*,
Ilan Erez, MD
,
Ita Litmanovitc, MD
, and
Robert Jedeikin, BSc, MB ChB, FFA*
Departments of
*Anesthesia and Critical Care Medicine,
Pediatric Surgery, and
Neonatal Intensive Care Unit, Meir General Hospital, Kfar-Saba, Sakler School of Medicine, Tel Aviv University, Israel
Address correspondence to Ze’ev Shenkman, MD, Department of Anesthesia, Meir General Hospital, 59 Tshernichovsky St., Kfar-Saba 44281, Israel. Address e-mail to shlomsh{at}netvision.net.il
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Abstract
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Implications: We report a case of spinal anesthesia in an infant with nemaline myopathy. Spinal anesthesia was administered to preempt the potential problems of difficult tracheal intubation and prolonged mechanical ventilation secondary to muscle weakness after the use of IV anesthetics in this patient.
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Introduction
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Infantile nemaline myopathy (NM) is a rare genetic disease with varying inheritance characterized by thin muscle mass and generalized hypotonia (1,2). The anesthetic management of patients with NM may be complicated by poor respiratory reserve (2–4), difficult tracheal intubation (2,3,5), and a possible susceptibility to malignant hyperthermia (2). The administration of general anesthesia in these patients has been reported (2–5). We describe the use of spinal anesthesia in an infant with NM undergoing gastrostomy and muscle biopsy.
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Case Report
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A 6-wk-old, 3.0-kg, full-term infant was scheduled for gastrostomy and quadriceps femoris muscle biopsy because of severe hypotonia and poor sucking. Family history disclosed a sibling with NM confirmed histologically and two hypotonic siblings who had died at age 6 days and 1 yr. Immediately after birth, tracheal intubation and mechanical ventilation were needed for 24 h because of apnea and cyanosis. Recurrent bradycardia and desaturation appeared after extubation and were treated by using nasal oxygen. Physical examination disclosed a moderately severe, generalized hypotonia, high-arched cleft palate, and a 2/6 systolic murmur. Echocardiography revealed a patent foramen ovale and a 1- to 2-mm ventricular septal defect with a small left-to-right shunt.
Preoperative fasting lasted 6 h, during which the infant received IV fluids at a rate of 12 mL/h. Thirty minutes before anesthesia, IV ampicillin and gentamicin were administered. After routine monitors were applied, the patient was placed in the sitting position, and oxygen was blown over her face. Spinal anesthesia was performed by using 0.5% hyperbaric bupivacaine (3 mg) with 40 µg epinephrine injected through a 25-gauge, 25.4-mm spinal needle at the L4-5 intervertebral space. The patient was held in the reverse-Trendelenburg position for 2 min before returning her to the supine position. She was awake and comfortable throughout the 65-min procedure, except at the end when the bowel had to be contained inside the abdomen to allow suturing of the surgical wound. Bupivacaine infiltration into the surgical wound and 70% N2O in oxygen administration via a face mask for 12 min resulted in the cessation of crying. The rest of the procedure was uneventful. Hemodynamic stability and normal oxygenation were maintained. The surgical wound at the biopsy site was also infiltrated with bupivacaine, and the patient was returned to the neonatal intensive care unit. The postoperative course was unremarkable.
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Discussion
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In infantile NM, rod-shaped, inclusion-like abnormal structures made of excessive Z-band material are found within skeletal muscle fibers (1,2). All skeletal muscles, including the diaphragm, may be affected (3). Clinical manifestations include thin muscle mass, generalized hypotonia, restrictive lung disease (2–4) with increased dead-space respiration and alveolar-to-arterial oxygen gradient (5), respiratory insufficiency (2,3), swallowing abnormality (2,4), pulmonary aspiration (2,3), recurrent pneumonia (2), facial dysmorphism (2,3) including prognathism (2,5), micrognathia (3), high arched (1,2) or cleft (1) palate, and chest (3) and other skeletal deformities (2). Severe weakness may appear at birth, and some patients die in the neonatal period; survivors must use a wheelchair (1). Serum creatine phosphokinase levels are usually (1,2), but not always (2), normal. Congenital heart disease (2) or cardiomyopathy (1,5) rarely coexist.
Factors that may complicate the perianesthetic course of patients with NM include difficult tracheal intubation (2,3,5), respiratory compromise (2–5), pulmonary aspiration (2,3), possible susceptibility for malignant hyperthermia (2), abnormal response to muscle relaxants, and cardiomyopathy (1,5) or congenital heart disease (2). Unexpectedly, a normal response to pancuronium and resistance to succinylcholine with no hyperkalemia after its administration were reported in a single patient (4). No cases of malignant hyperthermia have been reported in patients with NM (3).
Because respiratory failure is the most frequent cause of death, prevention of perioperative pulmonary complications is crucial (2). We provided spinal anesthesia to avoid these upper airway and respiratory complications and to prevent possible prolonged mechanical ventilation secondary to muscle weakness after the administration of IV anesthetics or muscle relaxants (3). A possible disadvantage of spinal anesthesia is loss of lower intercostal muscle activity (6), further jeopardizing the respiratory status (5,6). The use of bupivacaine, rather than tetracaine, may reduce this risk because of its less profound motor block. Preoperative fasting lasted longer than normal to prevent pulmonary aspiration (2,3).
In conclusion, a successful case of spinal anesthesia in an infant with NM is reported. We consider spinal anesthesia a useful technique in such infants. Appropriate patient positioning, drug selection, and dosing are important for a successful anesthesia management.
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References
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Accepted for publication June 13, 2000.
This article has been cited by other articles:
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Z.'e. Shenkman, D. Hoppenstein, I. Litmanowitz, S. Shorer, M. Gutermacher, L. Lazar, I. Erez, R. Jedeikin, and E. Freud
Spinal anesthesia in 62 premature, former-premature or young infants-technical aspects and pitfalls: [La rachianesthesie chez 62 enfants prematures, anciens prematures ou jeunes enfants - aspects techniques et pieges]
Can J Anesth,
March 1, 2002;
49(3):
262 - 269.
[Abstract]
[Full Text]
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Abstract
Introduction
