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Combined General and Regional Anesthetic in a Child with Aromatic l-Amino Acid Decarboxylase Deficiency

Section Editor(s): Shafer, Steven L.Berkowitz, Darryl H. MD; Ganesh, Arjunan MBBS

doi: 10.1213/01.ane.0000247197.78421.93
Letters to the Editor: Letters & Announcements

Department of Anesthesiology and Critical Care Medicine; The Children’s Hospital of Philadelphia and University of Pennsylvania School of Medicine; Philadelphia, Pennsylvania;

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To the Editor:

Aromatic l-amino acid decarboxylase deficiency (ALADD) is an autosomal, recessive disease, described in 12 patients (1), and associated with deficiencies in the dopamine and serotonin pathways. Clinically these patients have problems modulating psychomotor function; hormone secretion; cardiovascular, pulmonary and gastrointestinal control; sleep mechanisms; body temperature; and pain. Vutskits et al. (1) recently described the anesthetic management of a child with ALADD. We report a 4 yr 5-mo old boy with ALADD, who presented for a Nissen fundoplication and gastrostomy tube placement, whom we managed with combined general and regional anesthetic techniques.

The patient underwent a modified rapid-sequence induction, followed by placement of additional venous catheters and a radial arterial catheter. An epidural catheter was placed at the T9–10 interspaced and bolused with bupivicaine. Infusions of dopamine and epinephrine were available. In the postoperative period, we infused bupivicaine (0.1%) and fentanyl (2.5 mcg/mL) at 3 mL/h via the epidural catheter. Despite our predictions of hemodynamic instability, none occurred either intraoperatively or during the rest of his hospitalization. He was discharged on postoperative Day 3.

Two groups of patients have been described with ALADD (3), one consisting of boys, who are treatment responders, and the other consisting of boys and girls, who are nonresponders. It is possible that our patient was from the first group, as he behaved very differently under anesthesia from the young girl described by Vutskits et al. who required dopamine infusions during three of four anesthetics.

Darryl H. Berkowitz, MD

Arjunan Ganesh, MBBS

Department of Anesthesiology and Critical Care Medicine

The Children’s Hospital of Philadelphia and University of Pennsylvania School of Medicine

Philadelphia, Pennsylvania

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1. Vutskits L, Menache C, Manzano S, et al. Anesthesia management in a young child with aromatic l-amino acid decarboxylase deficiency. Pediatr Anaesth 2006;16:82–4.
2. Swoboda KJ, Saul JP, McKenna CE, et al. Aromatic l-amino acid decarboxylase deficiency: overview of clinical features and outcomes. Ann Neurol 2003;54(Suppl 6): S49–S55.
    3. Pons R, Ford B, Chiriboga CA, et al. Aromatic l-amino acid decarboxylase deficiency: clinical features, treatment, and prognosis. Neurology 2004;62:1058–65.
    © 2006 International Anesthesia Research Society