To the Editor:
Hemoglobin Cheverly is rare inheritable variant of unstable hemoglobin resulting from a mutation that determines abnormal structure of one of the globin chains, modifying the amino-acid β chain sequence (1 ). We report a case of a boy with hemoglobin Cheverly, in whom the pulse oximeter showed false low measurements.
A 3-yr-old, 17-kg, boy with an unremarkable medical history, presented for repair of a right inguinal hernia. After induction of general anesthesia via facemask, an oxygen saturation (Spo2 ) value of 85% was noted. Adequate mask ventilation with Fio2 = 1 and change of probe site did not change the result (despite a good plethysmographic waveform). Arterial blood gas showed normal oxygenation (arterial oxygen saturation = 100%). The surgical procedure was performed uneventfully. Postoperative Spo2 values ranged 82%–89%.
Further investigation into the family tree revealed a 5-yr-old brother with a room air Spo2 value of 76%, a 27-yr-old father with room air Spo2 of 61%–62% and a paternal grandfather with room air Spo2 of 80%–82%. Results of hemoglobin electrophoresis showed an abnormal hemoglobin variant, known as hemoglobin Cheverly (Table 1 ).
Table 1: Hemoglobin Values
In summary, a pulse oximeter is not an accurate monitor for patients with unstable hemoglobin Cheverly and possibly other hemoglobinopathies (2,3 ).
Uriel A. Yodfat, MD
Riverside Anesthesia Associates
Harrisburg, PA
Sonia J. Vaida, MD
Visiting International Associate Professor
Department of Anesthesiology
Penn State Milton S. Hershey Medical Center
Hershey, PA
[email protected]
[email protected]
REFERENCES
1. Beutler E. Hemoglobinopathies associated with unstable hemoglobin. In: Beutler E, Lichtman MA, Coller BS, Kipps TJ, eds. Williams hematology, 6th ed. New York: McGraw-Hill, 1995;607–9.
2. Katoh R, Miyake T, Arai T. Unexpectedly low pulse oximeter readings in a boy with unstable hemoglobin. Anesthesiology 1994;80:472–4.
3. Kuji A, Satoh Y, Kikuchi K, et al. The anesthetic management of a patient with hemoglobin M (Iwate). Anesth Analg 2001;93:1192–3.