Published online July 2, 2007
PEDIATRICS Vol. 120 No. 1 July 2007, pp. 235-236 (doi:10.1542/peds.2007-0823)

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LETTER TO THE EDITOR

Increased Cerebral Blood Flow Velocity in Children With Sickle Cell Disease: Adenotonsillectomy or Transfusion Regimens?

Brigitte Bader-Meunier, MD
Martine François, MD
Suzanne Verlhac, MD
Monique Elmaleh, MD
Ghislaine Ithier, MD
Malika Benkerrou, MD
Assistance Publique-Hôpitaux de Paris
Hôpital Robert Debré
Paris 75 019, France

To the Editor.—

We read with great interest the report from Hill et al,¹ who showed evidence of a significant increase of cerebral blood flow velocity (CBFV) in children recruited from adenotonsillectomy waiting lists because of a history of snoring compared with controls. These features had major implications in the management of children with sickle cell disease (SCD) by underlining a relationship between abnormal cerebral high velocities and upper airway obstruction. Since the Stroke Prevention in Sickle Cell Anemia (STOP) trial, it has been recommended that children aged 2 to 16 years with SCD be screened by transcranial Doppler (TCD) to identify those at high risk for stroke who present with abnormal high velocities.² These children at high risk are then offered transfusion regimens for stroke prevention.

We report here 2 boys with SCD, aged 6 and 5 years, whose abnormal, high velocities of 200 cm/second (middle cerebral artery) returned to normal values of <170 cm/second after they had undergone adenoidectomy and/or tonsillectomy. Both patients had a history of snoring with adenotonsillar hypertrophy. Their hemoglobin values were 8 and 8.5 g/dL, respectively, and their cerebral MRI and magnetic resonance angiography findings were normal. Adenotonsillectomy was performed after 1 exchange transfusion and adenoidectomy after 1 simple transfusion; none of the patients experienced postoperative complications. No additional transfusions were provided. TCD was repeated 2 and 28 months after surgery in one of the patients and 3 months afterward in the other one; normal CBFV was found for both children.

High velocities are related to cerebral artery stenosis and severe anemia as well as tissue hypoxia. Adenotonsillar hypertrophy results in obstructive sleep apnea syndrome and nocturnal hypoxia, which is cured in 75% to 100% of children by adenotonsillectomy.³ Thus, upper airway obstruction may lead to a reversible increase of CBFV and must be carefully ruled out in children with SCD who have abnormal TCD findings before including them in transfusion regimens.

REFERENCES

1. Hill CM, Hogan AM, Onugha N, et al. Increased cerebral blood flow velocity in children with mild sleep-disordered breathing: a possible association with abnormal neuropsychological function. Pediatrics. 2006;118(4) . Available at: www.pediatrics.org/cgi/content/full/118/4/e1100
2. Adams RJ, McKie VC, Hsu L, et al. Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography. N Engl J Med. 1998;339 :5 –11[Abstract/Free Full Text]
3. Schechter MS; American Academy of Pediatrics, Section on Pediatric Pulmonology, Subcommittee on Obstructive Sleep Apnea Syndrome. Technical report: diagnosis and management of childhood obstructive sleep apnea syndrome. Pediatrics. 2002;109(4) . Available at: www.pediatrics.org/cgi/content/full/109/4/e69

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PEDIATRICS (ISSN 1098-4275). ©2007 by the American Academy of Pediatrics

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