Published online June 1, 2007
PEDIATRICS Vol. 119 No. 6 June 2007, pp. 1249-1250 (doi:10.1542/10.1542/peds.2007-0198)
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LETTER TO THE EDITOR

Subarachnoid Hemorrhage in a Young Child With Sickle Cell Disease: Is Transcranial Doppler Helpful?

Christina S. Ross
Jane M. Hennessy, RN, MPH, CNP
Stephen C. Nelson, MD

Pediatric Hematology/Oncology
Children's Hospitals and Clinics of Minnesota
Minneapolis, MN 55404

To the Editor.—

We read with interest the article by Strouse et al.1 This work provided an excellent review of intracranial hemorrhage in children with sickle cell disease (SCD). The study also identified risk factors for the development of primary hemorrhagic stroke that included hypertension, red blood cell transfusion, and corticosteroid therapy. We would like to report a case of atraumatic subarachnoid hemorrhage (SAH) with intriguing transcranial Doppler (TCD) results in a child with SCD that may add to the risk profile described in the Strouse et al article.

An 8-year-old girl with hemoglobin SS disease was admitted to Children's Hospitals and Clinics of Minnesota for management of a vasoocclusive painful event. On the second hospital day, she developed acute chest syndrome. She received 2 red blood cell transfusions as part of her management. She did not receive corticosteroid therapy. By hospital day 8 she was prepared for discharge but developed headache, hypertension, and a generalized seizure. A computed tomography scan, MRI, and lumbar puncture confirmed the presence of a left frontal SAH. Interestingly, the patient had 2 TCD examinations performed during her hospitalization, one on the day of her seizure and one 6 days earlier.

The patient's baseline hemoglobin level was 7.4 g/dL and decreased to 6.3 g/dL on hospital day 2. After red blood cell transfusion, her hemoglobin level increased by 65% to 10.4 g/dL. Steady-state blood pressures were normal at 117/61 mmHg. On the day of her SAH she had 2 episodes of hypertension with readings of 140/91 and 137/97 mmHg. A mean decrease in velocity of 91 cm/second was seen on the TCD that was performed 6 hours before the patient's seizure when compared with the study performed 6 days earlier. Results are shown in Table 1.


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TABLE 1 TCD Results

 
Isolated SAH is quite rare in children with SCD. Our patient represents only the seventh reported case in a child under the age of 9 years.15 She had exhibited 2 of the reported risk factors for primary hemorrhagic stroke: red blood cell transfusion and hypertension. Results of a magnetic resonance angiogram and conventional cerebral angiography were normal. She recovered fully and continues to do well without neurologic sequelae. Of particular interest in this case is the TCD examination on the afternoon of the hemorrhage. Low velocities or rapid changes in velocities on TCD may be useful in identifying vasospasm associated with SAH in adults.6,7 Perhaps vasospasm played a role in our patient's hemorrhage. Of note, her TCD velocities on hospital day 2 may have been affected by her acute painful event, lower hemoglobin level, and narcotic use.

The role of TCD in the setting of pediatric SAH is unclear. With additional study, TCD findings could potentially be included in the risk assessment for primary hemorrhagic stroke in children with SCD.

REFERENCES

  1. Strouse JJ, Hulbert ML, DeBaun MR, Jordan LC, Casella JF. Primary hemorrhagic stroke in children with sickle cell disease is associated with recent transfusion and use of corticosteroids. Pediatrics. 2006;118 :1916 –1924[Abstract/Free Full Text]
  2. Cook W. A case of sickle cell anemia with associated subarachnoid hemorrhage. J Med. 1930;11 :541 –542
  3. Bridgers W. Cerebral vascular disease accompanying sickle cell anemia. Am J Pathol. 1939;15 :353 –361[Web of Science]
  4. Greer M, Schotland D. Abnormal hemoglobin as a cause of neurologic disease. Neurology. 1962;12 :114 –123[Free Full Text]
  5. Van Hoff J, Ritchey AK, Shaywitz BA. Intracranial hemorrhage in children with sickle cell disease. Am J Dis Child. 1985;139 :1120 –1123[Abstract/Free Full Text]
  6. Lysakowski C, Wlader B, Constanza MC, et al. Transcranial Doppler versus angiography in patients with vasospasm due to ruptured cerebral aneurysm: a systematic review. Stroke. 2001;32 :2292 –2298[Abstract/Free Full Text]
  7. Grosset DG, Straiton J, McDonald I, Cockburn M, Bullock R. Use of transcranial Doppler sonography to predict development of a delayed ischemic deficit after subarachnoid hemorrhage. J Neurosurg. 1993;78 :183 –187[Web of Science][Medline]

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

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