Letter to the Editor

ORBITAL APEX SYNDROME DUE T O SICKLE CELL ANEMIA

Sinasi Ozsoylu, MD, Hasan iama, MD, Ciilyiiz Ertiirk, MD, and AySegul Tokatli, MD 0 Hacettepe University Faculty of Medicine, Department of Pediatrics, Hematology Unit, and Hacettepe Children's Hospital, Ankara, Turkey

INTRODUCTION

Orbital apex syndrome is defined as a clinical entity characterized by marked restriction of mobility of the globe associated with proptosis, lid edema, reduction of vision, and pain in the orbital region and forehead.' It has been reported as a result of infection, trauma, and vascular, malignant, and inflam- matory processes of the orbital region, but only once in a patient with sickle cell anemia.' We report this unusual syndrome in an 18-year-old boy with sickle cell-homozygous-alpha thalassemia-2 combination.

CASE REPORT

This white boy, the fourth child of a marriage of second cousins, has been followed at Hacettepe Children's Hospital since the age of 15 with a diagnosis of sickle cell anemia-homozygous thalassemia-2 combination with mild anemia (Hb 9.6 g/dl; Hct 31%) and a modest reticulocytosis (7.2%). In addition to sickling, mild hypochromia was detected. Sickling tests on his parents and six siblings were also positive. Hemoglobin electrophoresis (starch gel at p H 8.6 and agar gel at p H 6.4) of his family members revealed HbS trait except in one sibling who also had sickle cell anemia.3

A growth spurt occurred with early adolescence at about 16 years of age with improvement of his anemia. His and his parents' hematological findings revealed that the father had HB S-a-thalassemia-2, the mother HbS trait with homozygous-a-thalassemia-2, and the patient homozygous for both HbS and a-thalassemia-2 (Table 1).

He was last seen because of pain in the extremities for 2 weeks and head- ache and orbital swelling and pain for a week. With the exception of spleno-

Pediatric HematoloQ and Oncology, 3:183-185, 1986 Copyright 0 1986 by Hemisphere Publishing Corporaiion

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184 LETTER TO THE EDITOR

TABLE 1 . Hematological Values of the Patient and His Parents

Hb Hct RBC MCV MCH HbA, HbF HbS Age (g/dl) (%) (10”/L) (fl) (pg) ( % ) (%) (%) cdnon-cu

Patient 17 12.5 37 5.2 7 1 24.3 3 . 7 15 81 0.75 5.6 71.5 24.3 3.3 0.7 25 0.75 Mother 46 13.6 -

Father 45 15.15 - 5.97 78 25.4 3.3 0.8 34 -

megaly (5 cm below left costal margin), all pathological findings were related to both eyes, much marked on the right. Both palpeprae had a blue-gray tinge, ptosis, and marked edema. Conjunctivae were wrinkled and edematous, but both corneas were intact. Both eyes protruded (2.7 mm, right eye; 2.5 mm, left). Eye movements were markedly decreased in all directions. Eye grounds were pale, but no hemorrhage was seen. Visual acuity was limited to finger counting on the left, and light perception on the right. With the diagnosis of orbital apex syndrome he was given corticosteroid (50 mg/day) to decrease orbital edema. A saline sponge was continuously applied to both eyes to de- crease marked conjunctival edema, and aspirin (200 mg/day) was given to decrease platelet aggregation.

Within 10 days the left eye and its movements became completely normal, with complete visual acuity. The right eye improved greatly, despite slight limi- tation in movement to the right with visual acuity of 0.4. He was sent home with aspirin (200 mg/day). Two weeks later his right eye movements became completely normal, and the visual acuity was found to be 0.8.

DISCUSSION

If transfusion requirements and hospital admissions are taken into consider- ation, sickle-cell anemia in general is a relatively mild disease in Turkey.’ How- ever, we have seen almost every complication of this disease, but not this unu- sual syndrome.

There are some genetic, cellular, and acquired conditions that, altogether, may influence the hematological and clinical diversity of sickle cell disease.’ Among these factors, G6PD deficiency was shown in the first patient with orbital apex syndrome’ but not in our patient.

Since our patient’s vasoocclusive crises increased after puberty, including a hepatic crisis and aseptic necrosis of both femur heads, with elevation of hemo- globin and hematocrit values to about normal limits, it might be suggested that mild anemia in homozygous sickle cell disease is beneficial and should not be corrected. Therefore no “hematinic” such as folic acid was given to this patient after this last complication.

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LETTER TO THE EDITOR 185

REFERENCES

1 . Walsh FB, Hoyt WF: Clinical Neurophthalmology, 3d ed, pp. 1488-2011. Baltimore: Williams and

2. Al-Rashid RA: Orbital apex syndrome secondary to sickle cell anemia. J Pediatr 95:426-427, 1977. 3. Ozsoylu 8, Gurgey A: Sickle cell anemia without anemia: A case report. In: North-Cyprus Symposium

on Abnormal Hemoglobins and Thalassemia, edited by Aksoy and Huisman, pp. 61-66. Tubitak, 1984. 4. Ozsoylu $, Altinoz N: Sickle cell anemia in Turkey. Evaluation of 97 cases (with parents’ findings).

Scand J Haematol 19:85-92, 1977. 5. Steinberg M H , Hebbel RP: Clinical diversity of sickle cell disease: Genetic and cellular modulation of

disease severity. Am J Hematol 14:405-416, 1983.

Wilkins, 1969.

Received December 16, 1985 Accepted February 7, 1986

Request reprints &om S i n a i Ozsoylu.

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