Acute soft head syndrome and orbital compression syndrome in a child with sickle cell disease: a case report.

Abstract

Background: Sickle cell disease affects 7.7 million people worldwide, mostly in sub-Saharan Africa. However, due to migration trends, patients with sickle cell disease are increasingly found in the Western world. As such, knowing rare complications of sickle cell disease, such as acute soft head syndrome and orbital compression syndrome, is important to avoid misdiagnosis and mismanagement.

Case presentation: A 9-year-old Ugandan male patient known to have sickle cell anemia presented to our pediatric emergency unit with areas of swelling of the head that progressed in a couple of hours to involve the right eye and were associated with a low-grade fever but no headache. A diagnosis of acute soft head syndrome complicated by orbital compression syndrome was made. The patient was treated conservatively with fluids, analgesia, steroids and prophylactic antibiotics. The orbital compression syndrome was complicated by a corneal ulcer; however, vision was retained in all visual fields due to the corneal ulcer's location below the pupillary axis.

Conclusion: We highlight the reversible nature of acute soft head syndrome and orbital compression syndrome in a child with sickle cell disease. We also highlight the importance of protective eye care in orbital compression syndrome to avoid exposure keratopathy. Physicians should resist the temptation to aspirate these areas of swelling, as this can introduce infection.