PGY-2 Mount Sinai New York, New York, United States
Case Diagnosis: Sickle Cell Disease
Case Description: 28 y.o. African American male with a pmhx of sickle cell disease, htn, and avascular necrosis of the right hip presented with ongoing pain located in the back across his waist for one month that began after an episode of sickle cell crisis that was more prolonged than usual. Noted changes in position were painful. CT L-spine w/contrast showed unchanged H-shaped wedging endplate deformities at every level from T11 through L5 with mild loss of height and an area of possible avascular necrosis/deformity in the L1 vertebrae. On physical exam, tenderness was noted over the upper lumbar segments and at the base of the lumbar spine. Stooped posture was noted when patient was standing and was ambulating with straight cane. Pain has improved from a 5/10 to 2/10 with hydromorphone and methadone. Lumbosacral corset was provided for additional pain control.
Discussions: The “Fish Vertebrae Sign” typically presents as a biconcave deformity of the vertebrae which is likely due to ischemia of the central part of the vertebral growth plate. The most common pathological mechanism involves chronic sickling of rbcs leading to recurrent vassocclusive crises. There is a high prevalence of bone pathologies in patients with sickle cell disease with most concern for osteoporosis in the lumbar spine. 64% of adults with SCD had a lower bone mineral density compared with age, race, and sex-matched controls . In addition, low bone mineral density is associated with lower bmi, lower plasma zinc levels, and higher markers of bone turnover. Although there are significant correlations in assessing these patients, there is minimal longitudinal data of measures to prevent future fractures in high-risk patients.
Conclusions: One of the biggest takeaways is the need for further longitudinal research on effective treatment modalities to reduce the incidence of fractures in patients with sickle cell disease.
