Proptosis, skull infarction and epidural haematoma in sickle thalassemia

Jose, Tony; Subramanya, G.; Kallur, Kumar; Chalapathy, A. V.; Sheshadri, Shubha; Lakhkar, Bhushan N.

doi:10.1136/pgmj.71.837.445

Cited by 14 publications

(4 citation statements)

References 2 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Three pathophysiologic explanations have been proposed over the years for the causation of this rare entity: (1) Vaso-occlusion of the haematopoietically active calvarial diploic bone resulting in bone infarction and subsequent leaking of blood and proteinaceous material in the subperiosteal, epidural, or subgaleal space [38–41, 43, 45]. (2) Acute rapid expansion of hematopoiesis with resultant microfracture of already thinned inner cortex and extravasation of blood and hematopoietic tissue [24, 28, 36].…”

Section: Discussionmentioning

confidence: 99%

Spontaneous Epidural Hemorrhage in Sickle Cell Disease, Are They All the Same? A Case Report and Comprehensive Review of the Literature

Saha

2019

Case Reports in Hematology

View full text Add to dashboard Cite

Trauma to the skull causing injury to the middle meningeal artery, middle meningeal vein, or dural venous sinuses is responsible for most cases of epidural hemorrhage (EDH). Spontaneous EDH is a rare entity in clinical practice. Common causes include sinusitis, coagulation abnormalities, dural metastasis, and Langerhans cell histiocytosis. Isolated nontraumatic EDH is an exceedingly rare complication of sickle cell disease (SCD). We report a case of spontaneous EDH in a patient with SCD and review the world literature regarding this rare entity. A 20-year-old African American female with sickle cell disease presented with vaso-occlusive crisis. About 24 hours after hospital admission, the patient had sudden deterioration of her mental status. An emergent CT scan of the head revealed a large right-sided frontoparietal epidural hematoma with midline shift, subfalcine, and uncal herniation. The patient underwent emergent hematoma evacuation but died 24 hours after surgery.

show abstract

Section: Discussionmentioning

confidence: 99%

Spontaneous Epidural Hemorrhage in Sickle Cell Disease, Are They All the Same? A Case Report and Comprehensive Review of the Literature

Saha

2019

Case Reports in Hematology

View full text Add to dashboard Cite

show abstract

“…In the other reported cases, the EDH was small enough to be managed conservatively [11,17,19,21,27,40]. There are very few cases in the literature where sickle-cell disease has caused other neurosurgical complications [1,23].…”

Section: Discussionmentioning

confidence: 99%

A review of spontaneous intracranial extradural hematoma in sickle-cell disease

et al. 2015

View full text Add to dashboard Cite

Sickle-cell disease is common among patients of Afro-Caribbean origin. Though it can precipitate neurological conditions, it only rarely causes neurosurgical problems, with very few reported cases. We describe the case of a 7-year-old girl with a background of sickle-cell disease (SCD) brought into an acute neurosurgical unit in extremis, signs of a raised ICP, and with no history of recent trauma. Following further investigations, an acute drop in the hemoglobin and hematocrit levels were noted, with the cause of her presentation being attributed to a sickling crisis causing skull convexity infarction and resulting in spontaneous bilateral extradural hematomas requiring emergency evacuation. We review the current literature and propose the pathophysiological mechanism behind this phenomenon.

show abstract

“…There are a small number of case reports of spontaneous EDHs in the medical literature, approximately 15 cases per review. [1][2][3][5][6][7][8][9][10] The exact incidence is unknown. Even fewer cases are reported with associated calvarial infarction 1,6-8,10 on CT or MRI as demonstrated in this case.…”

Section: Discussionmentioning

confidence: 99%

Spontaneous epidural hematoma secondary to bone infarction in sickle cell anemia: case report

Komarla¹,

Soares

Chern

et al. 2018

Journal of Neurosurgery: Pediatrics

View full text Add to dashboard Cite

Spontaneous epidural hematoma (EDH) is a rare occurrence in patients with sickle cell disease, with a small number of cases reported. Appropriate diagnosis is critical, because rapid neurosurgical intervention may be required. This unique case illustrates clinical and MRI features of an 18-year-old woman presenting with a headache and subsequent progression to severe focal neurological symptoms. Imaging demonstrated a large EDH of mixed signal characteristics and underlying calvarial infarction, requiring emergency decompression and evacuation. A second companion case is also presented. The authors discuss proposed pathophysiology of the formation of EDHs in sickle cell anemia.

show abstract

Proptosis, skull infarction and epidural haematoma in sickle thalassemia

Cited by 14 publications

References 2 publications

Spontaneous Epidural Hemorrhage in Sickle Cell Disease, Are They All the Same? A Case Report and Comprehensive Review of the Literature

Spontaneous Epidural Hemorrhage in Sickle Cell Disease, Are They All the Same? A Case Report and Comprehensive Review of the Literature

A review of spontaneous intracranial extradural hematoma in sickle-cell disease

Spontaneous epidural hematoma secondary to bone infarction in sickle cell anemia: case report

Contact Info

Product

Resources

About