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HbSC Disease and Spontaneous Epidural Hematoma with Kernohan's Notch Phenomena.

Yogarajah M, Agu CC, Sivasambu B, Mittler MA - Case Rep Hematol (2015)

Bottom Line: A 19-year-old African American male with hemoglobin SC disease (HbSC) presented with generalized body aches and was managed for acute painful crisis.We report the first case of HbSC disease associated with catastrophic epidural hematoma progressing to transtentorial herniation and Kernohan's notch phenomena within few hours with rapid clinical deterioration.The etiopathogenesis and the rare presentation are discussed in detail in this case report.

View Article: PubMed Central - PubMed

Affiliation: Department of Medicine, Interfaith Medical Center, Brooklyn, NY 11213, USA.

ABSTRACT
Spontaneous (nontraumatic) acute epidural hematoma is a rare and poorly understood complication of sickle cell disease. A 19-year-old African American male with hemoglobin SC disease (HbSC) presented with generalized body aches and was managed for acute painful crisis. During his hospital stay he developed rapid deterioration of his mental status and computed topography revealed a spontaneous massive epidural hematoma with mass effect and midline shift with Kernohan's notch phenomena for which urgent craniotomy and evacuation was done. We report the first case of HbSC disease associated with catastrophic epidural hematoma progressing to transtentorial herniation and Kernohan's notch phenomena within few hours with rapid clinical deterioration. The etiopathogenesis and the rare presentation are discussed in detail in this case report.

No MeSH data available.


Related in: MedlinePlus

Postop MR demonstrating infarcts from herniation including the left cerebral crus infarcts (white arrow) and right parietal infarcts.
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fig5: Postop MR demonstrating infarcts from herniation including the left cerebral crus infarcts (white arrow) and right parietal infarcts.

Mentions: A “19-year-old” African American male with HbSC disease presented with generalized body aches for “4 hours' duration” which did not respond to ibuprofen which he usually uses for pain. He denied any other systemic complaints and was unable to identify any trigger factors for his painful crisis. He had multiple inpatient admissions in the past for painful crisis but did not have any history of previous stroke, acute chest syndrome, or other sickle cell disease associated complications. On examination patient was afebrile and hemodynamically stable with a blood pressure of 121/83 mmhg and pulse rate of 63/min. Systemic examination was unrevealing. Initial labs revealed white blood cell count of 10.3 k/uL, hemoglobin of 12.7 g/dL, hematocrit of 39.4%, and platelet count of 177 k/uL. The electrolytes, renal function test, and liver enzymes were normal except for mild hyperbilirubinemia of 2.2 mg/dL (0.3–1.2). His coagulation panel was normal. Patient was managed for acute vasoocclusive crisis with intravenous hydration and opioids optimized according to the pain scale. Patient continued to have generalized body aches during the hospital stay and his opioids were increased after which he was comfortable. On the second day of admission patient suddenly became diaphoretic and unresponsive. Naloxone was administered with improvement in mentation; however, in 3 hours patient again became drowsy and was only responding to deep painful stimuli. Emergent computed topography of the head revealed large 3.6 × 9.7 cm right acute epidural hematoma with mass effect on the adjacent sulci and right lateral ventricle with 1.6 cm right-to-left midline shift (Figure 1). There was no history of trauma prior to hospitalization and he did not sustain any trauma during the hospital stay. Patient underwent urgent craniotomy and evacuation of the epidural hematoma (Figure 2). There was no evidence of skull infarction and the bony margins were regular and not thickened with no periosteal elevation and a bleeding vessel was not identified. On recovery patient had mild ipsilateral hemiparesis and postoperative imaging revealed Kernohan's notch phenomena (Figures 3, 4, and 5).


HbSC Disease and Spontaneous Epidural Hematoma with Kernohan's Notch Phenomena.

Yogarajah M, Agu CC, Sivasambu B, Mittler MA - Case Rep Hematol (2015)

Postop MR demonstrating infarcts from herniation including the left cerebral crus infarcts (white arrow) and right parietal infarcts.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4631867&req=5

fig5: Postop MR demonstrating infarcts from herniation including the left cerebral crus infarcts (white arrow) and right parietal infarcts.
Mentions: A “19-year-old” African American male with HbSC disease presented with generalized body aches for “4 hours' duration” which did not respond to ibuprofen which he usually uses for pain. He denied any other systemic complaints and was unable to identify any trigger factors for his painful crisis. He had multiple inpatient admissions in the past for painful crisis but did not have any history of previous stroke, acute chest syndrome, or other sickle cell disease associated complications. On examination patient was afebrile and hemodynamically stable with a blood pressure of 121/83 mmhg and pulse rate of 63/min. Systemic examination was unrevealing. Initial labs revealed white blood cell count of 10.3 k/uL, hemoglobin of 12.7 g/dL, hematocrit of 39.4%, and platelet count of 177 k/uL. The electrolytes, renal function test, and liver enzymes were normal except for mild hyperbilirubinemia of 2.2 mg/dL (0.3–1.2). His coagulation panel was normal. Patient was managed for acute vasoocclusive crisis with intravenous hydration and opioids optimized according to the pain scale. Patient continued to have generalized body aches during the hospital stay and his opioids were increased after which he was comfortable. On the second day of admission patient suddenly became diaphoretic and unresponsive. Naloxone was administered with improvement in mentation; however, in 3 hours patient again became drowsy and was only responding to deep painful stimuli. Emergent computed topography of the head revealed large 3.6 × 9.7 cm right acute epidural hematoma with mass effect on the adjacent sulci and right lateral ventricle with 1.6 cm right-to-left midline shift (Figure 1). There was no history of trauma prior to hospitalization and he did not sustain any trauma during the hospital stay. Patient underwent urgent craniotomy and evacuation of the epidural hematoma (Figure 2). There was no evidence of skull infarction and the bony margins were regular and not thickened with no periosteal elevation and a bleeding vessel was not identified. On recovery patient had mild ipsilateral hemiparesis and postoperative imaging revealed Kernohan's notch phenomena (Figures 3, 4, and 5).

Bottom Line: A 19-year-old African American male with hemoglobin SC disease (HbSC) presented with generalized body aches and was managed for acute painful crisis.We report the first case of HbSC disease associated with catastrophic epidural hematoma progressing to transtentorial herniation and Kernohan's notch phenomena within few hours with rapid clinical deterioration.The etiopathogenesis and the rare presentation are discussed in detail in this case report.

View Article: PubMed Central - PubMed

Affiliation: Department of Medicine, Interfaith Medical Center, Brooklyn, NY 11213, USA.

ABSTRACT
Spontaneous (nontraumatic) acute epidural hematoma is a rare and poorly understood complication of sickle cell disease. A 19-year-old African American male with hemoglobin SC disease (HbSC) presented with generalized body aches and was managed for acute painful crisis. During his hospital stay he developed rapid deterioration of his mental status and computed topography revealed a spontaneous massive epidural hematoma with mass effect and midline shift with Kernohan's notch phenomena for which urgent craniotomy and evacuation was done. We report the first case of HbSC disease associated with catastrophic epidural hematoma progressing to transtentorial herniation and Kernohan's notch phenomena within few hours with rapid clinical deterioration. The etiopathogenesis and the rare presentation are discussed in detail in this case report.

No MeSH data available.


Related in: MedlinePlus