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Spontaneous extradural hemorrhage due to Langerhans cell histiocytosis of the skull in a child: A rare presentation.

Bakhaidar MG, Alghamdi FA, Baeesa SS - J Pediatr Neurosci (2016 Jan-Mar)

Bottom Line: Three weeks prior his scheduled surgery, he presented to the emergency department with a 2-day history of sudden increased of the subcutaneous swelling associated with a headache, vomiting, and decreased the level of consciousness; there was no history of trauma.Histopathological examination confirmed the diagnosis of EG.We aim to raise the awareness of physicians of this rare spontaneous hemorrhagic complication of EG and review the literature.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurosurgery, Faculty of Medicine, King Abdulaziz University, Jeddah, Saudi Arabia.

ABSTRACT
Eosinophilic granuloma (EG) represents a local form of Langerhans cell histiocytosis that occurs mostly in children. It usually presents with a gradually enlarging painless skull mass, and rarely presents a rapid clinical deterioration. This 7-year-old boy who was diagnosed with EG, based on a magnetic resonance imaging scan, after presenting with a painless right parietal swelling of 7-week duration. Three weeks prior his scheduled surgery, he presented to the emergency department with a 2-day history of sudden increased of the subcutaneous swelling associated with a headache, vomiting, and decreased the level of consciousness; there was no history of trauma. Brain computed tomography revealed a right parietal bone defect with large subgaleal and extradural hematoma. He underwent emergent surgical excision of the skull lesion and evacuation of the hematoma. Histopathological examination confirmed the diagnosis of EG. We aim to raise the awareness of physicians of this rare spontaneous hemorrhagic complication of EG and review the literature.

No MeSH data available.


Related in: MedlinePlus

Photomicrograph showing polymorphic infiltrate of Langerhans histiocytes (polygonal cells with kidney-shaped and elongated nuclei had longitudinal groove) admixed with many eosinophils
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Figure 3: Photomicrograph showing polymorphic infiltrate of Langerhans histiocytes (polygonal cells with kidney-shaped and elongated nuclei had longitudinal groove) admixed with many eosinophils

Mentions: Histopathology examination revealed polymorphic infiltrate composed predominantly of histiocytes and plenty of eosinophils. Hemosiderin-laden macrophages and multinucleated giant cells with areas of necrosis were also noted. The neoplastic histiocytes showed kidney-shaped and elongated nuclei with a longitudinal groove (coffee beans like), consistent with Langerhans histiocytes [Figure 3]. Immunohistochemistry revealed strong staining to S100, CD1a and CD68 in those Langerhans histiocytes confirming the diagnosis of Langerhans histiocytosis. There was no organisms’ growth on culture of the fluids obtained during surgery from the cyst or extradural space.


Spontaneous extradural hemorrhage due to Langerhans cell histiocytosis of the skull in a child: A rare presentation.

Bakhaidar MG, Alghamdi FA, Baeesa SS - J Pediatr Neurosci (2016 Jan-Mar)

Photomicrograph showing polymorphic infiltrate of Langerhans histiocytes (polygonal cells with kidney-shaped and elongated nuclei had longitudinal groove) admixed with many eosinophils
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4862289&req=5

Figure 3: Photomicrograph showing polymorphic infiltrate of Langerhans histiocytes (polygonal cells with kidney-shaped and elongated nuclei had longitudinal groove) admixed with many eosinophils
Mentions: Histopathology examination revealed polymorphic infiltrate composed predominantly of histiocytes and plenty of eosinophils. Hemosiderin-laden macrophages and multinucleated giant cells with areas of necrosis were also noted. The neoplastic histiocytes showed kidney-shaped and elongated nuclei with a longitudinal groove (coffee beans like), consistent with Langerhans histiocytes [Figure 3]. Immunohistochemistry revealed strong staining to S100, CD1a and CD68 in those Langerhans histiocytes confirming the diagnosis of Langerhans histiocytosis. There was no organisms’ growth on culture of the fluids obtained during surgery from the cyst or extradural space.

Bottom Line: Three weeks prior his scheduled surgery, he presented to the emergency department with a 2-day history of sudden increased of the subcutaneous swelling associated with a headache, vomiting, and decreased the level of consciousness; there was no history of trauma.Histopathological examination confirmed the diagnosis of EG.We aim to raise the awareness of physicians of this rare spontaneous hemorrhagic complication of EG and review the literature.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurosurgery, Faculty of Medicine, King Abdulaziz University, Jeddah, Saudi Arabia.

ABSTRACT
Eosinophilic granuloma (EG) represents a local form of Langerhans cell histiocytosis that occurs mostly in children. It usually presents with a gradually enlarging painless skull mass, and rarely presents a rapid clinical deterioration. This 7-year-old boy who was diagnosed with EG, based on a magnetic resonance imaging scan, after presenting with a painless right parietal swelling of 7-week duration. Three weeks prior his scheduled surgery, he presented to the emergency department with a 2-day history of sudden increased of the subcutaneous swelling associated with a headache, vomiting, and decreased the level of consciousness; there was no history of trauma. Brain computed tomography revealed a right parietal bone defect with large subgaleal and extradural hematoma. He underwent emergent surgical excision of the skull lesion and evacuation of the hematoma. Histopathological examination confirmed the diagnosis of EG. We aim to raise the awareness of physicians of this rare spontaneous hemorrhagic complication of EG and review the literature.

No MeSH data available.


Related in: MedlinePlus