Proteus syndrome revealing itself after the treatment of a bilateral subdural haematoma.

[proteus syndrome]

Hypertrophy of the calvarium has different aetiologies , among them the rare Proteus syndrome .We report here the case of a young girl initially treated for relapsing right then left large chronic subdural haematoma , who progressively developed craniofacial hypertrophy consistent with the diagnosis of Proteus syndrome . Calvarium hypertrophy was shaved and remodelled combining midface advancement , essentially for cosmetic purposes . During the first calvarium remodelling , important bleeding of the bone required large volume of blood replacement . Haemostasis workup revealed platelets aggregation anomalies . Bleeding issues during subsequent surgeries were controlled with tranexamic acid and desmopressin acetate .Other manifestations of Proteus syndrome , such as a right hypertrophy of the face with hypoplasia of its middle third , a pigmented epidermal nevus and asymmetric limbs and scoliosis , appeared progressively over time . Blood and fibroblast phosphatase and tensin homolog mutation was not found .Literature review of operated patients with Proteus syndrome did not reveal an association with platelets anomalies . A complete haemostasis workup following this unexpected haemorrhagic complication is recommended for this rare pathology .

Diseases
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Diseases presenting "a pigmented epidermal nevus and asymmetric limbs and scoliosis" symptom

proteus syndrome

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