Hutchinson syndrome (adrenal cortex neuroblastoma with orbital metastasis; pepper syndrome)

General: Metastatic infraorbital neuroblastoma after hemato 313d38d genous dissemination of primary tumor; occurs in infants and children up to age 6 years; poor prognosis; in children neuroblastoma commonly involves the orbit; 15% of patients with neuroblastoma had proptosis and ecchymosis.

Ocular: Exophthalmos; lid hematoma; extraocular muscle palsy; subconjunctival hemorrhages; choroidal metastatic tumor; papilledema; optic atrophy.

Clinical: Severe anemia; increased sedimentation rate; urinary excretion of 3-methoxy-4-hydroxy mandelic acid.

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Hutchinson R. Suprarenal sarcoma in children with metastasis in the skull. Q J Med 1907-l908; 1:33.

Musarella MA, et al. Ocular involvement in neuroblastoma: prognostic implications. Ophthalmology 1984; 91:936.

Volpe NJ, Albert DM. Metastases to the uvea. In: Albert DM, Jakobiec FA, eds. Principles and Practice of Ophthalmology. vol. V. Philadelphia: WB Saunders, 1994:3260.