Intraocular Langerhans cell histiocytosis in a neonate resulting in bilateral loss of vision.
Intraocular involvement in Langerhans cell histiocytosis (LCH) is rare. We describe the case of a neonate with congenital disseminated LCH involving skin, liver, spleen, and intraocular structures including uvea and retina. Early and aggressive treatment according to the LCH-II treatment protocol was administered and resulted in remission of the disease. However, despite close follow-up and additional local treatment, involvement of intraocular structures resulted in severe long-term ophthalmological sequelae including complete bilateral loss of vision.
Boztug
Kaan
K
Department of Oncology, Great Ormond Street Hospital for Children NHS Trust, and Department of Ophthalmology, University College Hospital NHS Trust, London, UK.
Frimpong-Ansah
Kwabena
K
Nanduri
Vasanta Rao
VR
Lawson
Joanna
J
Russell-Eggitt
Isabelle
I
Brock
Penelope
P
D001766:Blindness / Q000209:etiology* / Q000473:pathology / Q000628:therapy; D018450:Disease Progression; D005128:Eye Diseases / Q000151:congenital* / Q000473:pathology / Q000628:therapy; D005260:Female; D005500:Follow-Up Studies; D006646:Histiocytosis, Langerhans-Cell / Q000151:congenital* / Q000473:pathology / Q000628:therapy; D006801:Humans; D007231:Infant, Newborn; D007232:Infant, Newborn, Diseases; D012074:Remission Induction; D016896:Treatment Outcome