Gross Enophthalmos After Cerebrospinal Fluid Shunting for Childhood Hydrocephalus: The “Silent Brain Syndrome”

Ophthal Plast Reconstr Surg, Vol. 25, No. 6, 2009

Francesco P. Bernardini, M.D.*, Geoffrey E. Rose, M.D., Augusto A. V. Cruz, M.D., Ph.D.,and Enrico Priolo, M.D.

*Oculoplastic and Orbital Service, Istituto G. Gaslini, Genova, Italy; Orbital Service, Moorfields Eye Hospital NHS Trust, London, United Kingdom; and Orbital Service, School of Medicine of Ribeira ao Preto, University of Sao Paulo, Sao Paulo, Brazil

Nontraumatic enophthalmos is generally due to 1 of 3 mechanisms: 1) orbital fat atrophy, as in cases of hemifacial atrophy or HIV-lipodystrophy; 2) severe fibrosis of orbital fat, as with metastatic scirrhous breast carcinoma; or 3) expansion of the orbit, as in “imploding antrum” or “silent sinus” syndrome; only rarely, however, are these conditions bilateral.

Both of our patients were free of any prior or concurrent systemic disease. Severe bilateral enophthalmos after ventriculo-peritoneal (VP) shunting was first reported in 3 patients without an explanation for this condition.

Together with description of a surgical correction for the condition, Cruz et al. were the first to report the association of upward bowing of the orbital roof and prior shunting in one patient.

We now describe the clinical features in 2 further cases and propose a conjectural mechanism for this disfiguring condition that we call “silent brain syndrome.”

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