Application of lateral tarsoconjunctival flap to correct ocular complications of Madelung's disease: A case report and literature review

Abstract

Rationale: Madelung's disease is a specific type of benign symmetric lipomatosis, which is characterized by overgrowing fat distributed at the neck and shoulder. This excessive fat accumulation may occur in different regions, including vital organs, such as the larynx, trachea, or the orbits. Surgery is usually performed to correct the esthetic or functional concern of the affected area. There are only few case reports mentioned about the orbital involvement of this disease. This study aimed to describe a case of Madelung's disease with ocular complications and the successful treatment using tarsoconjunctival flap.

Patient concerns: A 90-year-old obese male presented with decreased visual acuity accompanied with severe band form punctate epithelial erosions and ciliary injections in both eyes. Exophthalmos and ectropion of the lower eyelids were observed.

Diagnoses: Orbital computed tomography revealed a rare entity of Madelung's disease with significant massive deposits of lipomas located symmetrically around the orbit and neck region.

Interventions: Bilateral lateral tarsoconjunctival flaps with lateral tarsal strip procedures were performed to correct the lower eyelid ectropion-related exposure keratopathy.

Outcomes: The patient obtained improved visual acuity, intact and clear corneal surfaces, and perfect lower eyelid positions over both eyes.

Lessons: Madelung's disease is a rare condition. There are few reports to describe its ocular complications, due to lipoma overgrowing in the orbit. We shared our successful experience to correct ocular complications using tarsoconjunctival flap technique.

Figure 1

A 90-year-old man presented with progressive redness of both eyes with tearing and blurry vision for several years. Exophthalmos of both eyes with large lid bagging and moderate mid-face drop were eminent upon observation (A). Ectropion throughout the lower lids with inner tarsal conjunctiva show and severe band form punctate epithelial erosions over the lower third of the cornea in both eyes were noted (B and C).

Figure 2

Orbital CT revealed significant massive deposits of lipomas located symmetrically around the orbit and neck region (A–D). The x-ray scan also showed buffy upper trunk figure (A). Coronal view (B) showed obvious subcutaneous fat and macroglosia. Sagittal view (C) showed prominent intra and extra-orbital fat causing the large bagging. Transverse view (D) also revealed significant intraconal fat causing a grade 3 proptosis and stretching of the optic nerve. The orange line is the interzygomatic line.

Figure 3

After bilateral lateral tarsoconjunctival flaps with tarsal strip procedures, the lower eyelid ectropion-related exposure keratopathy was greatly improved (A and B). The patient was satisfactory with improved vision, intact corneal surface, and perfect lower lid positions (C and D).