Korean J Ophthalmol.  2011 Apr;25(2):142-145. 10.3341/kjo.2011.25.2.142.

Surgical Correction of Hallermann-Streiff Syndrome: A Case Report of Esotropia, Entropion, and Blepharoptosis

• Cho WK
• Park JW
• Park MR

• KMID: 1111879
• DOI: http://doi.org/10.3341/kjo.2011.25.2.142

Abstract

We report a case of surgical treatment for Hallermann-Streiff syndrome in a patient with ocular manifestations of esotropia, entropion, and blepharoptosis. A 54-year-old man visited Yeouido St. Mary's Hospital complaining of ocular discomfort due to cilia touching the corneas of both eyes for several years. He had a bird-like face, pinched nose, hypotrichosis of the scalp, mandibular hypoplasia with forward displacement of the temporomandibular joints, a small mouth, and proportional short stature. His ophthalmic features included sparse eyelashes and eyebrows, microphthalmia, nystagmus, lower lid entropion in the right eye, and upper lid entropion with blepharoptosis in both eyes. There was esodeviation of the eyeball of more than 100 prism diopters at near and distance, and there were limitations in ocular movement on lateral gaze. The capsulopalpebral fascia was repaired to treat the right lower lid entropion, but an additional Quickert suture was required to prevent recurrence. Blepharoplasty and levator palpebrae repair were performed for blepharoptosis and dermatochalasis. Three months after lid surgery, the right medial rectus muscle was recessed 7.5 mm, the left medial rectus was recessed 7.25 mm, and the left lateral rectus muscle was resected 8.0 mm.

Keyword

Entropion; Esotropia; Hallermann's syndrome; Ptosis; Surgical correction

MeSH Terms

Blepharoptosis/physiopathology/*surgery
Entropion/physiopathology/*surgery
Esotropia/physiopathology/*surgery
Eye Movements
Follow-Up Studies
Hallermann's Syndrome/*surgery
Humans
Male
Middle Aged
Oculomotor Muscles/physiopathology/*surgery
Ophthalmologic Surgical Procedures/*methods

Figure

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  Fig. 1 Initial preoperative photography shows bird-like facies with a pinched nose, hypotrichosis of the hair and eyebrows, mandibular hypoplasia with a small mouth, and abnormal dentition. These signs were all indicative of Hallermann-Streiff syndrome.

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  Fig. 2 Preoperative 9-cardinal photograph shows esotropia of more than 100 prism diopters. Ocular movement was limited in lateral gaze.

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  Fig. 3 (A) Preoperative photography shows both upper and right lower eyelid entropion. An overactive frontalis muscle compensates for both blepharoptosis and dermatochalasis. (B) Photography after surgical eyelid correction. Frontalis muscle overactivity is relieved. (C) Photograph obtained on the 7th day after correction of esotropia. Slight esodeviation remains, and subconjunctival hemorrhage is present.

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