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.
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

PURPOSE: To evaluate the clinical effects of conjunctiva-Muller muscle resection through conjunctival incision in anophthalmic patients with mild ptosis. METHODS: Conjunctiva-Muller muscle resection was performed by one surgeon in 8 patients (8 eyes) who had received evisceration or enucleation and responded to 10% phenylephrine solution to correct ptosis. The average age of the patients was 35.87+/-13.4 years. Ptosis was seen from 1 to 34 months after evisceration or enucleation. The preoperative MRD 1 was -2 to 0.5 mm (average: -0.25+/-1.10 mm) and the difference of MRD 1 between before and after 10% phenylephrine use was 2.56+/-0.98 mm. The Muller muscle was resected 7.5 to 9 mm through conjunctival incision during surgery to match the MRD 1 of sound eye. Mean follow-up period after the operation was 2 to 16 months (average: 8.1 months). RESULTS: Postoperatively, the MRD 1 increased by 1.81+/-0.88 mm on the average, corresponding to the improvement in lid elevation after the use of 10% phenylephrine performed before resection. Surgery was successful in most patients, and postoperative difference in MRD 1 was less than 1 mm from the sound eye. No special postoperative complication was observed. CONCLUSIONS: Conjunctiva-Muller muscle resection is one of the effective methods of correcting mild ptosis in anophthalmic patients.
Adult ; Anophthalmos/*complications ; Blepharoptosis/etiology/*surgery ; Conjunctiva/*surgery ; Eyelids/physiopathology ; Facial Muscles/*surgery ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Muscle Contraction ; Ophthalmologic Surgical Procedures/*methods ; Time Factors ; Treatment Outcome

PURPOSE: To evaluate the clinical effects of conjunctiva-Muller muscle resection through conjunctival incision in anophthalmic patients with mild ptosis. METHODS: Conjunctiva-Muller muscle resection was performed by one surgeon in 8 patients (8 eyes) who had received evisceration or enucleation and responded to 10% phenylephrine solution to correct ptosis. The average age of the patients was 35.87+/-13.4 years. Ptosis was seen from 1 to 34 months after evisceration or enucleation. The preoperative MRD 1 was -2 to 0.5 mm (average: -0.25+/-1.10 mm) and the difference of MRD 1 between before and after 10% phenylephrine use was 2.56+/-0.98 mm. The Muller muscle was resected 7.5 to 9 mm through conjunctival incision during surgery to match the MRD 1 of sound eye. Mean follow-up period after the operation was 2 to 16 months (average: 8.1 months). RESULTS: Postoperatively, the MRD 1 increased by 1.81+/-0.88 mm on the average, corresponding to the improvement in lid elevation after the use of 10% phenylephrine performed before resection. Surgery was successful in most patients, and postoperative difference in MRD 1 was less than 1 mm from the sound eye. No special postoperative complication was observed. CONCLUSIONS: Conjunctiva-Muller muscle resection is one of the effective methods of correcting mild ptosis in anophthalmic patients.
Adult ; Anophthalmos/*complications ; Blepharoptosis/etiology/*surgery ; Conjunctiva/*surgery ; Eyelids/physiopathology ; Facial Muscles/*surgery ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Muscle Contraction ; Ophthalmologic Surgical Procedures/*methods ; Time Factors ; Treatment Outcome