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

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.

Hallermann-Streiff syndrome: airway problems during anaesthesia.

In the patients with Hallermann-Streiff Syndrome, presence of mandibular hypoplasia and microstomia results in difficult intubation. The anaesthetic management of a three month old child with this syndrome for lensectomy is presented in this report. There was difficulty in maintaining the airway patency during induction which was solved with the help of a modified, improvised airway. Intubation was done after induction with a combination of inhalational and intravenous anaesthetic agents and muscle relaxant. Recognition of this syndrome should alert the physician to the possibility of difficulty in airway maintenance.