Surgical Correction of Hallermann-Streiff Syndrome: A Case Report of Esotropia, Entropion, and Blepharoptosis
0
조회
0
다운로드
2
인용
0
참고문헌
키워드
초록
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.
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.
참고문헌 (17)
- David LR, "Hallermann-Streiff syndrome:experience with 15 patients and review of the literature", J Craniofac Surg, 10, pp.160~168 (1999)
- Francois J, "A new syndrome:dyscephalia with bird face and dental anomalies,nanism,hypotrichosis,cutaneous atrophy,microphthalmia,and congenital cataract", AMA Arch Ophthalmol, 60, pp.842~862 (1958)
- Mirshekari A, "Hallermann-Streiff syndrome:a case review", Clin Exp Dermatol, 29, pp.477~479 (2004)
- Warkany J, "Congenital malformations: notes and comments", pp.390~ (1971), Year Book Medical Publications
- Nicholson AD, "Hallerman-Streiff syndrome", J Postgrad Med, 41, pp.22~23 (1995)
- Wiedemann HR, "Hallermann-Streiff syndrome. In: Atlas of clinical syndromes: a visual aid to diagnosis for clinicians and practicing physicians", pp.530~531 (1992), Mosby Year Book
- François J, "The François dyscephalic syndrome and skin manifestations", Am J Ophthalmol, 71, pp.1241~1250 (1971)
- Morice-Picard F, "Hallerman-Streiff-like syndrome presenting with laterality and cardiac defects", Clin Dysmorphol, 18, pp.116~119 (2009)
- 박홍재, "할러만-스트라이프 증후군 1예", 대한안과학회지, 48(9), pp.1285~1290 (2007), 대한안과학회
- Ertekin V, "Non-lethal Hallermann-Streiff syndrome with bone fracture:report of a case", Ann Genet, 47, pp.387~391 (2004)
- Sharp EC, "Craniofacial and oral manifestations in oculomandibulodyscephaly(Hallermann-Streiff syndrome)", Oral Surg Oral Med Oral Pathol, 30, pp.488~496 (1970)
- Koo BS, "A case of Hallermann-Streiff syndrome", J Korean Ophthalmol Soc, 8, pp.55~60 (1967)
- Shim WS, "A case of Hallermann-Streiff syndrome", J Korean Ophthalmol Soc, 17, pp.297~301 (1976)
- Lee HB, "A case of Hallermann Streiff syndrome", J Korean Ophthalmol Soc, 19, pp.455~459 (1978)
- Ryoo MH, "A case of Hellermann-Streiff syndrome", J Korean Ophthalmol Soc, 31, pp.831~836 (1990)
- Son JS, "A case of Hallermann-Streiff syndrome", J Korean Pediatr Soc, 30, pp.691~694 (1987)
- 김승연, "자궁내 발육지연을 동반한 Hallermann Streiff 증후군 1례", Korean Journal of Pediatrics, 46(9), pp.926~929 (2003), 대한소아과학회