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ISSN 2992-8036 (Electronic)
ISSN 2306-4102 (Print)
Órgano Oficial del Colegio Mexicano de Ortopedia y Traumatología

2007, Number 6

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Acta Ortop Mex 2007; 21 (6)

Freeman-Sheldon´s syndrome. Report of five cases

Vergara FHJ, Román CL

Language: Spanish
References: 13
Page: 338-343
PDF size: 201.15 Kb.


ABSTRACT

Introduction: Freeman-Sheldon syndrome, described in 1938, is an infrequent form of arthrogryposis. Material and methods: We reviewed the files of the patients with the diagnosis of Freeman-Sheldon syndrome from 1990 to 2003, and carried out an evaluation of the clinical data, family history and malformations of extremities. Results: 5 patients were included, 4 male and 1 female. Upper extremities: All patients presented adducted thumbs, 3 patients were treated with a first web release and dorsal graft with improvement of grip on larger objects. Feet: Four patients had severe bilateral rigid clubfeet, they were operated on with a posteromedial release and bilateral talectomy, satisfactory results were obtained in 4 of 6 feet according to Niki criteria. Other non orthopedic anomalies: In three patients unilateral right undescended testis; 1 right inguinal hernia. Conclusions: The patients have as common factor the whistler appearance, accompanied with mill crosses hand deformity, adducted thumbs, accompanied or not with inferior extremities deformities, intellectual capacity was normal.


REFERENCES

  1. Kohyama J, Shiiki T: Sleep disordered breathing during REM sleep in Freeman-Sheldon syndrome. Act Neurol Scand 2000; 102(6): 395-7.

  2. Alonso JL, Ali K: Síndrome de Freeman-Sheldon: Manifestaciones clínicas y manejo anestésico y quirúrgico. Ann Esp Pediat 2002; 56(2): 175-9.

  3. Oyston J: Online mendelian inheritance in man. Anesthesiology 1998; 89(3): 811-2.

  4. Ohyama K, Susami T, Kato Y, Amano H, Kuroda T: Freeman-Sheldon Syndrome: Case management from age 6 to 16 years. Cleft Pal Craniofac J 1997; 34(2): 151-3.

  5. Wenner SM, Shalvoy RM, Mass S: Two-stage correction of thumb adduction contracture in Freeman-Sheldon syndrome (craniocarpotarsal dysplasia). J Hand Surg Am 1989; 14(6): 937-40.

  6. Niké H, Staheli LT, Mosca VS: Management of clubfoot deformity in amyloplasia. J Pediatr Orthop Am 1997; 17(6): 803-7.

  7. Nualart L, Ochoa R, Hedrosa J: Apertura del espacio en el pulgar aducto congénito con técnica del colgajo dorsal. Rev Mex Ortop Traum 1994; 8(3): 138-9.

  8. Littler JW: The prevention and the correction of adduction contracture of the thumb. Clin Orthop 1959; 13: 182-5.

  9. Hall J: Arthrogryposis multiplex congenital: etiology, genetics, classification, diagnostic proach and general aspects. J Pediatr Orthop Br 1997; 6(3): 159-65.

  10. Rinsky LA, Bleck EE: Freeman-Sheldon («whistling face») syndrome. J Bone Joint Surg Am 1976; 58(1): 148-50.

  11. Rabb M, Minkowitz H, Hagberg CA: Blind intubation through the laryngeal mask airway for management of the difficult airway in infants. Anesthesiology 1996; 84(6): 1510-1.

  12. Dorit L, Miriam Y, Shlomo W, Tally LS: Progressive neurological deterioration in a child with distal arthrogryposis and whistling face. J Medical Genet 2000; 37(3): 231-3.

  13. Darin N, Kimber E, Kroksmark AK, Tulinius M: Multiple congenital contractures: Birth prevalence, etiology and outcome. J Pediatr 2002; 140(1): 61-7.




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