Freeman–Sheldon syndrome

Freeman–Sheldon syndrome
Classification and external resources
ICD-9-CM 759.89
OMIM 193700
DiseasesDB 31817
Orphanet 2053

Freeman–Sheldon syndrome (FSS), also termed distal arthrogryposis type 2A (DA2A), craniocarpotarsal dysplasia (or dystrophy), Cranio-carpo-tarsal syndrome, Windmill-Vane-Hand syndrome, or Whistling-face syndrome, was originally described by Freeman and Sheldon in 1938.[1][2]:577 Freeman–Sheldon syndrome is a rare form of multiple congenital contracture (MCC) syndromes (arthrogryposes) and is the most severe form of distal arthrogryposis (DA).[3][4][5]

Signs and symptoms

The symptoms of Freeman–Sheldon syndrome include drooping of the upper eyelids, strabismus, low-set ears, a long philtrum, gradual hearing loss, scoliosis, and walking difficulties. Gastroesophageal reflux has been noted during infancy, but usually improves with age. The tongue may be small, and the limited movement of the soft palate may cause nasal speech. Often there is an H- or Y-shaped dimpling of the skin over the chin.

Diagnosis

Freeman–Sheldon syndrome is a type of distal arthrogryposis, related to distal arthrogryposis type 1 (DA1).[6] In 1996, more strict criteria for the diagnosis of Freeman–Sheldon syndrome were drawn up, assigning Freeman–Sheldon syndrome as distal arthrogryposis type 2A (DA2A).[5]

On the whole, DA1 is the least severe; DA2B is more severe with additional features that respond less favourably to therapy. DA2A (Freeman–Sheldon syndrome) is the most severe of the three, with more abnormalities and greater resistance to therapy.[5]

Freeman–Sheldon syndrome has been described as a type of congenital myopathy.[7]

In March 2006, Stevenson et al. published strict diagnostic criteria for distal arthrogryposis type 2A (DA2A) or Freeman–Sheldon syndrome. These included two or more features of distal arthrogryposis: microstomia, whistling-face, nasolabial creases, and 'H-shaped' chin dimple.[4]

Cause

FSS is caused by genetic changes. Krakowiak et al. (1998) mapped the distal arthrogryposis multiplex congenita (DA2B; MIM #601680) gene, a syndrome very similar in phenotypic expression to classic FSS, to 11p15.5-pter.[8][9] Other mutations have been found as well.[10][11] In FSS, inheritance may be either autosomal dominant, most often demonstrated.[12][13][14] or autosomal recessive (MIM 277720).[15][16][17][18] Alves and Azevedo (1977) note most reported cases of DA2A have been identified as new allelic variation.[19] Toydemir et al. (2006) showed that mutations in embryonic myosin heavy chain 3 (MYH3; MIM *160270), at 17p-13.1-pter, caused classic FSS phenotype, in their screening of 28 (21 sporadic and 7 familial) probands with distal arthrogryposis type 2A.[20][21] In 20 patients (12 and 8 probands, respectively), missense mutations (R672H; MIM *160270.0001 and R672C; MIM *160270.0002) caused substitution of arg672, an embryonic myosin residue retained post-embryonically.[20][21][21] Of the remaining 6 patients in whom they found mutations, 3 had missense private de novo (E498G; MIM *160270.0006 and Y583S) or familial mutations (V825D; MIM *160270.0004); 3 other patients with sporadic expression had de novo mutations (T178I; MIM *160270.0003), which was also found in DA2B; 2 patients had no recognized mutations.[20][21]

Management

Surgical and anaesthetic considerations

Patients must have early consultation with craniofacial and orthopaedic surgeons, when craniofacial,[22][23][24] clubfoot,[25] or hand correction[26][27][28][29] is indicated to improve function or aesthetics. Operative measures should be pursued cautiously, with avoidance of radical measures and careful consideration of the abnormal muscle physiology in Freeman–Sheldon syndrome. Unfortunately, many surgical procedures have suboptimal outcomes, secondary to the myopathy of the syndrome.

When operative measures are to be undertaken, they should be planned for as early in life as is feasible, in consideration of the tendency for fragile health. Early interventions hold the possibility to minimise developmental delays and negate the necessity of relearning basic functions.

Due to the abnormal muscle physiology in Freeman–Sheldon syndrome, therapeutic measures may have unfavourable outcomes.[30] Difficult endotracheal intubations and vein access complicate operative decisions in many DA2A patients, and malignant hyperthermia (MH) may affect individuals with FSS, as well.[31][32][33][34] Cruickshanks et al. (1999) reports uneventful use of non-MH-triggering agents.[35] Reports have been published about spina bifida occulta in anaesthesia management[36] and cervical kyphoscoliosis in intubations.[37]

Psychiatric considerations

Patients and their parents must receive psychotherapy, which should include marriage counselling.[38] Mitigation of lasting psychological problems, including depression secondary to chronic illness and posttraumatic stress disorder (PTSD), can be very successfully addressed with early interventions.[39] This care may come from the family physician, or other attending physician, whoever is more appropriate; specialist care is generally not required.[40][41][42] Lewis and Vitulano (2003) note several studies suggesting predisposal for psychopathology in paediatric patients with chronic illness.[43] Esch (2002) advocates preventive psychiatry supports to facilitate balance of positive and negative stressors associated with chronic physical pathology.[44] Patients with FSS should have pre-emptive and ongoing mixed cognitive therapy-psychodynamic psychotherapy for patients with FSS and cognitive-behavioural therapy (CBT), if begun after onset of obvious pathology.

Adler (1995) cautioned the failure of modern medicine to implement the biopsychosocial model,[45] which incorporates all aspects of a patient’s experience in a scientific approach into the clinical picture,[46][47][48][49] often results in chronically-ill patients deferring to non-traditional and alternative forms of therapy, seeking to be understood as a whole, not a part,[50] which may be problematic among patients with FSS.

Furthermore, neuropsychiatry, physiological, and imaging studies[51][52][53][54][55] have shown PTSD and depression to be physical syndromes, in many respects, as they are psychiatric ones in demonstrating limbic system physiological and anatomy disturbances. Attendant PTSD hyperarousal symptoms, which additionally increase physiological stress, may play a part in leading to frequent MH-like hyperpyrexia and speculate on its influence on underlying myopathology of FSS in other ways. PTSD may also bring about developmental delays or developmental stagnation, especially in paediatric patients.[56]

With psychodynamic psychotherapy, psychopharmacotherapy may need to be considered. Electroconvulsive therapy (ECT) is advised against, in light of abnormal myophysiology, with predisposal to MH.

Medical emphasis

General health maintenance should be the therapeutic emphasis in Freeman–Sheldon syndrome. The focus is on limiting exposure to infectious diseases because the musculoskeletal abnormalities make recovery from routine infections much more difficult in FSS. Pneumonitis and bronchitis often follow seemingly mild upper respiratory tract infections. Though respiratory challenges and complications faced by a patient with FSS can be numerous, the syndrome’s primary involvement is limited to the musculoskeletal systems, and satisfactory quality and length of life can be expected with proper care.

Prognosis

There are little data on prognosis. Rarely, some patients have died in infancy from respiratory failure; otherwise, life expectancy is considered to be normal.

Epidemiology

By 1990, 65 patients had been reported in the literature, with no sex or ethnic preference notable.[57] Some individuals present with minimal malformation; rarely patients have died during infancy as a result of severe central nervous system involvement[58] or respiratory complications.[59] Several syndromes are related to the Freeman–Sheldon syndrome spectrum, but more information is required before undertaking such nosological delineation.[60][61][62]

Research directions

One research priority is to determine the role and nature of malignant hyperthermia in FSS. Such knowledge would benefit possible surgical candidates and the anaesthesiology and surgical teams who would care for them. MH may also be triggered by stress in patients with muscular dystrophies.[63] Much more research is warranted to evaluate this apparent relationship of idiopathic hyperpyrexia, MH, and stress. Further research is wanted to determine epidemiology of psychopathology in FSS and refine therapy protocols.

References

  1. ↑ Freeman, EA; Sheldon JH (1938). "Cranio-carpo-tarsal dystrophy: undescribed congenital malformation". Arch Dis Child. 13: 277–83. doi:10.1136/adc.13.75.277.
  2. ↑ James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. ISBN 0-7216-2921-0.
  3. ↑ Online Mendelian Inheritance in Man (OMIM) 193700
  4. 1 2 Stevenson, DA; Carey JC; Palumbos J; Rutherford A; Dolcourt J; Bamshad MJ (March 2006). "Clinical characteristics and natural history of Freeman-Sheldon syndrome". Pediatrics. 117 (3): 754–62. doi:10.1542/peds.2005-1219. PMID 16510655.
  5. 1 2 3 Bamshad M, Jorde LB, Carey JC (November 1996). "A revised and extended classification of the distal arthrogryposes". Am. J. Med. Genet. 65 (4): 277–81. doi:10.1002/(SICI)1096-8628(19961111)65:4<277::AID-AJMG6>3.0.CO;2-M. PMID 8923935.
  6. ↑ Hall JG, Reed SD, Greene G (February 1982). "The distal arthrogryposes: delineation of new entities—review and nosologic discussion". Am. J. Med. Genet. 11 (2): 185–239. doi:10.1002/ajmg.1320110208. PMID 7039311.
  7. ↑ Vanĕk J, Janda J, Amblerová V, Losan F (June 1986). "Freeman-Sheldon syndrome: a disorder of congenital myopathic origin?". J. Med. Genet. 23 (3): 231–6. doi:10.1136/jmg.23.3.231. PMC 1049633Freely accessible. PMID 3723551.
  8. ↑ Krakowiak PA, O'Quinn JR, Bohnsack JF, et al. (1997). "A variant of Freeman-Sheldon syndrome maps to 11p15.5-pter". Am. J. Hum. Genet. 60 (2): 426–32. PMC 1712403Freely accessible. PMID 9012416.
  9. ↑ Krakowiak PA, Bohnsack JF, Carey JC, Bamshad M (1998). "Clinical analysis of a variant of Freeman-Sheldon syndrome (DA2B)". Am. J. Med. Genet. 76 (1): 93–8. doi:10.1002/(SICI)1096-8628(19980226)76:1<93::AID-AJMG17>3.0.CO;2-K. PMID 9508073.
  10. ↑ Sung SS, Brassington AM, Krakowiak PA, Carey JC, Jorde LB, Bamshad M (2003). "Mutations in TNNT3 cause multiple congenital contractures: a second locus for distal arthrogryposis type 2B". Am. J. Hum. Genet. 73 (1): 212–4. doi:10.1086/376418. PMC 1180583Freely accessible. PMID 12865991.
  11. ↑ Sung SS, Brassington AM, Grannatt K, et al. (2003). "Mutations in genes encoding fast-twitch contractile proteins cause distal arthrogryposis syndromes". Am. J. Hum. Genet. 72 (3): 681–90. doi:10.1086/368294. PMC 1180243Freely accessible. PMID 12592607.
  12. ↑ Aalam M, Kühhirt M (1972). "[Windmill vane-like finger deformities]". Z Orthop Ihre Grenzgeb (in German). 110 (3): 395–8. PMID 4263226.
  13. ↑ Jorgenson RJ (1974). "M--craniocarpotarsal dystrophy (whistling face syndrome) in two families". Birth Defects Orig. Artic. Ser. 10 (5): 237–42. PMID 4220006.
  14. ↑ Wettstein A, Buchinger G, Braun A, von Bazan UB (1980). "A family with whistling-face-syndrome". Hum. Genet. 55 (2): 177–89. doi:10.1007/BF00291765. PMID 7450762.
  15. ↑ Kousseff BG, McConnachie P, Hadro TA (1982). "Autosomal recessive type of whistling face syndrome in twins". Pediatrics. 69 (3): 328–31. PMID 7199706.
  16. ↑ Kaul KK (1981). "Whistling face syndrome (craniocarpotarsal dysplasia)". Indian Pediatr. 18 (1): 72–3. PMID 7262998.
  17. ↑ Guzzanti V, Toniolo RM, Lembo A (1990). "[The Freeman-Sheldon syndrome]". Arch Putti Chir Organi Mov (in Italian). 38 (1): 215–22. PMID 2136374.
  18. ↑ Sánchez JM, Kaminker CP (1986). "New evidence for genetic heterogeneity of the Freeman-Sheldon syndrome". Am. J. Med. Genet. 25 (3): 507–11. doi:10.1002/ajmg.1320250312. PMID 3789012.
  19. ↑ Alves AF, Azevedo ES (1977). "Recessive form of Freeman-Sheldon's syndrome or 'whistling face',". J. Med. Genet. 14 (2): 139–41. doi:10.1136/jmg.14.2.139. PMC 1013533Freely accessible. PMID 856233.
  20. 1 2 3 Toydemir RM, Rutherford A, Whitby FG, Jorde LB, Carey JC, Bamshad MJ (2006). "Mutations in embryonic myosin heavy chain (MYH3) cause Freeman-Sheldon syndrome and Sheldon-Hall syndrome". Nat. Genet. 38 (5): 561–5. doi:10.1038/ng1775. PMID 16642020.
  21. 1 2 3 4 Online Mendelian Inheritance in Man (OMIM) MYOSIN, HEAVY CHAIN 3, SKELETAL MUSCLE, EMBRYONIC; MYH3 -160720
    b .0001 c .0002 d .0004 e .0006, .0003
  22. ↑ Vaitiekaitis AS, Hornstein L, Neale HW (September 1979). "A new surgical procedure for correction of lip deformity in cranio-carpo-tarsal dysplasia (whistling face syndrome)". J Oral Surg. 37 (9): 669–72. PMID 288890.
  23. ↑ Nara T (July 1981). "Reconstruction of an upper lip and the coloboma in the nasal ala accompanying with Freeman-Sheldon syndrome". Nippon Geka Hokan. 50 (4): 626–32. PMID 7316645.
  24. ↑ Ferreira LM, Minami E, Andrews Jde M (April 1994). "Freeman-Sheldon syndrome: surgical correction of microstomia". Br J Plast Surg. 47 (3): 201–2. doi:10.1016/0007-1226(94)90056-6. PMID 8193861.
  25. ↑ Malkawi H, Tarawneh M (July 1983). "The whistling face syndrome, or craniocarpotarsal dysplasia. Report of two cases in a father and son and review of the literature". J Pediatr Orthop. 3 (3): 364–9. doi:10.1097/01241398-198307000-00017. PMID 6874936.
  26. ↑ Call WH, Strickland JW (March 1981). "Functional hand reconstruction in the whistling-face syndrome". J Hand Surg [Am]. 6 (2): 148–51. doi:10.1016/s0363-5023(81)80168-2. PMID 7229290.
  27. ↑ Martini AK, Banniza von Bazan U (1983). "[Hand deformities in Freeman-Sheldon syndrome and their surgical treatment]". Z Orthop Ihre Grenzgeb (in German). 121 (5): 623–9. doi:10.1055/s-2008-1053288. PMID 6649810.
  28. ↑ Martini AK, Banniza von Bazan U (December 1982). "[Surgical treatment of the hand deformity in Freeman-Sheldon syndrome]". Handchir Mikrochir Plast Chir (in German). 14 (4): 210–2. PMID 6763591.
  29. ↑ Wenner SM, Shalvoy RM (November 1989). "Two-stage correction of thumb adduction contracture in Freeman-Sheldon syndrome (craniocarpotarsal dysplasia)". J Hand Surg [Am]. 14 (6): 937–40. doi:10.1016/S0363-5023(89)80040-1. PMID 2584652.
  30. ↑ Aldinger G, Eulert J (1983). "[The Freeman-Sheldon Syndrome]". Z Orthop Ihre Grenzgeb (in German). 121 (5): 630–3. doi:10.1055/s-2008-1053289. PMID 6649811.
  31. ↑ Laishley RS, Roy WL (1986). "Freeman-Sheldon syndrome: report of three cases and the anaesthetic implications". Can Anaesth Soc J. 33 (3 Pt 1): 388–93. doi:10.1007/BF03010755. PMID 3719442.
  32. ↑ Munro HM, Butler PJ, Washington EJ (1997). "Freeman-Sheldon (whistling face) syndrome. Anaesthetic and airway management". Paediatr Anaesth. 7 (4): 345–8. doi:10.1046/j.1460-9592.1997.d01-90.x. PMID 9243695.
  33. ↑ Yamamoto S, Osuga T, Okada M, et al. (1994). "[Anesthetic management of a patient with Freeman-Sheldon syndrome]". Masui (in Japanese). 43 (11): 1748–53. PMID 7861610.
  34. ↑ Sobrado CG, Ribera M, Martí M, Erdocia J, Rodríguez R (1994). "[Freeman-Sheldon syndrome: generalized muscular rigidity after anesthetic induction]". Rev Esp Anestesiol Reanim (in Spanish). 41 (3): 182–4. PMID 8059048.
  35. ↑ Cruickshanks GF, Brown S, Chitayat D (1999). "Anesthesia for Freeman-Sheldon syndrome using a laryngeal mask airway". Can J Anaesth. 46 (8): 783–7. doi:10.1007/BF03013916. PMID 10451140.
  36. ↑ Namiki M, Kawamata T, Yamakage M, Matsuno A, Namiki A (2000). "[Anesthetic management of a patient with Freeman-Sheldon syndrome]". Masui (in Japanese). 49 (8): 901–2. PMID 10998888.
  37. ↑ Vas L, Naregal P (1998). "Anaesthetic management of a patient with Freeman Sheldon syndrome". Paediatr Anaesth. 8 (2): 175–7. doi:10.1046/j.1460-9592.1998.00676.x. PMID 9549749.
  38. ↑ Doherty WJ, McDaniel SH, Hepworth J (1998). "[Medical family therapy in children with chronic illness]". Prax Kinderpsychol Kinderpsychiatr (in German). 47 (1): 1–18. PMID 9522592.
  39. ↑ Benierakis CE (1995). "The function of the multidisciplinary team in child psychiatry--clinical and educational aspects". Can J Psychiatry. 40 (6): 348–53. PMID 7585406.
  40. ↑ Röhrborn H (December 1986). "[Psychotherapy in internal medicine? Systematic considerations on the problem]". Z Gesamte Inn Med (in German). 41 (23): 664–6. PMID 3577259.
  41. ↑ Martin JR, Wilikofsky AS (December 2005). "Integrating genetic counseling into family medicine". Am Fam Physician. 72 (12): 2444, 2446. PMID 16370402.
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  43. ↑ Lewis M, Vitulano LA (2003). "Biopsychosocial issues and risk factors in the family when the child has a chronic illness". Child Adolesc Psychiatr Clin N Am. 12 (3): 389–99, v. doi:10.1016/S1056-4993(03)00024-5. PMID 12910814.
  44. ↑ Esch T (2002). "[Health in stress: change in the stress concept and its significance for prevention, health and life style]". Gesundheitswesen (in German). 64 (2): 73–81. doi:10.1055/s-2002-20275. PMID 11904846.
  45. ↑ Lesinskiene S, Kajokiene A, Pūras D (2002). "[Complexity of help to children with developmental disabilities in the context of the establishment of Early Rehabilitation Services]" (PDF). Medicina (Kaunas) (in Lithuanian). 38 (4): 458–65. PMID 12474797.
  46. ↑ Engel GL (May 1980). "The clinical application of the biopsychosocial model". Am J Psychiatry. 137 (5): 535–44. doi:10.1176/ajp.137.5.535. PMID 7369396.
  47. ↑ Engel GL (1997). "From biomedical to biopsychosocial. Being scientific in the human domain". Psychosomatics. 38 (6): 521–8. doi:10.1016/s0033-3182(97)71396-3. PMID 9427848.
  48. ↑ Adler RH (December 1996). "[Steps to integration or the fight with the Hydra "dualism"]". Schweiz. Rundsch. Med. Prax. (in German). 85 (51-52): 1641–6. PMID 9026876.
  49. ↑ Adler RH (April 1996). "[Are there psychological factors which lead to iatrogenic disorders?]". Schweiz Med Wochenschr (in German). 126 (15): 612–5. PMID 8668975.
  50. ↑ Adler RH (1995). "[Is there a scientific alternative to alternative medicine?]". Schweiz. Rundsch. Med. Prax. (in German). 84 (51-52): 1517–21. PMID 8571011.
  51. ↑ Fu CH, McGuire PK (July 1999). "Functional neuroimaging in psychiatry". Philos. Trans. R. Soc. Lond., B, Biol. Sci. 354 (1387): 1359–70. doi:10.1098/rstb.1999.0484. PMC 1692639Freely accessible. PMID 10466156.
  52. ↑ Sheline YI, Wang PW, Gado MH, Csernansky JG, Vannier MW (April 1996). "Hippocampal atrophy in recurrent major depression". Proc. Natl. Acad. Sci. U.S.A. 93 (9): 3908–13. doi:10.1073/pnas.93.9.3908. PMC 39458Freely accessible. PMID 8632988.
  53. ↑ Liberzon I, Zubieta JK, Fig LM, Phan KL, Koeppe RA, Taylor SF (May 2002). "mu-Opioid receptors and limbic responses to aversive emotional stimuli". Proc. Natl. Acad. Sci. U.S.A. 99 (10): 7084–9. doi:10.1073/pnas.102174799. PMC 124532Freely accessible. PMID 12011464.
  54. ↑ Kim JJ, Foy MR, Thompson RF (May 1996). "Behavioral stress modifies hippocampal plasticity through N-methyl-D-aspartate receptor activation". Proc. Natl. Acad. Sci. U.S.A. 93 (10): 4750–3. doi:10.1073/pnas.93.10.4750. PMC 39350Freely accessible. PMID 8643474.
  55. ↑ Coplan JD, Andrews MW, Rosenblum LA, et al. (February 1996). "Persistent elevations of cerebrospinal fluid concentrations of corticotropin-releasing factor in adult nonhuman primates exposed to early-life stressors: implications for the pathophysiology of mood and anxiety disorders". Proc. Natl. Acad. Sci. U.S.A. 93 (4): 1619–23. doi:10.1073/pnas.93.4.1619. PMC 39991Freely accessible. PMID 8643680.
  56. ↑ States JH, St Dennis CD (2003). "Chronic Sleep Disruption and the Reexperiencing Cluster of Posttraumatic Stress Disorder Symptoms Are Improved by Olanzapine: Brief Review of the Literature and a Case-Based Series". Prim Care Companion J Clin Psychiatry. 5 (2): 74–9. doi:10.4088/PCC.v05n0203. PMC 353040Freely accessible. PMID 15156234. (subscription required (help)).
  57. ↑ "Freeman-Sheldon syndrome". Orphanet Encyclopedia. Comier-Daire.
  58. ↑ Zampino G, Conti G, Balducci F, Moschini M, Macchiaiolo M, Mastroiacovo P (March 1996). "Severe form of Freeman-Sheldon syndrome associated with brain anomalies and hearing loss". Am. J. Med. Genet. 62 (3): 293–6. doi:10.1002/(SICI)1096-8628(19960329)62:3<293::AID-AJMG17>3.0.CO;2-F. PMID 8882790.
  59. ↑ Rao SS, Chary R, Karan S (March 1979). "Freeman Sheldon syndrome in a newborn (whistling face)--a case report". Indian Pediatr. 16 (3): 291–2. PMID 110675.
  60. ↑ Fitzsimmons JS, Zaldua V, Chrispin AR (October 1984). "Genetic heterogeneity in the Freeman-Sheldon syndrome: two adults with probable autosomal recessive inheritance". J. Med. Genet. 21 (5): 364–8. doi:10.1136/jmg.21.5.364. PMC 1049318Freely accessible. PMID 6502650.
  61. ↑ Träger D (1987). "[Cranio-carpo-tarsal dysplasia syndrome (Freeman-Sheldon syndrome, whistling face syndrome)]". Z Orthop Ihre Grenzgeb (in German). 125 (1): 106–7. doi:10.1055/s-2008-1039687. PMID 3577337.
  62. ↑ Simosa V, Penchaszadeh VB, Bustos T (February 1989). "A new syndrome with distinct facial and auricular malformations and dominant inheritance". Am. J. Med. Genet. 32 (2): 184–6. doi:10.1002/ajmg.1320320209. PMID 2929657.
  63. ↑ Litman RS, Rosenberg H (2005). "Malignant hyperthermia: update on susceptibility testing". JAMA. 293 (23): 2918–24. doi:10.1001/jama.293.23.2918. PMID 15956637.

Eponym

It is named for Ernest Arthur Freeman (1900-1975), British Orthopedic Surgeon and Joseph Harold Sheldon (1920-1964), British Physician.

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