eMedicine Specialties > Orthopedic Surgery > Pediatrics

Streeter Dysplasia: Workup

Author: Twee Do, MD, Assistant Professor, Department of Pediatric Orthopedic Surgery, University of Cincinnati College of Medicine; Director, Neuromuscular Orthopedic Services, Cincinnati Children's Hospital Medical Center
Contributor Information and Disclosures

Updated: Mar 30, 2009

Workup

Laboratory Studies

  • Because amniotic band syndrome is an extrinsic yet spontaneous phenomenon, no good laboratory tests exist to detect its presence. Levels of a -fetoprotein have been shown to be elevated with normal acetylcholinesterase activity, but this elevation may be due to the anencephaly (with positive acetylcholinesterase isoenzymes) or fetal demise (negative acetylcholinesterase isoenzymes).

Imaging Studies

  • Serial ultrasounds can show the gross lack of formation, such as anencephaly or intrauterine amputations, but ultrasound studies of monozygotic twins have been disappointing.17 One study reported ultrasonic evidence of ABS in both twins, but only one twin had clinical manifestations (and those were severe). With the advancement of ultrasound technology and the availability of 3-D and 4-D ultrasound, the defect can now be characterized down to the level of how the nerves and muscles are affected distally.18,19,20 Prenatal diagnosis of atypical facial clefting should alert physicians to the possibility of ABS and other anomalies.21 This may help prenatal counseling and family preparation.
  • MRI could be considered preoperatively on limbs with deep bands to evaluate the neurovascular status.
  • Magnetic resonance angiography of limbs affected by ABS may reveal vessel deficiencies and variable anatomy that could affect the surgical outcome.

Diagnostic Procedures

  • Amniocentesis has been associated with membrane rupture after needle placement. Therefore, it has no significant place in the diagnosis or treatment of this syndrome.

More on Streeter Dysplasia

Overview: Streeter Dysplasia
Workup: Streeter Dysplasia
Treatment: Streeter Dysplasia
Follow-up: Streeter Dysplasia
Multimedia: Streeter Dysplasia
References
Further Reading
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References

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  2. Simpson J. Essays on diseases of the placenta. Dublin J Med Sci. 1836;10:220.

  3. Streeter G. Focal deficiencies in fetal tissues and their relation to intrauterine amputations. Contributions Embroyol Carnegie Inst. 1930;22:1-46.

  4. Patterson TJ. Congenital ring-constrictions. Br J Plast Surg. Apr 1961;14:1-31. [Medline].

  5. Torpin R. Amniochorionic Mesoblastic Fibrous Strings and Amnionic Bands: Associate Constricting Fetal Malformations or Fetal Death. Am J Obstet Gynecol. Jan 1 1965;91:65-75. [Medline].

  6. Hennigan, SP, Kuo, KN. Resistant talipes equinovarus associated with congenital constriction band syndrome. J Pediatr Orthop. 2000;20(2):240-245. [Medline].

  7. Zionts LE, Osterkamp JA, Crawford TO. Congenital annular bands in identical twins. A case report. J Bone Joint Surg Am. Mar 1984;66(3):450-3. [Medline].

  8. Ossipoff V, Hall BD. Etiologic factors in the amniotic band syndrome: a study of 24 patients. Birth Defects Orig Artic Ser. 1977;13(3D):117-32. [Medline].

  9. Ross MG. Pathogenesis of amniotic band syndrome. Am J Obstet Gynecol. Aug 2007;197(2):219-20; author reply 220. [Medline].

  10. Bouguila J, Ben Khoud N, Ghrissi A, Bellalah Z, Belghith A, Landolsi E, et al. [Amniotic band syndrome and facial malformations]. Rev Stomatol Chir Maxillofac. Dec 2007;108(6):526-9. [Medline].

  11. Light TR, Ogden JA. Congenital constriction band syndrome. Pathophysiology and treatment. Yale J Biol Med. May-Jun 1993;66(3):143-55. [Medline].

  12. Tanguy AF, Dalens BJ, Boisgard S. Congenital constricting band with pseudarthrosis of the tibia and fibula. A case report. Journal of Bone and Joint Surgery. 1995;77A(8):1251-4. [Medline].

  13. Zych, GA, Ballard, A. Constriction band causing pseudarthrosis and impending gangrene of the leg. A case report with successful treatment. Journal of Bone and Joint Surgery. 1983;65A(3):410-2. [Medline].

  14. Light TR. Growth and Development of the Hand. In: Carter PR, ed. Reconstruction of the Child's Hand. Philadelphia, Pa: Lea & Febilger;1991:122.

  15. Allington NJ, Kumar SJ, Guille JT. Clubfeet associated with congenital constriction bands of the ipsilateral lower extremity. J Pediatr Orthop. Sep-Oct 1995;15(5):599-603. [Medline].

  16. Gomez, VR. Clubfeet in congenital annular constricting bands. Clin Orthop Rel Res. 1996;323:155-162. [Medline].

  17. Donnenfeld AE, Dunn LK, Rose NC. Discordant amniotic band sequence in monozygotic twins. Am J Med Genet. Apr 1985;20(4):685-94. [Medline].

  18. Paladini D, Foglia S, Sglavo G. Congenital constriction band of the upper arm: the role of three-dimensional ultrasound in diagnosis, counseling and multidisciplinary consultation. Ultrasound Obstet Gynecol. May 2004;23(5):520-2. [Medline].

  19. Quintero RA, Morales WJ, Phillips J. In utero lysis of amniotic bands. Ultrasound Obstet Gynecol. Nov 1997;10(5):316-20. [Medline].

  20. Inubashiri E, Hanaoka U, Kanenishi K, Yamashiro C, Tanaka H, Yanagihara T, et al. 3D and 4D sonographic imaging of amniotic band syndrome in early pregnancy. J Clin Ultrasound. Nov-Dec 2008;36(9):573-5. [Medline].

  21. Chen CP. Prenatal diagnosis of atypical facial clefting should alert amniotic band syndrome and prompt a search for associated amniotic bands and other structural anomalies. Genet Couns. 2007;18(2):255-7. [Medline].

  22. Coyle S, Karp JM, Shirakura A. Oral rehabilitation of a child with amniotic band syndrome. J Dent Child (Chic). Jan-Apr 2008;75(1):74-9. [Medline].

  23. Crombleholme TM, Dirkes K, Whitney TM, et al. Amniotic band syndrome in fetal lambs. I: Fetoscopic release and morphometric outcome. J Pediatr Surg. Jul 1995;30(7):974-8. [Medline].

  24. Ronderos-Dumit D, Briceno F, Navarro H. Endoscopic release of limb constriction rings in utero. Fetal Diagn Ther. 2006;21(3):255-8. [Medline].

  25. Soldado F, Aguirre M, Peiró JL, Fontecha CG, Esteves M, Velez R, et al. Fetal surgery of extremity amniotic bands: an experimental model of in utero limb salvage in fetal lamb. J Pediatr Orthop. Jan-Feb 2009;29(1):98-102. [Medline].

  26. Upton J, Tan C. Correction of constriction rings. J Hand Surg [Am]. Sep 1991;16(5):947-53. [Medline].

  27. Isogai N, Landis W, Kim TH. Formation of phalanges and small joints by tissue-engineering. J Bone Joint Surg Am. Mar 1999;81(3):306-16. [Medline].

  28. Dobyns JH. Congenital ring syndrome. In: Green's Operative Hand Surgery. 2nd ed. New York, NY: Churchill Livingstone;1988:505.

  29. Dal Monte A, Soncini G, Calderoni P. The treatment of congenital constricting bands by Ombredanne''s two stage operation. Review of 13 cases. Ital J Orthop Traumatol. Sep 1983;9(3):351-5. [Medline].

  30. Greene, WB. One stage release of congenital constriction bands. JBJS. 1993;75(A):650-655. [Medline].

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Further Reading

Related eMedicine topics

Anencephaly

Cleft Lip

Cleft Palate

Clubfoot

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Keywords

Streeter dysplasia, congenital constriction band syndrome, amniotic band syndrome, ABS, constricting rings, acrosyndactyly, intrauterine amputation, digital amputations, ADAM complex, limb body wall syndrome, pseudo-ainhum, pseudoainhum, hemihypertrophy, anterolateral bowing, pseudarthrosis, leg-length discrepancy, teratologic clubfeet

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Contributor Information and Disclosures

Author

Twee Do, MD, Assistant Professor, Department of Pediatric Orthopedic Surgery, University of Cincinnati College of Medicine; Director, Neuromuscular Orthopedic Services, Cincinnati Children's Hospital Medical Center
Twee Do, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons and American Academy of Pediatrics
Disclosure: Nothing to disclose.

Medical Editor

Charles T Mehlman, DO, MPH, Director, Musculoskeletal Outcomes Research, Associate Professor, Division of Pediatric Orthopedic Surgery, Cincinnati Children's Hospital Medical Center
Charles T Mehlman, DO, MPH is a member of the following medical societies: American Academy of Pediatrics, American Fracture Association, American Medical Association, American Orthopaedic Foot and Ankle Society, American Osteopathic Association, Arthroscopy Association of North America, North American Spine Society, Ohio State Medical Association, Pediatric Orthopaedic Society of North America, and Scoliosis Research Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

George H Thompson, MD, Director, Pediatric Orthopedics, Rainbow Babies and Children's Hospital
George H Thompson, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American Orthopaedic Association, Pediatric Orthopaedic Society of North America, and Scoliosis Research Society
Disclosure: Nothing to disclose.

CME Editor

Dinesh Patel, MD, FACS, Associate Clinical Professor of Orthopedic Surgery, Harvard Medical School; Chief of Arthroscopic Surgery, Department of Orthopedic Surgery, Massachusetts General Hospital
Dinesh Patel, MD, FACS is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American Association of Physicians of Indian Origin, American College of International Physicians, and American College of Surgeons
Disclosure: Nothing to disclose.

Chief Editor

Dennis P Grogan, MD, Clinical Professor, Department of Orthopedic Surgery, University of South Florida College of Medicine; Chief of Staff, Department of Orthopedic Surgery, Shriners Hospital for Children of Tampa
Dennis P Grogan, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American Medical Association, American Orthopaedic Association, American Orthopaedic Foot and Ankle Society, Eastern Orthopaedic Association, Irish American Orthopaedic Society, Pediatric Orthopaedic Society of North America, and Scoliosis Research Society
Disclosure: Nothing to disclose.

 
 
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