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Congenital Malformations

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Plastic and Reconstructive Surgery

Part of the book series: Springer Specialist Surgery Series ((SPECIALIST))

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Summary

Vascular birthmarks are a common cutaneous finding in newborns and infants. Characterization of these vascular anomalies is challenging and the terminology has historically been confusing. A classification system was proposed by Mulliken and Glowacki and modified by the International Society for the Study of Vascular Anomalies in 1996 to help clarify this confusion. It separates vascular anomalies into two distinct categories, tumors and vascular malformations, based on clinical appearance, natural history, and pathologic characteristics. The term hemangioma is reserved for congenital vascular tumors, while vascular malformations include a variety of lesions with varying clinical presentations. Proper identification and classification of these lesions have important implications in their management and treatment.

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Abbreviations

AVM:

Arteriovenous malformations

BRBN:

Blue rubber bleb nevus

CM:

Capillary malformations

CMTC:

Cutis marmorata telangiectatica congenital

GVM:

Glomuvenous malformations

ISSVA:

International Society for the Study of Vascular Anomalies

KTS:

Klippel—Trenaunay syndrome

LM:

Lymphatic malformation

NICH:

Noninvoluting congenital heman-giomas

PWS:

Port wine stains

PHACES:

Posterior fossa, Hemangioma, Arterial, Cardiac defect, Coarctation of the aorta, Eye anomaly, Sternal clefting, or Supraumbilical raphe

PDL:

Pulsed dye laser

RICH:

Rapidly Involuting Congenital Hemangiomas

SWS:

Sturge—Weber syndrome

VM:

Venous malformations

References

  1. Ashinoff R, Geronemus RJ. Flashlamp-pumped pulsed dye laser for port-wine stains in infancy: earlier versus later treatment. J Am Acad Dermatol. 1991;24:467–472.

    Article  PubMed  CAS  Google Scholar 

  2. Amitai DB, Fichman S, Merlob P, Morad Y, Lapidoth M, Metzker A. Cutis marmorata telangiectatica congenita: clinical findings in 85 patients. Pediatr Dermatol. 2000;17:100–104.

    Article  PubMed  CAS  Google Scholar 

  3. Bauland CG, van Steensel MA, Steijlen PM, Rieu PN, Spauwen PH. The pathogenesis of hemangiomas: a review. Plast Reconstr Surg. 2006;117:29e–35e.

    Article  PubMed  Google Scholar 

  4. Bennett ML, Fleischer AB Jr, Chamlin SL, Frieden IJ. Oral corticosteroid use is effective for cutaneous heman-giomas: an evidence-based evaluation. Arch Dermatol. 2001;137:1208–1213.

    PubMed  CAS  Google Scholar 

  5. Biesecker LG, Happle R, Mulliken JB, et al. Proteus syndrome: diagnostic criteria, differential diagnosis, and patient evaluation. Am J Med Genet. 1999;84:389–395.

    Article  PubMed  CAS  Google Scholar 

  6. Blei F, Walter J, Orlow SJ, Marchuk DA. Familial segregation of hemangimas and vascular malformations as an autosomal dominant trait. Arch Dermatol. 1998;134:718–722.

    Article  PubMed  CAS  Google Scholar 

  7. Bloom DC, Perkins JA, Manning SC. Management of lymphatic malformations. Curr Opin Otolaryngol Head Neck Surg. 2004;131:784–786.

    Article  Google Scholar 

  8. Boon LM, Mulliken JB, Enjolras O, Vikkula M. Glomuvenous malformation (glomangioma) and venous malformation. Distinct clinicopathologic and genetic entities. Arch Dermatol. 2004;140:971–976.

    Article  PubMed  Google Scholar 

  9. Bruckner AL, Frieden IJ. Hemangiomas of infancy. J Am Acad Dermatol. 2003;48:477–493.

    Article  PubMed  Google Scholar 

  10. Burton BK, Schulz CJ, Angle B, Burd LI. An increased incidence of haemangiomas in infants born following chorionic villus sampling (CVS). Prenat Diagn. 1995;15:209–214.

    Article  PubMed  CAS  Google Scholar 

  11. Ceisler EJ, Santos L, Blei F. Periocular hemangiomas: what every physician should know. Pediatr Dermatol. 2004;21:1–9

    Article  PubMed  Google Scholar 

  12. Chamlin SL, Haggstrom AN, Drolet BA, et al. Multicenter prospective study of ulcerated hemangiomas. J Pediatr. 2007;151:684–689.

    Article  PubMed  Google Scholar 

  13. Darmstadt GL. Perianal lymphangioma circumscriptum mistaken for genital warts. Pediatrics. 1996;98:461–463.

    PubMed  CAS  Google Scholar 

  14. Dubois J, Garel L. Imaging and therapeutic approach of hemangiomas and vascular malformationsin the pediat-ric age group. Pediatr Radiol. 1999;29:879–893.

    Article  PubMed  CAS  Google Scholar 

  15. Enjolras O, Mulliken JB. Vascular tumors and vascular malformations (new issues). Adv Dermatol. 1997;13:375–423.

    PubMed  CAS  Google Scholar 

  16. Finn MC, Glowacki J, Mulliken JB. Congenital vascular lesions: clinical application of a new classification. J Pediatr Surg. 1983;18:894–900

    Article  PubMed  CAS  Google Scholar 

  17. Frieden IJ, Eichenfield LF, Esterly NB, Geronemus R, Mallory SB. Guidelines of care for hemangiomas of infancy. American Academy of Dermatology Guidelines/ Outcomes Committee. J Am Acad Dermatol. 1997;37:631–637

    Article  PubMed  CAS  Google Scholar 

  18. Garzon MC, Huang JT, Enjolras O, Frieden IJ. Vascular malformations (Part I). J Am Acad Dermatol. 2007;56:353–370

    Article  PubMed  Google Scholar 

  19. Garzon MC, Huang JT, Enjolras O, Frieden IJ. Vascular malformations (Part II: Associated syndromes). J Am Acad Dermatol. 2007;56:541–564

    Article  PubMed  Google Scholar 

  20. Garzon MC, Lucky AW, Hawrot A, Frieden IJ. Ultrapotent topical corticosteroid treatment of hemangiomas of infancy. J Am Acad Dermatol. 2005;52:281–286.

    Article  PubMed  Google Scholar 

  21. Goldberg NS, Hebert AA, Esterly NB. Sacral heman-giomas and multiple congenital abnormalities. Arch Dermatol. 1986;122:684–687.

    Article  PubMed  CAS  Google Scholar 

  22. Hancock BJ, St-Vil D, Luks F, Di Lorenzo M, Blanchard H. Complications of lymphangiomas in children. J Pediatr Surg. 1992;27:220–224.

    Article  PubMed  CAS  Google Scholar 

  23. Hemangioma Investigator Group, Haggstrom AN, Drolet BA, Baselga E, et al. Prospective study of infantile heman-giomas: demographic, prenatal, and perinatal characteristics. J Pediatr. 2007;150:291–294.

    Article  PubMed  Google Scholar 

  24. Higuera S, Gordley K, Metry DW, Stal S. Management of hemangiomas and pediatric vascular malformations. J Craniofac Surg. 2006;17:783–789.

    Article  PubMed  Google Scholar 

  25. Ho NT, Lansang P, Pope E. Topical imiquimod in the treatment of infantile hemangiomas: a retrospective study. J Am Acad Dermatol. 2007;56:63–68.

    Article  PubMed  Google Scholar 

  26. Huang SA, Tu HM, Harney JW, et al. Severe hypo-thryoidism caused by type 3 iodothryronine deiodinase in infantile hemangiomas. N Engl J Med. 2000;343:185–189.

    Article  PubMed  CAS  Google Scholar 

  27. Jasim ZF, Handley JM. Treatment of pulsed dye laser-resistant port wine stain birthmarks. J Am Acad Dermatol. 2007;57:677–682.

    Article  PubMed  Google Scholar 

  28. Kohout MP, Hansen M, Pribaz JJ, Mulliken JB. Arterio-venous malformations of the head and neck: natural history and management. Plast Reconstr Surg. 1998;102:643–654.

    PubMed  CAS  Google Scholar 

  29. Krol A, MacArthur CJ. Congenital hemangiomas: rapidly involuting and noninvoluting congenital hemangiomas. Arch Facial Plast Surg. 2005;7:307–311.

    Article  PubMed  Google Scholar 

  30. Lopriore E, Markhorst DG. Diffuse neonatal heman-giomatosis: new views on diagnostic criteria and prognosis. Acta Paediatr. 1999;88:93–97.

    Article  PubMed  CAS  Google Scholar 

  31. Maari C, Frieden IJ. Klippel-Trénaunay syndrome: the importance of “geographic stains” in identifying lymphatic disease and risk of complications. J Am Acad Dermatol. 2004;51:391–398.

    Article  PubMed  Google Scholar 

  32. Maguiness S, Guenther L. Kasabach—Merritt syndrome. J Cutan Med Surg. 2002;6:335–359.

    Article  PubMed  Google Scholar 

  33. Mazereeuw-Hautier J, Syed S, Harper J. Bilateral facial capillary malformation associated with eye and brain abnormalities. Arch Dermatol. 2006;142:994–998.

    Article  PubMed  Google Scholar 

  34. McAlvany JP, Jorizzo JL, Zanolli D, et al. Magnetic resonance imaging in the evaluation of lymphangioma circumscriptum. Arch Dermatol. 1993;129:194–197.

    Article  PubMed  CAS  Google Scholar 

  35. Metry DW. Potential complications of segmental heman-giomas of infancy. Semin Cutan Med Surg. 2004;23:107–115.

    Article  PubMed  Google Scholar 

  36. Metry DW, Dowd CF, Barkovich AJ, Frieden IJ. The many faces of PHACE syndrome. J Pediatr. 2001;139:117–123.

    Article  PubMed  CAS  Google Scholar 

  37. Metry DW, Haggstrom AN. Drolet BA, et al. A prospective study of PHACE syndrome in infantile hemangiomas: demographic features, clinical findings, and complications. Am J Med Genet A. 2006;140:975–986.

    PubMed  CAS  Google Scholar 

  38. Metz BJ, Rubenstein MC, Levy ML, Metry DW. Response of ulcerated perineal hemangiomas of infancy to becaplermin gel, a recombinant human platelet-derived growth factor. Arch Dermatol. 2004;140:867–870.

    Article  PubMed  Google Scholar 

  39. Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg. 1982;69:412–422.

    Article  PubMed  CAS  Google Scholar 

  40. Paller AS. The Sturge-Weber syndrome. Pediatr Dermatol. 1987;4:300–304.

    Article  PubMed  CAS  Google Scholar 

  41. Peachey RD, Lim CC, Whimster IW. Lymphangioma of skin. A review of 65 cases. Br J Dermatol. 1970;83:519–527.

    Article  PubMed  CAS  Google Scholar 

  42. Powell TG, West CR, Pharoah PO, Cooke RW. Epidemiology of strawberry haemangioma in low birthweight infants. Br J Dermatol. 1987;116:635–641.

    Article  PubMed  CAS  Google Scholar 

  43. Railan D, Parlette EC, Uebelhoer NS, Rohrer TE. Laser treatment of vascular lesions. Clin Dermatol. 2006;24:8–15.

    Article  PubMed  Google Scholar 

  44. Puig S, Aref H, Chigot V, Bonin B, Brunelle F. Classification of venous malformations in children and implications for sclerotherapy. Pediatr Radiol. 2003;33:99–103.

    PubMed  Google Scholar 

  45. Renfro L, Geronemus RG. Anatomic differences of port-wine stains in response to treatment with the pulsed dye laser. Arch Dermatol. 1993;129:182–188.

    Article  PubMed  CAS  Google Scholar 

  46. Seccia A, Salgarello M, Farallo E, Falappa PG. Combined radiological and surgical treatment of arteriovenous malformations of the head and neck. Ann Plast Surg. 1999;43:359–366.

    Article  PubMed  CAS  Google Scholar 

  47. Tallman B, Tan OT, Morelli JG, et al. Location of port-wine stains and the liklihood of ophthalmic and/or central nervous system complications. Pediatrics. 1991;87:323–327.

    PubMed  CAS  Google Scholar 

  48. Tubbs RS, Wellons JC 3rd, Iskandar BJ, Oakes WJ. Isolated flat capillary midline lumbosacral hemangiomas as indicators of occult spinal dysraphism. J Neurosurg. 2004;100(2 Suppl Pediatrics):86–89.

    PubMed  Google Scholar 

  49. Turner JT, Cohen MM Jr, Biesecker LG. Reassessment of the Proteus syndrome literature: application of diagnostic criteria to published cases. Am J Med Genet. 2004;130:111–122.

    Article  Google Scholar 

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Author information

Authors and Affiliations

  1. Department of Dermatology and Plastic Surgery, Cleveland Clinic, Cleveland, OH, USA

    Jennifer Lucas

  2. Department of Dermatology, Cleveland Clinic Foundation, Cleveland, OH, USA

    Christopher Gasbarre & Allison T. Vidimos

Authors
  1. Jennifer Lucas
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  2. Christopher Gasbarre
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  3. Allison T. Vidimos
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Editor information

Editors and Affiliations

  1. Department of Plastic Surgery, Cleveland Clinic, Cleveland, OH, USA

    Maria Z. Siemionow (Professor of Surgery, Director of Plastic Surgery Research, Head of Microsurgery Training) (Professor of Surgery, Director of Plastic Surgery Research, Head of Microsurgery Training)

  2. Department of Plastic and Aesthetic, Hand, and Reconstructive Surgery, Caritas-Krankenhaus St. Josef, affiliated with Regensburg University, Regensburg, Germany

    Marita Eisenmann-Klein (Director) (Director)

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© 2010 Springer-Verlag London Limited

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Lucas, J., Gasbarre, C., Vidimos, A.T. (2010). Congenital Malformations. In: Siemionow, M.Z., Eisenmann-Klein, M. (eds) Plastic and Reconstructive Surgery. Springer Specialist Surgery Series. Springer, London. https://doi.org/10.1007/978-1-84882-513-0_14

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  • DOI: https://doi.org/10.1007/978-1-84882-513-0_14

  • Publisher Name: Springer, London

  • Print ISBN: 978-1-84882-512-3

  • Online ISBN: 978-1-84882-513-0

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