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婴幼儿血管瘤的流行病学、发病机制、临床特点和并发症

Author
Denise W Metry, MD
Section Editor
Moise L Levy, MD
Deputy Editor
Rosamaria Corona, MD, DSc
Translators
吴严, 主任医师,教授

引言

婴儿和儿童的血管病变分为两大类:肿瘤和血管畸形[1]。婴幼儿血管瘤是最常见的血管肿瘤。其他血管瘤包括化脓性肉芽肿、卡波西样血管内皮瘤和丛状血管瘤等。人们所称的快速退化型先天性血管瘤(rapidly involuting congenital hemangiomas, RICH)和非退化型先天性血管瘤(non-involuting congenital hemangiomas, NICH)在组织病理学上貌似为杂交体,既有血管肿瘤的特点,也有血管畸形的特点。因为有过RICH演变为NICH的病例报道,有人提出RICH与NICH属于同一疾病谱范畴[2,3]。

婴幼儿血管瘤的特点是有一个生长期和一个退化期。与之相对,血管畸形可随儿童成长而相应增大,通常并不退化。血管畸形是源于毛细血管、动脉、静脉、淋巴管的结构异常或上述异常的组合。血管畸形将单独讨论。 (参见“新生儿血管病变”)

尽管婴幼儿血管瘤是良性的并具有自限性,但部分血管瘤可引起溃疡和永久性损容等并发症。此外,有的血管瘤可能损害重要的器官功能,或预示脊柱、中枢神经系统、循环系统和/或眼部的潜在发育异常。肝脏、脑部或消化道的血管瘤可能会引起危及生命的并发症,但这种情况较少见。血管瘤面积较大、位于面部和/或呈节段性形态是短期结局(以并发症发病率和治疗率测定)不佳最常见的预测指标[4]。 (参见下文‘节段性血管瘤’)

本文将概述婴幼儿血管瘤的流行病学、发病机制、临床特点和并发症。婴幼儿血管瘤的评估与治疗将单独讨论。 (参见“婴儿血管瘤的评估与诊断”“婴儿血管瘤的处理”)

流行病学

血管瘤是婴儿期最常见的肿瘤。婴幼儿血管瘤的真实发病率尚不清楚[5]。虽然传统上认为该病在白种人婴儿中的发病率高达10%[6,7],但估值为4%-5%可能更为准确[5,8]。婴幼儿血管瘤常在出生后数天到数月内被发现[9,10]。尽管多数血管瘤为散发性的,但也有过家族常染色体显性遗传的报道[11]。

                        

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Literature review current through: 2017-07 . | This topic last updated: 2017-07-06.
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References
Top
  1. Enjolras O, Mulliken JB. Vascular tumors and vascular malformations (new issues). Adv Dermatol 1997; 13:375.
  2. Bruckner AL, Frieden IJ. Hemangiomas of infancy. J Am Acad Dermatol 2003; 48:477.
  3. Mulliken JB, Enjolras O. Congenital hemangiomas and infantile hemangioma: missing links. J Am Acad Dermatol 2004; 50:875.
  4. Haggstrom AN, Drolet BA, Baselga E, et al. Prospective study of infantile hemangiomas: clinical characteristics predicting complications and treatment. Pediatrics 2006; 118:882.
  5. Kilcline C, Frieden IJ. Infantile hemangiomas: how common are they? A systematic review of the medical literature. Pediatr Dermatol 2008; 25:168.
  6. Jacobs AH, Walton RG. The incidence of birthmarks in the neonate. Pediatrics 1976; 58:218.
  7. Alper JC, Holmes LB. The incidence and significance of birthmarks in a cohort of 4,641 newborns. Pediatr Dermatol 1983; 1:58.
  8. Munden A, Butschek R, Tom WL, et al. Prospective study of infantile haemangiomas: incidence, clinical characteristics and association with placental anomalies. Br J Dermatol 2014; 170:907.
  9. PRATT AG. Birthmarks in infants. AMA Arch Derm Syphilol 1953; 67:302.
  10. JACOBS AH. Strawberry hemangiomas; the natural history of the untreated lesion. Calif Med 1957; 86:8.
  11. Blei F, Walter J, Orlow SJ, Marchuk DA. Familial segregation of hemangiomas and vascular malformations as an autosomal dominant trait. Arch Dermatol 1998; 134:718.
  12. Chiller KG, Passaro D, Frieden IJ. Hemangiomas of infancy: clinical characteristics, morphologic subtypes, and their relationship to race, ethnicity, and sex. Arch Dermatol 2002; 138:1567.
  13. Drolet BA, Esterly NB, Frieden IJ. Hemangiomas in children. N Engl J Med 1999; 341:173.
  14. Hemangioma Investigator Group, Haggstrom AN, Drolet BA, et al. Prospective study of infantile hemangiomas: demographic, prenatal, and perinatal characteristics. J Pediatr 2007; 150:291.
  15. Enjolras O, Gelbert F. Superficial hemangiomas: associations and management. Pediatr Dermatol 1997; 14:173.
  16. Finn MC, Glowacki J, Mulliken JB. Congenital vascular lesions: clinical application of a new classification. J Pediatr Surg 1983; 18:894.
  17. Leon-Villapalos J, Wolfe K, Kangesu L. GLUT-1: an extra diagnostic tool to differentiate between haemangiomas and vascular malformations. Br J Plast Surg 2005; 58:348.
  18. North PE, Waner M, Mizeracki A, Mihm MC Jr. GLUT1: a newly discovered immunohistochemical marker for juvenile hemangiomas. Hum Pathol 2000; 31:11.
  19. North PE, Waner M, Mizeracki A, et al. A unique microvascular phenotype shared by juvenile hemangiomas and human placenta. Arch Dermatol 2001; 137:559.
  20. Barnés CM, Huang S, Kaipainen A, et al. Evidence by molecular profiling for a placental origin of infantile hemangioma. Proc Natl Acad Sci U S A 2005; 102:19097.
  21. Yu Y, Fuhr J, Boye E, et al. Mesenchymal stem cells and adipogenesis in hemangioma involution. Stem Cells 2006; 24:1605.
  22. Boye E, Yu Y, Paranya G, et al. Clonality and altered behavior of endothelial cells from hemangiomas. J Clin Invest 2001; 107:745.
  23. Walter JW, North PE, Waner M, et al. Somatic mutation of vascular endothelial growth factor receptors in juvenile hemangioma. Genes Chromosomes Cancer 2002; 33:295.
  24. Nguyen VA, Fürhapter C, Romani N, et al. Infantile hemangioma is a proliferation of beta 4-negative endothelial cells adjacent to HLA-DR-positive cells with dendritic cell morphology. Hum Pathol 2004; 35:739.
  25. Khan ZA, Boscolo E, Picard A, et al. Multipotential stem cells recapitulate human infantile hemangioma in immunodeficient mice. J Clin Invest 2008; 118:2592.
  26. Ritter MR, Reinisch J, Friedlander SF, Friedlander M. Myeloid cells in infantile hemangioma. Am J Pathol 2006; 168:621.
  27. Bielenberg DR, Bucana CD, Sanchez R, et al. Progressive growth of infantile cutaneous hemangiomas is directly correlated with hyperplasia and angiogenesis of adjacent epidermis and inversely correlated with expression of the endogenous angiogenesis inhibitor, IFN-beta. Int J Oncol 1999; 14:401.
  28. Dadras SS, North PE, Bertoncini J, et al. Infantile hemangiomas are arrested in an early developmental vascular differentiation state. Mod Pathol 2004; 17:1068.
  29. Nguyen VA, Kutzner H, Fürhapter C, et al. Infantile hemangioma is a proliferation of LYVE-1-negative blood endothelial cells without lymphatic competence. Mod Pathol 2006; 19:291.
  30. Yu Y, Flint AF, Mulliken JB, et al. Endothelial progenitor cells in infantile hemangioma. Blood 2004; 103:1373.
  31. Ritter MR, Dorrell MI, Edmonds J, et al. Insulin-like growth factor 2 and potential regulators of hemangioma growth and involution identified by large-scale expression analysis. Proc Natl Acad Sci U S A 2002; 99:7455.
  32. Takahashi K, Mulliken JB, Kozakewich HP, et al. Cellular markers that distinguish the phases of hemangioma during infancy and childhood. J Clin Invest 1994; 93:2357.
  33. Tan ST, Velickovic M, Ruger BM, Davis PF. Cellular and extracellular markers of hemangioma. Plast Reconstr Surg 2000; 106:529.
  34. Jinnin M, Medici D, Park L, et al. Suppressed NFAT-dependent VEGFR1 expression and constitutive VEGFR2 signaling in infantile hemangioma. Nat Med 2008; 14:1236.
  35. Claesson-Welsh L. Healing hemangiomas. Nat Med 2008; 14:1147.
  36. Chang E, Boyd A, Nelson CC, et al. Successful treatment of infantile hemangiomas with interferon-alpha-2b. J Pediatr Hematol Oncol 1997; 19:237.
  37. Razon MJ, Kräling BM, Mulliken JB, Bischoff J. Increased apoptosis coincides with onset of involution in infantile hemangioma. Microcirculation 1998; 5:189.
  38. Ritter MR, Moreno SK, Dorrell MI, et al. Identifying potential regulators of infantile hemangioma progression through large-scale expression analysis: a possible role for the immune system and indoleamine 2,3 dioxygenase (IDO) during involution. Lymphat Res Biol 2003; 1:291.
  39. Metry DW, Hebert AA. Benign cutaneous vascular tumors of infancy: when to worry, what to do. Arch Dermatol 2000; 136:905.
  40. Frieden IJ, Eichenfield LF, Esterly NB, et al. Guidelines of care for hemangiomas of infancy. American Academy of Dermatology Guidelines/Outcomes Committee. J Am Acad Dermatol 1997; 37:631.
  41. Hand JL, Frieden IJ. Vascular birthmarks of infancy: resolving nosologic confusion. Am J Med Genet 2002; 108:257.
  42. Fishman SJ, Mulliken JB. Hemangiomas and vascular malformations of infancy and childhood. Pediatr Clin North Am 1993; 40:1177.
  43. Martinez-Perez D, Fein NA, Boon LM, Mulliken JB. Not all hemangiomas look like strawberries: uncommon presentations of the most common tumor of infancy. Pediatr Dermatol 1995; 12:1.
  44. Enjolras O, Mulliken JB. The current management of vascular birthmarks. Pediatr Dermatol 1993; 10:311.
  45. Chang LC, Haggstrom AN, Drolet BA, et al. Growth characteristics of infantile hemangiomas: implications for management. Pediatrics 2008; 122:360.
  46. Tollefson MM, Frieden IJ. Early growth of infantile hemangiomas: what parents' photographs tell us. Pediatrics 2012; 130:e314.
  47. Maguiness SM, Hoffman WY, McCalmont TH, Frieden IJ. Early white discoloration of infantile hemangioma: a sign of impending ulceration. Arch Dermatol 2010; 146:1235.
  48. Suh KY, Frieden IJ. Infantile hemangiomas with minimal or arrested growth: a retrospective case series. Arch Dermatol 2010; 146:971.
  49. Haggstrom AN, Lammer EJ, Schneider RA, et al. Patterns of infantile hemangiomas: new clues to hemangioma pathogenesis and embryonic facial development. Pediatrics 2006; 117:698.
  50. Waner M, North PE, Scherer KA, et al. The nonrandom distribution of facial hemangiomas. Arch Dermatol 2003; 139:869.
  51. Metry DW, Garzon MC, Drolet BA, et al. PHACE syndrome: current knowledge, future directions. Pediatr Dermatol 2009; 26:381.
  52. PHACE Association. Online Mendelian Inheritance in Man (OMIM) website. OMIM entry #606519. www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=OMIM (Accessed on January 18, 2006).
  53. Frieden IJ, Reese V, Cohen D. PHACE syndrome. The association of posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities. Arch Dermatol 1996; 132:307.
  54. Metry D, Heyer G, Hess C, et al. Consensus Statement on Diagnostic Criteria for PHACE Syndrome. Pediatrics 2009; 124:1447.
  55. 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.
  56. Opitz JM, Gilbert EF. CNS anomalies and the midline as a "developmental field". Am J Med Genet 1982; 12:443.
  57. Bhattacharya JJ, Luo CB, Alvarez H, et al. PHACES syndrome: a review of eight previously unreported cases with late arterial occlusions. Neuroradiology 2004; 46:227.
  58. Metry DW, Dowd CF, Barkovich AJ, Frieden IJ. The many faces of PHACE syndrome. J Pediatr 2001; 139:117.
  59. Haggstrom AN, Garzon MC, Baselga E, et al. Risk for PHACE syndrome in infants with large facial hemangiomas. Pediatrics 2010; 126:e418.
  60. Pascual-Castroviejo I. Vascular and nonvascular intracranial malformation associated with external capillary hemangiomas. Neuroradiology 1978; 16:82.
  61. Pascual-Castroviejo I, Velez A, Pascual-Pascual SI, et al. Dandy-Walker malformation: analysis of 38 cases. Childs Nerv Syst 1991; 7:88.
  62. Poindexter G, Metry DW, Barkovich AJ, Frieden IJ. PHACE syndrome with intracerebral hemangiomas, heterotopia, and endocrine dysfunction. Pediatr Neurol 2007; 36:402.
  63. Burrows PE, Robertson RL, Mulliken JB, et al. Cerebral vasculopathy and neurologic sequelae in infants with cervicofacial hemangioma: report of eight patients. Radiology 1998; 207:601.
  64. Drolet BA, Dohil M, Golomb MR, et al. Early stroke and cerebral vasculopathy in children with facial hemangiomas and PHACE association. Pediatrics 2006; 117:959.
  65. Heyer GL, Dowling MM, Licht DJ, et al. The cerebral vasculopathy of PHACES syndrome. Stroke 2008; 39:308.
  66. Hess CP, Fullerton HJ, Metry DW, et al. Cervical and intracranial arterial anomalies in 70 patients with PHACE syndrome. AJNR Am J Neuroradiol 2010; 31:1980.
  67. Reese V, Frieden IJ, Paller AS, et al. Association of facial hemangiomas with Dandy-Walker and other posterior fossa malformations. J Pediatr 1993; 122:379.
  68. Duffy KJ, Runge-Samuelson C, Bayer ML, et al. Association of hearing loss with PHACE syndrome. Arch Dermatol 2010; 146:1391.
  69. McAtee-Smith J, Hebert AA, Rapini RP, Goldberg NS. Skin lesions of the spinal axis and spinal dysraphism. Fifteen cases and a review of the literature. Arch Pediatr Adolesc Med 1994; 148:740.
  70. Goldberg NS, Hebert AA, Esterly NB. Sacral hemangiomas and multiple congenital abnormalities. Arch Dermatol 1986; 122:684.
  71. Albright AL, Gartner JC, Wiener ES. Lumbar cutaneous hemangiomas as indicators of tethered spinal cords. Pediatrics 1989; 83:977.
  72. Tubbs RS, Wellons JC 3rd, Iskandar BJ, Oakes WJ. Isolated flat capillary midline lumbosacral hemangiomas as indicators of occult spinal dysraphism. J Neurosurg 2004; 100:86.
  73. Iacobas I, Burrows PE, Frieden IJ, et al. LUMBAR: association between cutaneous infantile hemangiomas of the lower body and regional congenital anomalies. J Pediatr 2010; 157:795.
  74. Girard C, Bigorre M, Guillot B, Bessis D. PELVIS Syndrome. Arch Dermatol 2006; 142:884.
  75. Stockman A, Boralevi F, Taïeb A, Léauté-Labrèze C. SACRAL syndrome: spinal dysraphism, anogenital, cutaneous, renal and urologic anomalies, associated with an angioma of lumbosacral localization. Dermatology 2007; 214:40.
  76. Drolet BA, Chamlin SL, Garzon MC, et al. Prospective study of spinal anomalies in children with infantile hemangiomas of the lumbosacral skin. J Pediatr 2010; 157:789.
  77. Weitz NA, Bayer ML, Baselga E, et al. The "biker-glove" pattern of segmental infantile hemangiomas on the hands and feet. J Am Acad Dermatol 2014; 71:542.
  78. Metry DW, Hawrot A, Altman C, Frieden IJ. Association of solitary, segmental hemangiomas of the skin with visceral hemangiomatosis. Arch Dermatol 2004; 140:591.
  79. Christison-Lagay ER, Burrows PE, Alomari A, et al. Hepatic hemangiomas: subtype classification and development of a clinical practice algorithm and registry. J Pediatr Surg 2007; 42:62.
  80. Lopriore E, Markhorst DG. Diffuse neonatal haemangiomatosis: new views on diagnostic criteria and prognosis. Acta Paediatr 1999; 88:93.
  81. Boon LM, Burrows PE, Paltiel HJ, et al. Hepatic vascular anomalies in infancy: a twenty-seven-year experience. J Pediatr 1996; 129:346.
  82. Frieden IJ, Haggstrom AN, Drolet BA, et al. Infantile hemangiomas: current knowledge, future directions. Proceedings of a research workshop on infantile hemangiomas, April 7-9, 2005, Bethesda, Maryland, USA. Pediatr Dermatol 2005; 22:383.
  83. Chamlin SL, Haggstrom AN, Drolet BA, et al. Multicenter prospective study of ulcerated hemangiomas. J Pediatr 2007; 151:684.
  84. Kim HJ, Colombo M, Frieden IJ. Ulcerated hemangiomas: clinical characteristics and response to therapy. J Am Acad Dermatol 2001; 44:962.
  85. Orlow SJ, Isakoff MS, Blei F. Increased risk of symptomatic hemangiomas of the airway in association with cutaneous hemangiomas in a "beard" distribution. J Pediatr 1997; 131:643.
  86. Ceisler EJ, Santos L, Blei F. Periocular hemangiomas: what every physician should know. Pediatr Dermatol 2004; 21:1.
  87. Dinehart SM, Kincannon J, Geronemus R. Hemangiomas: evaluation and treatment. Dermatol Surg 2001; 27:475.
  88. Enjolras O, Wassef M, Mazoyer E, et al. Infants with Kasabach-Merritt syndrome do not have "true" hemangiomas. J Pediatr 1997; 130:631.
  89. Osio A, Fraitag S, Hadj-Rabia S, et al. Clinical spectrum of tufted angiomas in childhood: a report of 13 cases and a review of the literature. Arch Dermatol 2010; 146:758.