Vascular Lesions including Hemangiomas and Vascular Malformations

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Vascular Lesions including Hemangiomas and Vascular Malformations

Pediatric Otolaryngology
Author Credentials 

William O. Collins, MD
Associate Professor
Department of Otolaryngology
University of Florida College of Medicine
Gainesville, FL

Module Summary

Vascular lesions of the head and neck in pediatric patients can look very similar upon presentation. Accurate diagnosis is critical, as the etiology, histology, and clinical course of these lesions can vary considerably.  Proper recognition of the type of lesion, can lead the clinician to offer appropriate medical and surgical options, and avoid unnecessary therapeutic interventions which may not be efficacious and may even be harmful. Complications of treatment must be identified, as well as complications in untreated vascular lesions.

Module Learning Objectives 
  1. Differentiate between hemangiomas and vascular malformations.
  2. Describe the potential complications caused by these lesions.
  3. Review the medical and surgical treatment options.

Embryology

Learning Objectives 

To understand the embryologic derivations of hemangiomas and vascular malformations.

References 
  1. Adams DM, Lucky AW. Cervicofacial vascular anomalies. I. Hemangiomas and other benign vascular tumors. Semin Pediatr Surg. 2006 May;15(2):124-32
  2. Elluru RG, Azizkhan RG. Cervicofacial vascular anomalies. II. Vascular malformations. Semin Pediatr Surg. 2006 May;15(2):133-9.
  3. North PE, Waner W, Mizeracki A, et al. A unique microvascular phenotype shared by juvenile hemangiomas and human placenta. Arch Dermatol 2001;137:559-70.
  4. Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg. 1982;69:412-22.
  5. Bauland CG, van Steensel MA, Steijlen PM, et al. The pathogenesis of hemangiomas: a review. Plast Reconstr Surg. 2006;117:29e-35e.

Anatomy

Learning Objectives 

To understand the anatomical distribution of head and neck hemangiomas & vascular malformations.

References 
  1. Adams DM, Lucky AW. Cervicofacial vascular anomalies. I. Hemangiomas and other benign vascular tumors. Semin Pediatr Surg. 2006 May;15(2):124-32
  2. Elluru RG, Azizkhan RG. Cervicofacial vascular anomalies. II. Vascular malformations. Semin Pediatr Surg. 2006 May;15(2):133-9.
  3. Wendelin G, Kitzmuller E, Salzer-Muhar U. PHACES: a neurocutaneous syndrome with anomalies of the aorta and supraaortic vessels. Cardiol Young. 2004;14:206-9.
  4. Greene AK, Rogers GF, Mulliken JB. Management of parotid hemangioma in 100 children. Plast Reconstr Surg. 2004;113:53-60.
  5. 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;131:643-6.

Pathogenesis

Learning Objectives 

To understand the etiology of common head and neck vascular lesions.

References 
  1. North PE, Waner W, Mizeracki A, et al. A unique microvascular phenotype shared by juvenile hemangiomas and human placenta. Arch Dermatol. 2001;137:559-70.
  2. Adams DM, Lucky AW. Cervicofacial vascular anomalies. I. Hemangiomas and other benign vascular tumors. Semin Pediatr Surg. 2006 May;15(2):124-32
  3. Elluru RG, Azizkhan RG. Cervicofacial vascular anomalies. II. Vascular malformations. Semin Pediatr Surg. 2006 May;15(2):133-9.
  4. North PE, Waner M, Mizeracki A, et al. GLUT1: a newly discovered immunohistochemical marker for juvenile hemangiomas. Hum Pathol. 2000;31:11-22.
  5. Bauland CG, van Steensel MA, Steijlen PM, et al. The pathogenesis of hemangiomas: a review. Plast Reconstr Surg. 2006;117:29e-35e.

Incidence

Learning Objectives 

To describe the incidence of head and neck vascular lesions:

  1. Hemangiomas
  2. Vascular malformations
References 
  1. Adams DM, Lucky AW. Cervicofacial vascular anomalies. I. Hemangiomas and other benign vascular tumors. Semin Pediatr Surg. 2006 May;15(2):124-32
  2. Elluru RG, Azizkhan RG. Cervicofacial vascular anomalies. II. Vascular malformations. Semin Pediatr Surg. 2006 May;15(2):133-9.
  3. Drolet BA, Esterly NB, Frieden IJ. Hemangiomas in children. N Engl J Med. 1999;341:173-81.

Genetics

Learning Objectives 

To describe genetic markers for development of head and neck vascular lesions.

References 
  1. North PE, Waner W, Mizeracki A, et al. A unique microvascular phenotype shared by juvenile hemangiomas and human placenta. Arch Dermatol. 2001;137:559-70.
  2. Chang J, Most D, Bresnick S, et al. Proliferative hemangiomas: analysis of cytokine gene expression and angiogenesis. Plast Reconstr Surg. 1999;103:1-9; discussion 10.
  3. Blei F, Walter J, Orlow SJ, et al. Familial segregation of hemangiomas and vascular malformations as an autosomal dominant trait. Arch Dermatol 1998;134:718-22.

Patient Evaluation

Learning Objectives 

To describe the steps in a thorough history and physical examination in a pediatric patient with a vascular lesion of the head and neck region.

References 
  1. Adams DM, Lucky AW. Cervicofacial vascular anomalies. I. Hemangiomas and other benign vascular tumors. Semin Pediatr Surg. 2006 May;15(2):124-32
  2. Elluru RG, Azizkhan RG. Cervicofacial vascular anomalies. II. Vascular malformations. Semin Pediatr Surg. 2006 May;15(2):133-9.
  3. Chang LC, Haggstrom AN, Drolet BA, et al. Growth characteristics of infantile hemangiomas: implications for management. Pediatrics. 2008;122:360-367.

Imaging

Learning Objectives 

To identify the best imaging techniques for pediatric patients with head and neck vascular lesions.

  1. Ultrasound
  2. CT scanning
  3. MRI imaging
References 
  1. Bittles MA, Sidhu MK, Sze RW, et al. Multidetector CT angiography of pediatric vascular malformations and hemangiomas: utility of 3-D reformatting in differential diagnosis. Pediatr Radiol. 2005;35:1100-6.

Pathology

Learning Objectives 

To recognize the predominant cell types and histologic architecture in:

  1. Hemangiomas
  2. Vascular malformations
References 
  1. Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg. 1982;69:412-22.

Treatment

Learning Objectives 

To understand the indications and implications of both medical and surgical therapeutic options for head and neck vascular lesions, including when to treat vs. continuing with benign observation.

References 
  1. Adams DM, Lucky AW. Cervicofacial vascular anomalies. I. Hemangiomas and other benign vascular tumors. Semin Pediatr Surg. 2006 May;15(2):124-32
  2. Elluru RG, Azizkhan RG. Cervicofacial vascular anomalies. II. Vascular malformations. Semin Pediatr Surg. 2006 May;15(2):133-9.
  3. Morgan P, Keller R, Patel K. Evidence-Based Management of Vascular Malformations. Facial Plastic Surg. 2016;32(2):162-176.

Medical Therapies

Learning Objectives 

To describe medical treatment options for:

  1. Hemangiomas
  2. Vascular malformations
References 
  1. Drolet BA, Frommelt PC, Chamlin SL, et al. Initiation and use of propranolol for infantile hemangioma: report of a consensus conference. Pediatrics. 2013;131(1):128-140.
  2. Buckmiller LM, Munson PD, Dyamenahalli U, Dai Y, Richter GT. Propranolol for infantile hemangiomas: early experience at a tertiary vascular anomalies center. Laryngoscope. 2010;12;676-681.
  3. Tan ST, Itinteang T, Leadbitter P. Low-dose propranolol for infantile hemangioma. J Plast Reconstr Aesthet Surg. 2011 Mar;64(3):292-9.
  4. Perkins JA, Manning SC, Tempero RM, Cunningham MJ, Edmonds JL, Hoffer FA, Egbert MA. Lymphatic Malformations: Review of Current Treatment.  Otolaryngol Head Neck Surg. 2010;142:795-801.
  5. Smith MC, Zimmerman B, Burke DK, Bauman NM, Soto Y, Smith RJH. Efficacy and Safety of OK-432 Immunotherapy of Lymphatic Malformations. Laryngoscope. 2009;199:107-115.
  6. Leaute-Labreze C, et al. A Randomized, Controlled Trial of Oral Propranolol in Infantile Hemangioma. NEJM. 2015;372:735-746.

Pharmacology

Learning Objectives 

To describe the pharmacologic basis for common medical treatments for head and neck vascular lesions in children.

References 
  1. Chiu HH, Chen RL, Lin KH, et al. Recombinant alpha -interferon treatment of intracranial hemangioma and Kasabach-Merritt syndrome in an infant with cytomegalovirus. J Formos Med Assoc. 1995;94:261-6.
  2.  Michaud AP, Bauman NM, Burke DK, et al. Spastic diplegia and other motor disturbances in infants receiving interferon-alpha. Laryngoscope. 2004;114:1231-6.
  3. Perez J, Pardo J, Gomez C. Vincristine--an effective treatment of corticoid-resistant life-threatening infantile hemangiomas. Acta Oncol. 2002;41:197-9.
  4. Drolet BA, Frommelt PC, Chamlin SL, et al. Initiation and use of propranolol for infantile hemangioma: report of a consensus conference. Pediatrics. 2013;131(1):128-140.
  5. Buckmiller LM, Munson PD, Dyamenahalli U, Dai Y, Richter GT. Propranolol for infantile hemangiomas: early experience at a tertiary vascular anomalies center. Laryngoscope. 2010;12:676-681.
  6. Tan ST, Itinteang T, Leadbitter P. Low-dose propranolol for infantile hemangioma. J Plast Reconstr Aesthet Surg. 2011 Mar;64(3):292-9.

Surgical Therapies

Learning Objectives 

To determine appropriate timing and type of procedures needed to surgically treat:

  1. Hemangiomas
  2. Vascular malformations
References 
  1. Arneja JS, Gosain AK. Vascular Malformations. Plast Reconstr Surg. 2008 Apr;121(4):195e-206e.
  2. Jackson IT, Carreño R, Potparic Z, Hussain K. Hemangiomas, vascular malformations, and lymphovenous malformations: classification and methods of treatment. Plast Reconstr Surg. 1993 Jun;91(7):1216-30.
  3.  Mulliken JB, Rogers GF, Marler JJ. Circular excision of hemangioma and purse-string closure: the smallest possible scar. Plast Reconstr Surg. 2002;109:1544-54;discussion 55.

Staging

Learning Objectives 

To describe common classification schemes for vascular lesions.

References 
  1. Jackson IT, Carreño R, Potparic Z, Hussain K. Hemangiomas, vascular malformations, and lymphovenous malformations: classification and methods of treatment. Plast Reconstr Surg. 1993 Jun;91(7):1216-30.
  2. Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg. 1982;69:412-22.
  3. Tekes A, Koshy J, Kalayci TO, et al. SE Mitchell Vascular Anomalies Flow Chart (SEMVAFC): a visual pathway combining clinical and imaging findings for classification of soft-tissue vascular anomalies. Clin Radiol. 2014;69(5):443-457.

Case Studies

  1. A newborn infant is born with a large lateral neck mass.  There is no immediate respiratory distress, and the child is breathing comfortably.  What characteristics of the history and physical examination are most helpful in establishing a diagnosis?  Which imaging modality would be recommended for initial evaluation?
  2. A 3 month old female develops a progressive enlarging right cheek mass, with some red discoloration of the overlying skin. She also has had intermittent cough, described as barking in quality. What imaging modality is suggested? If imaging is suggestive of a hemangioma, what medical therapeutic options are available? Are any other diagnostic interventions indicated?

Complications

Learning Objectives 

To recognize potential complications of both treated and untreated:

  1. Hemangiomas
  2. Vascular malformations
References 
  1. Adams DM, Lucky AW. Cervicofacial vascular anomalies. I. Hemangiomas and other benign vascular tumors. Semin Pediatr Surg. 2006 May;15(2):124-32
  2. Elluru RG, Azizkhan RG. Cervicofacial vascular anomalies. II. Vascular malformations. Semin Pediatr Surg. 2006 May;15(2):133-9.

Review

Review Questions 
  1. Describe the histologic differences between hemangiomas and vascular malformations.
  2. What are the first line medical therapies for infants with hemangiomas?
  3. Describe the classification of lymphatic malformations, based on radiographic findings and anatomic locations.
  4. What are the treatment options for macrocystic lymphatic malformations?  Microcystic lymphatic malformations?
Resources 

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