Vascular Anomalies

Vascular Anomalies
Arin K. Greene
Jeremy Goss
DEFINITION
  • Vascular anomalies are a group of lesions that can affect any part of the vasculature.
  • Lesions are divided into tumors (with dividing endothelium) and malformations (structural anomalies with minimal endothelial turnover).1
  • Eight types of vascular anomalies (four tumors and four malformations) constitute approximately 95% of lesions.
ANATOMY
  • Vascular anomalies most commonly involve the integument (although they can affect any anatomical structure).
  • The most common morbidity is lowered self-esteem because of a deformity.
  • Lesions involving the head and neck are more likely to be problematic.
NATURAL HISTORY
  • The somatic mutations responsible for most vascular anomalies recently have been identified.
  • Some vascular tumors improve over time (infantile hemangioma, rapidly involuting congenital hemangioma, kaposiform hemangioendothelioma).2
  • Vascular malformations slowly worsen, particularly during adolescence.
  • Following treatment of vascular malformations, recurrence is common and patients often require repeated procedures.
PATIENT HISTORY AND PHYSICAL FINDINGS
  • Ninety percent of vascular anomalies can be diagnosed by history and physical examination.
  • Before considering management, the type of vascular anomaly must be identified because lesions have different natural histories and treatments.3
  • The most common vascular anomalies encountered in clinical practice are (in order of frequency) pyogenic granuloma, infantile hemangioma, and capillary malformation.
  • A handheld Doppler can determine whether the lesion has fast flow, which aids the diagnosis.
  • Lesions with fast flow include infantile hemangioma, congenital hemangioma, kaposiform hemangioendothelioma, and arteriovenous malformation. Slow-flow anomalies are capillary malformation, lymphatic malformation, and venous malformation.
IMAGING
  • Less than 10% of patients require imaging to diagnose their vascular anomaly.
  • If the diagnosis is unclear by history and physical examination, ultrasound is the first-line imaging study because it is easy to perform and does not require sedation in children.
  • If the diagnosis remains uncertain after ultrasonography, then MRI with contrast is obtained.
  • Imaging is not required prior to resection of hemangiomas, pyogenic granulomas, or capillary malformations.
  • Before operative intervention for a venous malformation, lymphatic malformation, or arteriovenous malformation, MRI typically is obtained to determine the extent of disease.
DIFFERENTIAL DIAGNOSIS
  • Fibrosarcoma
  • Neurofibroma
  • Pilomatrixoma
  • Teratoma
NONOPERATIVE MANAGEMENT
  • Nonoperative intervention is the mainstay of treatment for vascular anomalies; lesions are benign and often can be observed.
  • Less than 10% of infantile hemangiomas require treatment. Problematic lesions during the proliferative phase can be managed with topical timolol, corticosteroid injection, prednisone, propranolol, or rarely, resection.
  • Kaposiform hemangioendothelioma is treated with vincristine or sirolimus.
  • First-line intervention for problematic vascular malformations typically is nonsurgical: capillary malformation (pulseddye laser), venous malformation (sclerotherapy), lymphatic malformation (sclerotherapy), arteriovenous malformation (embolization).4,5,6,7
SURGICAL MANAGEMENT
  • Operative treatment of vascular anomalies generally is performed for symptomatic patients who are not candidates or have failed nonoperative interventions.
  • Residual infantile or congenital hemangiomas causing a deformity can only be improved with resection (or pulseddye laser for telangiectasias).
  • The primary treatment of pyogenic granuloma is full-thickness skin excision or cautery (curettage, laser, and cryotherapy have a recurrence rate as high as 50%).
  • Capillary malformation can cause overgrowth of tissues beneath the stain, which can be improved by resection.
  • Operative intervention for venous malformation is reserved for small lesions that may be removed for cure or for a residual deformity following sclerotherapy.
  • Macrocystic lymphatic malformation may have redundant skin following sclerotherapy that can be resected. Microcystic lesions are not amenable to sclerotherapy, and thus, excision often is first-line treatment.
  • Arteriovenous malformations generally are removed if they remain symptomatic following embolization.
Preoperative Planning
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Feb 27, 2020 | Posted by in Pediatric plastic surgery | Comments Off on Vascular Anomalies

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