# Vascular Anomalies of the Head and Neck in Children

## Introduction

Vascular anomalies of the head and neck represent a diverse group of lesions that are among the most common congenital abnormalities encountered in pediatric otolaryngology. The **International Society for the Study of Vascular Anomalies (ISSVA)** classification system divides these lesions into two major categories: **vascular tumors** and **vascular malformations**. Accurate diagnosis is essential, as management strategies differ significantly between categories.

## Classification

### ISSVA Classification System

**Vascular tumors**: Lesions characterized by endothelial cell proliferation. **Infantile hemangioma** (most common vascular tumor of infancy). Congenital hemangioma (rapidly involuting [RICH] and non-involuting [NICH]). Kaposiform hemangioendothelioma. Tufted angioma. **Vascular malformations**: Structural anomalies of blood vessels without proliferation. **Capillary malformations** (port-wine stains). **Venous malformations**. **Lymphatic malformations** (macrocystic, microcystic, mixed). **Arteriovenous malformations (AVMs)**. Combined malformations.

### Key Distinguishing Features

| Feature | Vascular Tumors | Vascular Malformations |
|---------|----------------|----------------------|
| Present at birth | No (appear in first weeks) | Always present at birth |
| Growth pattern | Proliferative phase then involution | Proportional with child; never involute |
| GLUT-1 | Positive (infantile hemangioma) | Negative |
| Endothelial proliferation | Yes | No (structural anomaly) |
| Sex predominance | Female (3:1) | Equal |

Vascular tumors are **not present at birth** (or minimally so) and undergo a proliferative phase. Vascular malformations are **always present at birth**, grow proportionally with the child, and never involute spontaneously. **GLUT-1 positivity** is pathognomonic for infantile hemangioma.

## Infantile Hemangiomas

### Natural History

Appear within the first weeks of life. **Proliferative phase**: Rapid growth during months 1-9. **Involuting phase**: Gradual regression over years 1-5. **Involuted phase**: Completed by age 5-10; may leave residual fibrofatty tissue or telangiectasia. Up to **60%** of head and neck hemangiomas occur in the parotid region.

### Indications for Treatment

Airway compromise (subglottic hemangioma), Visual obstruction or amblyopia risk, Ulceration, bleeding, or infection, Significant cosmetic deformity.

### Medical Management

**Propranolol** is first-line systemic therapy (dose: 2-3 mg/kg/day divided BID-TID). Topical **timolol** for superficial, small lesions. Systemic corticosteroids as second-line therapy. Monitor heart rate, blood pressure, and blood glucose during propranolol initiation.

![Infantile hemangioma of the parotid region during proliferative phase](images/infantile-hemangioma-parotid.jpg)

## Vascular Malformations

### Lymphatic Malformations

Previously called **cystic hygromas** or **lymphangiomas**. Most common in the posterior triangle of the neck. **Macrocystic** lesions respond well to sclerotherapy (OK-432, doxycycline, bleomycin). **Microcystic** lesions are more difficult to treat and often require surgical excision. MRI is the imaging modality of choice; T2-weighted images show hyperintense cystic spaces.

### Venous Malformations

Soft, compressible, blue-tinged masses that enlarge with Valsalva or dependent positioning. Phleboliths may be visible on imaging. Treatment options include **sclerotherapy** (sodium tetradecyl sulfate, ethanol) and surgical excision. Frequently recur after incomplete excision.

### Arteriovenous Malformations

High-flow lesions with direct arteriovenous shunting. Staged according to the **Schobinger classification** (stages I-IV). May remain quiescent for years then expand rapidly after trauma, puberty, or pregnancy. Treatment requires **preoperative embolization** followed by complete surgical resection within 24-48 hours.

![MRI T2-weighted image demonstrating a macrocystic lymphatic malformation of the neck](images/lymphatic-malformation-mri.jpg)

## Diagnostic Workup

**Ultrasound with Doppler**: First-line imaging to distinguish high-flow from low-flow lesions. **MRI with gadolinium**: Gold standard for characterizing extent and tissue planes. **CT angiography**: Useful for AVMs to delineate feeding vessels. **Biopsy**: Rarely needed; reserved for atypical presentations or suspected malignancy. **GLUT-1 immunostaining**: Differentiates infantile hemangioma from other vascular tumors.

## Surgical Considerations

Complete excision is curative but not always feasible due to infiltrative nature. **Staged excision** may be necessary for extensive malformations. Preoperative embolization reduces intraoperative blood loss for high-flow lesions. Airway hemangiomas may require **open excision or CO2 laser ablation** if refractory to propranolol. Multidisciplinary team approach (otolaryngology, interventional radiology, dermatology, plastic surgery) is recommended.

![Intraoperative view of surgical excision of a venous malformation](images/venous-malformation-excision.jpg)

## Key Clinical Pearls

Always classify vascular anomalies using the ISSVA system; avoid outdated terminology such as "cavernous hemangioma". Propranolol has revolutionized the management of infantile hemangiomas and should be initiated early during the proliferative phase. GLUT-1 positivity is the single most reliable marker distinguishing infantile hemangioma from vascular malformations. Segmental facial hemangiomas warrant evaluation for **PHACE syndrome** (posterior fossa anomalies, hemangioma, arterial anomalies, coarctation, eye abnormalities). Macrocystic lymphatic malformations respond well to sclerotherapy; microcystic lesions often require surgery.

## 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. Wassef M, Blei F, Adams D, et al. Vascular anomalies classification: recommendations from the International Society for the Study of Vascular Anomalies. *Pediatrics*. 2015;136(1):e203-e214.
3. Perkins JA, Manning SC, Tempero RM, et al. Lymphatic malformations: review of current treatment. *Otolaryngol Head Neck Surg*. 2010;142(6):795-803.
4. Richter GT, Friedman AB. Hemangiomas and vascular malformations: current theory and management. *Int J Pediatr*. 2012;2012:645678.