Infantile Hemangioma

 
Infantile hemangioma is a benign neoplasm that commonly develops in neonates within their first few months of life. These birthmarks are more common in whites, and girls are three to five times more likely than boys to have a hemangioma. Most infantile hemangiomas undergo rapid initial proliferation, with a subsequently plateau in infants aged about 9-10 months; finally, they become involuted. The involuting phase extends from 1 year until 5 to 7 years of age.

   

Photo#1: Typical infantile hemangioma or birthmark in the forehead. Photo#2 and 3 show another infantile hemangioma (birthmark) in the lower lip. Discoloration which has sharp margins is clearly seen. The lesion is soft, but not easily compressible.  

Hemangiomas generally affect the head and neck (80-85%). The trunk and extremities are less commonly involved.

Some hemangiomas are easily noticeable (superficial) usually bright red, flat or raised patchs on the skin, whereas others are deeper and may have healthy overlying skin or only slightly bluish skin discoloration. Hemangiomas are usually firm, warm to the touch and can swell when the baby is upset or sick. Most hemangiomas are easily diagnosed without any additional diagnostic tests (eg, MRI). Deeper hemangiomas or questionable superficial lesions, however, may require imaging studies to confirm the diagnosis and to evaluate their extent. US may be used during the initial assessment or in place of MRI if it is unavailable.

 

 

Photo#1 shows a superficial hemangioma or birthmark, photo#2 shows a large deep hemangioma, the skin is intact. Photo#3 shows a small hemangioma lesion or birthmark in the mid neck area. Infantile hemangiomas are occasionally seen in the extremities. This is a typical hemangioma lesion in the foot (photo#4). 

During the involutional phase, decreasing vascularity, decreasing enhancement, and progressive fibro-fatty replacement of the tumor is present. As the hemangioma involutes, fatty replacement can easily be recognized on MRI, which demonstrate fat signal intensity with some residual prominent vessels. Lesions adjacent to bone may cause a smoothly marginated erosion of the bone. Phleboliths and calcifications are not features of hemangiomas.

Some hemangiomas completely disappear, while others not and may require corrective surgery. During the involution phase, hemangiomas become softer and less tender to the touch.

 
    If the patient is older than 5 years of age, another type of tumoral mass (eg, fibrosarcoma) should be favored in the differential diagnosis, and a biopsy may be required. When a high-flow lesion is noted on noninvasive studies, a biopsy should be performed carefully because the risk of significant bleeding is high. Angiography may be necessary for embolization of associated spontaneous hemorrhage.

               

Image#1 is an MRI image obtained following intravenous contrast administration. The lesion enhances (bright) and there are vessels within the periphery of the lesion, as well as near the lesion, which is typical appearance of infantile hemangioma on MR. Image#2 is also a post-contrast MRI with fat suppression clearly outlines the margins of this tumoral mass (infantile hemangioma)

On angiograms, hemangiomas appear as well-circumscribed masses that have a lobular architecture with intense and persistent tissue staining. Prominent and early draining veins may be present. Less intense tissue staining is evident with involuting lesions.

The differential diagnosis includes other neonatal and infantile tumors, particularly soft-tissue sarcomas. In general, hemangiomas are characterized by discrete margins, relatively homogenous signal intensity, and homogenous contrast enhancement, whereas sarcomas tend to be heterogeneous.

 
     

MRI examination of the head: 1st image is a sagittal T1 weighted MR without contrast demonstrating a mass lesion attached to the bony calvarium.  There are small tubular hypointensities (signal voids) in and around the mass lesion representing arterial feeders. These signal voids typically seen within the periphery of the mass, as well as in the surrounding tissues. This tumor demonstrates relatively homogeneous contrast enhancement (except for arterial signal voids) (2nd image). 3rd image is a coronal T2 weighted image. No evidence of intracranial abnormalities. Some patients with relatively large head and neck infantile hemangiomas may have associated intracranial abnormalities; therefore, MRI examination of the head is necessary in selected cases. 

 

Diagnosis & Treatment

   Diagnostic Workup ?
   Treatment / Management ?
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Abnormalities associated with hemangiomas? 

  PHACE sydrome: Group of findings including posterior fossa malformations, hemangioma, arterial anomalies, coarctation of the aorta and cardiac anomalies, eye anaomalies and sternal cleft. Affected patients (nearly always girls) usually have one or two of the associated abnormalites rather than all components.

Spinal Abnormalities: Association with spinal abnormalities in hemangiomas overlying the lumbosacral spine. The most common malformation is a tethered cord, often accompanied by an occult lipomeningocele. Rarely, genitourinary abnormalities may be seen. Please see Case Studies (Case#17)

Hypothroidism: Hypothyroidism is a potential complication of hemangiomas, particularly for hepatic hemangiomatosis. The mechanism is believed to be tumoral production of type 3 idothyronine deiodinase, causing peripheral inactivation of thyroid hormone. 

Recent Literature

  • This study evaluating 62 patients with an initial diagnosis of infantile hepatic hemangioma concluded that MRI features of hepatic hemangiomas are typical in most patients and certain imaging findings are predictive of the clinical course. MRI is the technique of choice in diagnosing infantile hepatic masses >>> abstract
  • Case presentation to report a case of PHACE syndrome associated with congenital glaucoma. This report concluded that Ophthalmologists who examine children with large facial hemangiomas should consider PHACE syndrome in the differential diagnosis and should obtain appropriate CNS imaging studies and cardiac evaluation when the diagnosis is suspected. Congenital glaucoma should be added to the list of PHACE-associated ocular anomalies >>> abstract
  • Retrospective study evaluating 100 consecutive patients wit parotid hemangioma concluded that infantile hemangioma in the parotid gland responds to pharmacological treatment in a similar manner as hemangioma in other locations. Drug therapy was effective in the majority of infants with parotid hemangioma, whether given because the tumor was large, deforming, ulcerated, or involved nearby structures with functional consequences >>> abstract

 
 
Imaging of Hemangiomas: Where, When, Which and Why?
By Patricia Burrows, MD - Infantile hemangiomas: Current Knowledge, Future Directions, Bethesda, 2005
While numerous imaging modalities can be used to investigate hemangiomas, clinical experience has shown that ultrasonography, with Doppler interrogation, and MR imaging are two most effective modalities. The choice will be based on the reason for studying the patient and available modalities in each institution.

If the study is indicated to distinguish between a deep hemangioma and other entities, ultrasonography by a radiologist familiar with vascular anomalies is the most noninvasive and efficient method. Typically, sonography imaging will confirm the presence of a solid mass containing high-flow vessels, and supplied and drained by high-flow vessels. Limitations include operator dependence and potential inability to predict extent and presence or absence of associated malformative anomalies. Doppler interrogation of the feeding and intralesional vessels demonstrates low resistance waveforms, and draining veins show turbulent or arterialized waveforms, consistent with microshunting.

MR imaging is the preferred study for patients who need documentation of the extent of disease or determination of the presence of absence of associated anomalies prior to the institution of pharmacotherapy. To best determine the nature of the lesion, the following sequences are recommended: T1W sequence without gadolinium, T1W with fat saturation post gadolinium, T2W with fat saturation or Inversion Recovery and gradient flow sequence or MR angiogram. The field of view should include not only the obvious lesion but tissues that could be involved by contiguity with the hemangioma, or associated malformed states. 

While CT is inferior to MRI in demonstrating the morphology of hemangioma, modern multi-detector scanners can produce high quality examinations without requiring sedation, because of ultrashort scan times. In certain locations, CT angiography may be more effective than MR angiography. In general, institutional availability may dictate the choice of examination. CT evaluation should be carried out without and with intravenous contrast administration. Angiography is performed only when endovascular therapy is necessary. Embolization is indicated rarely, generally to control high output cardiac failure or bleeding that is not responsive to pharmacotherapy.

Where: Hemangioma of the Head & Neck. When: Problematic hemangiomas that do or may require pharmacological therapy, including hemangiomas of the orbit and airway or involving high risk "patterns" such as the beard or regional distribution. Which: MR imaging should include the affected area (e.g., orbit, face, neck) some of the adjacent soft tissues (e.g., neck and mediastinum) and the brain.
Most associated arterial abnormalities can be identified on routine MR imaging sequences. Additional detail is provided by MR and CT angiography of the affected area, but these studies are not necessary for initial screening. Why: Brain imaging should be carried out to detect intracranial hemangiomas as well as associated malformative anomalies including absence, stenosis, or aneurysm formation of the cervical and intracranial arteries, cerebral infarction, and cerebral defects such as posterior fossa cystic anomalies, absent corpus collosum, cortical dysplasia and hydrocephalus. Hemangiomas in a beard distribution can involve the airway much more extensively than is clinically evident. 

Where: Cutaneous hemangioma overlying the spine. When: As soon as it is recognized. Which: MRI of the spine without and with intravenous gadolinium is optimal. In the fist three months of life, sonography can be used as a screen to rule out intraspinal pathology. Why: Hemangiomas overlying the spine can be associated with intraspinal extension and dysraphic lesions including spinal dermoid, tethered cord and lipomyeloschisis.

Where: Multiple (more than 4) cutaneous hemangiomas. When: When skin lesions appear to be enlarging or increasing in number. Which: Ultrasonography of the liver and brain is effective for screening patients with multiple cutaneous hemangiomas. When small hepatic hemangiomas without marked shunting are detected, monthly re-evaluation with abdominal ultrasonography is necessary until the lesions have stabilized or started to regress. However, in the case of extensive, high flow or enlarging hepatic hemangiomas, MRI should be obtained to document the extent of disease. Why: The infant with multiple cutaneous hemangiomas (disseminated hemangiomatosis) is at risk of having hepatic or cerebral involvement. MR imaging without/with gadolinium is the most effective imaging method to distinguish hemangiomas from other hepatic lesions. Multifocal hemangiomas have characteristic, consistent imaging findings. They appear as spherical, T2 hyperintense, enhancing masses, with flow voids. Contrast enhancement occurs centripetally. Involvement of adjacent organs (e.g., bowel) may also be identified. The presence of significant shunting can be predicted by the degree of vascularity (e.g., enlargement of the hepatic and mesenteric feeding arteries and the hepatic veins correlates with high flow and clinical signs of a high output cardiac state). Abdominal compartment syndrome, resulting from massive replacement of the liver parenchyma and compression of venous return form the lower body can be readily predicted. Focal lesions with large vascular structures may simulate arteriovenous malformations; the presence of a solid parenchymal component, sometimes not easily seen by ultrasonography, distinguishes hepatic hemangioma from AVM.

Where: Extensive perineal hemangioma in the presence of urogenital or anal anomalies. When: As soon as is feasible. Which: MRI of the pelvis and spine. Why: Perineal hemangiomas can be associated with structural ano-genito-urinary anomalies, such as imperforate anus, cloaca and renal ectopia. In this situation, intraspinal hemangioma with or without spinal dysraphism is relatively common. Arterial anomalies have also been seen. 

Where: Anywhere. When: In the presence of severe thrombocytopenia. Which: MRI with gadolinium. Why: The lesion most likely represents a kaposiform hemangioendothelioma rather than an infantile hemangioma.

Where: Focal cutaneous lesion with extremely high flow suggesting arteriovenous malformation. When: Symptoms warrant therapy. Which: MRI with MR angiography or sonography with Doppler. Why: Most focal high flow neonatal masses represent rapidly involuting congenital hemangioma (RICH) rather than arteriovenous malformation. MR imaging with MRA or sonography with Doppler are both useful to identify the focal parenchymal mass that distinguishes RICH from AVM. The diagnosis of RICH may obviate the need for invasive procedures to control the high output cardiac state (e.g., embolization or resection).

When: The hemangioma does not require imaging. Where: imaging is not necessary for cutaneous hemangiomas with typical clinical appearance and behavior that do not require pharmacological therapy.

When: The suspected hemangioma that presents after six months of age or has atypical clinical appearance or imaging features.  What: Requires biopsy or excision. Why: The differential diagnosis of atypical, hemangioma-like mass includes KHE, other vascular tumors (e.g., angiosarcoma, hemangiopericytoma, other hemangioandotheliomas), rhabdomyosarcoma, teratoma, infantile fibrosarcoma and other developmental lesions.      

 

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