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Infantile hemangioma - Anogenital in
See also in: Overview,External and Internal Eye
Other Resources UpToDate PubMed

Infantile hemangioma - Anogenital in

See also in: Overview,External and Internal Eye
Contributors: Nnenna Agim MD, Marilyn G. Liang MD, Elena B. Hawryluk MD, PhD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Infantile hemangiomas are the most common benign tumors of infancy, occurring in up to 10% of infants, with up to 50% involving the head and neck. Precursors may be present at birth, evolving into a more classic appearance within the first few months of life. Usually, infantile hemangiomas grow rapidly during the first few weeks of life. This initial proliferative phase typically peaks as early as 2 months of age. This is followed by a steady state lasting months, depending on the hemangioma type, followed by gradual involution over several years, with more than 90% completely involuting by age 10. Although infantile hemangiomas involute with time, residual skin changes such as telangiectasia, scarring, atrophy, and fibrosis persist in up to 30% of lesions after involution is complete.

Ulceration occurs in about 5% of hemangiomas and can occur at any location. Ulcerated hemangiomas are painful regardless of location, often become secondarily infected, and leave a scar. Bleeding and ulceration can incorrectly cause or increase concern for possible physical abuse; however, their characteristic development in early infancy and typical regression should help differentiate them from the former. Ulceration is of particular concern when it involves the periocular, nasal, perioral, or anogenital areas, given regional risk for severe complication. Anogenital ulceration adversely affects diaper changes, urination, and defecation. Only about 1%-2% of hemangiomas involve the genitalia. Such hemangiomas of the urethra, the labia, hymen, vulva, or perihymenal area may raise concern for sexual abuse. When extensive ulceration is present, there may be concern that these represent perineal burns. Knowledge of the clinical appearance and natural course of untreated hemangiomas may help prevent misdiagnosis.

Segmental infantile hemangiomas raise concern for regional abnormalities. If the anogenital area is involved in a segmental fashion, evaluation for associated anomalies associated with the synonymous LUMBAR syndrome (lower body hemangioma, lipoma, or other cutaneous anomalies, urogenital anomalies, myelopathy, bony deformities, anorectal and arterial anomalies, and renal anomalies), SACRAL syndrome (spinal dysraphism, anogenital anomalies, cutaneous anomalies, renal and urologic anomalies, associated with angioma of lumbosacral localization), or PELVIS syndrome (perineal hemangioma, external genitalia malformations, lipomyelomeningocele, vesicorenal abnormalities, imperforate anus, and skin tags) should ensue.

For more information, see OMIM.

Codes

ICD10CM:
D18.01 – Hemangioma of skin and subcutaneous tissue

SNOMEDCT:
83343001 – Infantile hemangioma

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Differential Diagnosis & Pitfalls

  • Congenital hemangioma (CH) – Fully formed vascular tumors at birth; 2 main types are: rapidly involuting congenital hemangiomas (RICH) and noninvoluting congenital hemangiomas (NICH), and variants have been reported. The proliferative phase in these tumors occurs in utero, and tumors do not typically proliferate postnatally in the pattern seen with infantile hemangiomas. RICHs present as exophytic pink to purple vascular plaques, nodules, or tumors, sometimes with ulceration. NICH presents as a bluish patch or plaque overlaid with coarse telangiectasia and a peripheral rim of pallor. Histologically, they are GLUT-1 negative, which helps to differentiate them from other hemangiomas. Partially involuting congenital hemangiomas (PICH) add to this spectrum with features of both RICH and NICH. PICH and NICH have been shown to demonstrate delayed enlargement years after diagnosis. These can also be painful without ulceration as compared to infantile hemangiomas.
  • Capillary malformation or "port-wine stain" – Early superficial or segmental infantile hemangiomas, or their precursor lesions, may be confused with port-wine stains, which are capillary malformations present at birth. They are pink or red patches that persist throughout life and do not have the typical infantile growth phase of hemangiomas. They do, however, proliferate after puberty.
  • Tufted angioma and kaposiform hemangioendothelioma represent a spectrum of vascular tumors that may mimic hemangiomas. They present as deep red, purple, or brown vascular plaques or nodules, sometimes with deep fibrotic consistency. They may be localized or segmental and carry risk for the Kasabach-Merritt platelet sequestration phenomenon.
  • Infantile myofibromatosis – Fibrohistiocytic proliferations characterized by skin-colored to purple, firm to hard or rubbery nodules, single or multiple in presentation. Failure to thrive and spasticity may be present.
  • Venous malformations have a similar blue tint as deep infantile hemangioma, but they do not proliferate rapidly unless there is vascular obstruction. Phleboliths are often seen in venous malformations on ultrasound, and compared to hemangiomas, they demonstrate slow flow.
  • Lymphangiomas may mimic deep infantile hemangiomas.
  • Neuroblastomas can mimic deep infantile hemangiomas, but they blanch when stroked / rubbed.
  • Cystic neurilemmoma
  • Fibrous histiocytoma (see, eg, malignant fibrous histiocytoma)
  • Vascular leiomyoma
  • Kaposi sarcoma
  • Abuse

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References

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Last Reviewed:10/06/2021
Last Updated:10/19/2021
Copyright © 2021 VisualDx®. All rights reserved.
Infantile hemangioma - Anogenital in
See also in: Overview,External and Internal Eye
Infantile hemangioma : Purple color, Red color, Tumor, Unilateral distribution, Vascular plaque
Clinical image of Infantile hemangioma
A deep red nodule on the lower forehead.
Copyright © 2021 VisualDx®. All rights reserved.