Amanda Schlak, MD

Dr Schlak is chief resident, Pediatrics, Children’s Hospital of Richmond at Virginia Commonwealth University (VCU), Richmond, Virginia. 

	A healthy newborn girl returns for a follow-up visit at age 7 days, and she is exhibiting multiple disseminated red macules and papules of varying sizes. While in the nursery, several red blanching macules had been noted on her trunk at age 2 days.

	Red macules and papules should raise the issue of the differential of vascular anomalies. According to the most recent International Society for the Study of Vascular Anomalies (ISSVA) classification, vascular anomalies can be divided into vascular malformations and vascular tumors.

	Vascular malformations are usually present at birth and can be divided into capillary malformation, venous malformation, lymphatic malformation, and arteriovenous malformation. Examples of capillary malformations include nevus simplex, also known as “salmon patch” or “stork bite,” and port wine stains.

	Unlike capillary malformation, venous malformations tend to appear as blue or purple, clustered and/or linear papules that may be painful. Lymphatic malformations can present as macrocystic (large mass with bluish hue), microcystic (small vesicles), or combined. Neck, axilla, groin, mediastinum, and retroperitoneum are the most common areas seen with lymphatic malformations, but any soft tissue or visceral site can be involved. Lastly, arteriovenous malformations are high-flow vascular shunts that may bleed or ulcerate and usually present as solitary bluish red nodules or deep seated masses.

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	Vascular tumors can be divided into 2 subtypes: benign and malignant.

 Benign vascular tumors include infantile hemangioma and congenital hemangioma. Infantile hemangiomas represent the most common vascular tumor of infancy and usually develop during the first 2 weeks of life. Some may be preceded by subtle telangiectasias of blanching red macules followed by the development of red and purple papules, plaques, and nodules with superficial and/or deep components.

 Classically, hemangiomas grow most rapidly from 2 to 8 weeks of age, stabilize, and often begin to regress during the second half of the first year. When there are more than 5 cutaneous hemangiomas, practitioners should consider the possibility of disseminated hemangiomas, often referred to as neonatal hemangiomatosis with involvement of multiple organ systems.

	Unlike infantile hemangiomas, congenital hemangiomas present at birth as round, partially compressible, reddish-blue plaques with a deep component and overlying telangiectasias and surrounding pallor. The rapidly involuting congenital hemangioma (RICH) variant regresses by 6 to 12 months of age, while noninvoluting congenital hemangiomas (NICH) grow very little but regress little or not at all.

 Malignant tumors include angiosarcoma and epithelioid hemangioendothelioma. Both are rare in the pediatric population and present with various morphologies, ranging from ecchymosis to enlarging mass.

	In this patient, the initial history and physical findings fit well with multiple infantile hemangiomas typical of neonatal hemangiomatosis. Further evaluation was necessary to exclude occult visceral involvement.

	Complete blood count (CBC), comprehensive metabolic panel (CMP), fecal occult blood test (FOBT), and abdominal ultrasound were obtained to evaluate for visceral involvement. The CBC revealed thrombocytopenia with platelet count of 44,000, and the CMP was unremarkable including normal liver function test. The FOBT was initially positive, but with reassuring hemoglobin and no grossly bloody stool she was monitored with serial FOBT and CBC. Abdominal ultrasound revealed innumerable high blood flow hepatic masses. This finding led to the diagnosis of diffuse neonatal hemangiomatosis. Because of the association between hemangiomas and hypothyroidism with increased type 3 iodothyronine deiodinase, a thyroid function test (TFT) was obtained and found to be normal.

	Infantile hepatic hemangiomas (IHH) are benign hepatic vascular neoplasms that share similar growth patterns, radiographic findings, and histologic marker patterns with cutaneous infantile hemangiomas.

 There are 3 major categories of IHH with distinct presentations: focal, multifocal, and segmental.

 Focal cutaneous lesions are the most common variant, presenting as round or oval red papules/plaques of varying size. When several hemangiomas of this type are present, they are called multifocal hemangiomas. Large lesions with geographic patterns are referred to as segmental hemangiomas, which may be associated with underlying soft tissue and bony defects and arterial vascular malformations.

Hepatic hemangiomas may present with focal, multifocal, or diffuse involvement. Focal hepatic hemangiomas are solitary round lesions that are frequently managed with observation.

 In many cases, these solitary lesions represent rapidly involuting congenital hemangiomas, and most resolve before the first birthday. On ultrasound, multifocal hepatic hemangiomas show multiple spherical lesions with intervening normal hepatic parenchyma. Rare diffuse infiltrative hemangiomas show near total replacement of hepatic parenchyma with innumerable tumors.

 Although both multifocal and diffuse IHH are associated with similar comorbidities, diffuse disease is associated with a significantly increased risk of congestive heart failure, hypothyroidism, hepatomegaly, hepatic failure, compartment syndrome, and death.

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	Management depends upon the type of hemangioma and includes serial ultrasounds, observation, and intervention when lesions are symptomatic. Propranolol is the first-line agent often resulting in rapid resolution of hemangiomas.

 Severe recalcitrant lesions may require resection, shunt embolization, or transplantation in extreme cases.

	During the patient’s hospitalization, the skin lesions continued to increase in number and in size. Dermatology and vascular surgery consultations were obtained, and propranolol therapy was initiated and advanced to 3 mg/kg/day in 3 divided doses, with instantaneous cessation in growth of the hemangiomas. A repeat ultrasound at discharge revealed no significant interval changes in size. Platelet count also recovered to 132,000 prior to discharge. The patient was discharged with propranolol therapy and with follow-up appointment at the vascular anomalies clinic.

	1. ISSVA classification for vascular anomalies. Issva.org. Available at: http://www.issva.org/UserFiles/file/Classifications-2014-Final.pdf. Published April 2014. Accessed April 17, 2017.

	2. Foley LS, Kulungowski AM. Vascular anomalies in pediatrics. 

	3. Deyrup AT, Miettinen M, North PE, et al. Pediatric cutaneous angiosarcomas: a clinicopathologic study of 10 cases. 

	4. Léauté-Labrèze C. Hoeger P, Mazereeuw-Hautier J, et al. A randomized, controlled trial of oral propranolol in infantile hemangioma. 

	5. Darrow DH, Greene AK, Mancini AJ, Nopper AJ; Section on Dermatology, Section on Otolaryngology–Head and Neck Surgery, and Section on Plastic Surgery. Diagnosis and management of infantile hemangioma. Pediatrics. 2015;136(4):e1060-e1104.

	6. Christison-Lagay ER, Burrows PE, Alomari A, et al. Hepatic hemangiomas: subtype classification and development of a clinical practice algorithm and registry. J Pediatr Surg. 2007;42(1):62-68.

	7. Kulungowski AM, Alomari AI, Chawla A, Christison-Lagay ER, Fishman SJ. Lessons from a liver hemangioma registry; subtype classification. J Pediatr Surg. 2012;47(1)165-170.

	8. Hsi Dickie B, Fishman SJ, Azizkhan RG. Hepatic vascular tumors. Semin Pediatr Surg. 2014;23(4):168-172.

	9. Dickie B, Dasgupta R, Nair R, et al. Spectrum of hepatic hemangiomas: management and outcome. J Pediatr Surg. 2009;44(1):125-133.

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A healthy newborn girl returns for a follow-up visit at age 7 days, and she is exhibiting multiple disseminated red macules and papules of varying sizes. While in the nursery, several red blanching macules had been noted on her trunk at age 2 days.

Author | Amanda Schlak, MD

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