A 20-day-old boy presented for evaluation of a blue nodule on the right shoulder that had been present since birth. The mother noted no changes in the size of the lesion since birth, and no bleeding or ulceration was noted. The patient’s past medical history was unremarkable. He was a full-term baby born at 39 weeks gestation via a normal, spontaneous vaginal delivery. The pregnancy was uncomplicated. The mother had no history of maternal hypertension and had no history of chorionic villus sampling or amniocentesis. The patient was not taking any medications and had no known allergies.
Physical exam revealed a well-developed, well-nourished male in no apparent distress. A full skin exam revealed a 1.8 cm × 2 cm discrete, blue-purple vascular nodule with coarse surface telangiectasias and a surrounding rim of pallor on the right shoulder (Figure 1). There was no bleeding or breakdown noted. There was no lymphadenopathy present.
A 20-day-old infant with a warm blue vascular nodule on the right shoulder.
Images courtesy of Lori Asztalos, MD.
Follow-up evaluation of this infant at 6 months of age revealed a stable lesion with no change in size or color. Further follow-up evaluation at 18 months of age revealed the lesion was essentially unchanged with enlargement proportional with the child’s growth (Figure 2).
Follow-up of nodule in Figure 1 at 18 months of life. The lesion is essentially unchanged.
Noninvoluting Congenital Hemangioma
An infantile hemangioma (IH) is the most common vascular tumor in infancy. They are most often not present at birth, unlike the more rare congenital hemangiomas (CHs) that develop in utero and are present at birth. An IH undergoes postnatal proliferation that peaks in the first several months of life and is followed by slow involution between ages 1 and 7 years.1
CHs were first described by Boon2 in 1996 as either rapidly involuting congenital hemangiomas (RICH) or noninvoluting congenital hemangiomas (NICH). RICH is defined by its spontaneous complete involution within 6 to 14 months of age (Figures 3–4) while NICH, the rarer type of CH, is defined by its proportional growth with the child without regression.3–5 Both RICH and NICH are often solitary lesions with a similar average diameter and are commonly found on the head, neck, and extremities near joints.1,5 Clinically, a NICH is a warm round or oval plaque with a pink, blue, or violaceous color with prominent central telangiectasias and peripheral pallor often with draining veins in the periphery.3,5,6 There is some question as to whether RICH and NICH are indeed separate entities or simply part of a spectrum because of reported overlapping clinical and histopathological features.7 A recent review describes eight cases of CHs that initially were thought to be RICH, but the usual rapid involution process stopped and the lesions persisted.8
A rapidly involuting congenital hemangioma at 6 days of life. It is a discrete vascular nodule with an erythematous center and peripheral rim of pallor.
Follow-up of lesion in Figure 3 at 5 months of life. It is now a flat vascular plaque with significant flattening.
CHs are reported as fast-flow vascular lesions on ultrasound and magnetic resonance imaging (MRI), with NICH demonstrating arteriovenous microfistulas.1,2,4 Unlike arteriovenous fistulas or vascular malformations, neither NICH nor RICH demonstrate venous filling or show flow voids and hyperintensity on T2-weighted sequences on MRI 1,9 A retrospective study by Guillaume et al found calcifications in 17% of NICH lesions and in 37.5% of RICH lesions on ultrasound, but not in IH lesions. On histological evaluation, CHs show increased reactivity with Wilms’ tumor antigen (WT1), whereas vascular malformations do not. Of note, IHs show increased staining with glucose transporter-1 (Glut-1) stain, whereas CHs do not.9–11
Although CHs and IHs are benign, complications have been reported. Moderate high-output cardiac failure was found in a 2-year-old boy with a large gluteal-area NICH.12 There have also been reports of thrombocytopenia and coagulopathy with RICH lesions that may mimic a mild Kasabach-Merritt phenomenon, although the true Kasabach-Merrit phenomenon — which has a more profound thrombocytopenia — is not associated with CH or IH.3,13 Ulceration and bleeding have also been reported in CH.
Unlike IH or RICH, NICH does not resolve. Of note, the lesions are usually asymptomatic and can be observed if the patients and parents are willing.1,9 Surgical excision can also be considered, particularly for disfiguring lesions. Laser therapy is likely of limited value but has been reported to decrease the color in some cases.10 RICHs demonstrate spontaneous involution (Figure 4) but may leave fibro-fatty tissue and atrophy that may later require surgical intervention.
- Mulliken JB, Enjolras O. Congenital hemangiomas and infantile hemangioma: missing links. J Am Acad Dermatol. 2004;50(6):875–882. doi:10.1016/j.jaad.2003.10.670 [CrossRef]
- Boon LM, Enjolras O, Mulliken JB. Congenital hemangioma: evidence of accelerated involution. J Pediatr. 1996;128(3):329–335. doi:10.1016/S0022-3476(96)70276-7 [CrossRef]
- Kanada KN, Merin MR, Munden A, Friedlander SF. A prospective study of cutaneous findings in newborns in the United States: correlation with race, ethnicity, and gestational status using updated classification and nomenclature. J Pediatr. 2012;161(2):240–245. doi:10.1016/j.jpeds.2012.02.052 [CrossRef]
- Gorincour G, Kokta V, Rypens F, et al. Imaging characteristics of two subtypes of congenital hemangiomas: rapidly involuting congenital hemangiomas and non-involuting congenital hemangiomas. Pediatr Radiol. 2005;35(12):1178–1185. doi:10.1007/s00247-005-1557-9 [CrossRef]
- Krol A, MacArthur CJ. Congenital hemangiomas: rapidly involuting and noninvoluting congenital hemangiomas. Arch Fac Plast Surg. 2005;7(5):307–311. doi:10.1001/archfaci.7.5.307 [CrossRef]
- Stein JA, Heidary N, Pulitzer M, Schaffer JV. Noninvoluting congenital hemangioma. Dermatol Online J. 2008;14(5):7.
- Berenguer B, Mulliken JB, Enjolras O, et al. Rapidly involuting congenital hemangioma: clinical and histopathologic features. Pediatr Dev Pathol. 2003;6(6):495–510. doi:10.1007/s10024-003-2134-6 [CrossRef]
- Nasseri E, Piram M, McCuaig CC, et al. Partially involuting congenital hemangiomas: a report of 8 cases and review of the literature. J Am Acad Dermatol. 2013; pii: S0190-9622(13)00969-9.
- Enjolras O, Mulliken JB, Boon LM, et al. Noninvoluting congenital hemangioma: a rare cutaneous vascular anomaly. Plast Reconstr Surg. 2001;107(7):1647–1654. doi:10.1097/00006534-200106000-00002 [CrossRef]
- Hoeger PH, Colmenero I. Vascular tumours in infants. Part I: benign vascular tumours other than infantile haemangioma. Br J Dermatol. 2013; doi:10.1111/bjd.12650 [CrossRef].
- North PE, Waner M, Mizeracki A, Mihm MC Jr, . GLUT1: a newly discovered immunohistochemical marker for juvenile hemangiomas. Hum Path. 2000;31(1):11–22. doi:10.1016/S0046-8177(00)80192-6 [CrossRef]
- Cole P, Kaufman Y, Metry D, Hollier L. Non-involuting congenital haemangioma associated with high-output cardiomyopathy. J Plast Reconstr Aesthet Surg. 2009;62(10):e379–382. doi:10.1016/j.bjps.2008.01.037 [CrossRef]
- Baselga E, Cordisco MR, Garzon M, et al. Rapidly involuting congenital haemangioma associated with transient thrombocytopenia and coagulopathy: a case series. Br J Dermatol. 2008;158(6):1363–1370. doi:10.1111/j.1365-2133.2008.08546.x [CrossRef]