INTRODUCTION — Congenital hemangiomas (CHs) are rare, benign vascular tumors that, unlike infantile hemangiomas (IHs), are present and fully grown at birth. They present as bossed plaques or exophytic masses located on the head, neck, or limbs (picture 1A-B). Based upon their natural history, two major subtypes of CH have been recognized: rapidly involuting congenital hemangioma (RICH) and noninvoluting congenital hemangioma (NICH) [1,2].
In most cases, RICH involutes completely or nearly completely by the age of 14 months, whereas NICH does not involute. NICHs typically grow in proportion with somatic growth but, in some cases, slowly expand [3-7]. A third intermediate subtype, the so-called partially involuting congenital hemangioma (PICH), shows overlapping features of RICH and NICH [8].
CH will be discussed in this topic. IH and other vascular tumors of childhood and capillary malformations are discussed separately.
●(See "Infantile hemangiomas: Epidemiology, pathogenesis, clinical features, and complications".)
●(See "Infantile hemangiomas: Evaluation and diagnosis".)
●(See "Infantile hemangiomas: Management".)
●(See "Tufted angioma, kaposiform hemangioendothelioma (KHE), and the Kasabach-Merritt phenomenon".)
●(See "Pyogenic granuloma (lobular capillary hemangioma)".)
●(See "Capillary malformations (port wine stains) and associated syndromes".)
EPIDEMIOLOGY — The precise incidence of congenital hemangioma (CH) is unknown. In a prospective study, RICH occurred in 2 of 594 newborns (0.3 percent) [9]. In a retrospective review of 6459 children with vascular anomalies seen in a vascular anomalies center, CHs were diagnosed in 14 percent, infantile hemangiomas (IHs) in 43 percent, and capillary malformations in 30 percent [10]. In contrast with IH, there is no known association between CH and premature birth or multiple gestation [11].
PATHOGENESIS — There is increasing evidence that most (or perhaps all) congenital hemangiomas (CHs), whether rapidly involuting, noninvoluting, or partially involuting, are due to somatic activating mutations in GNAQ and its paralog GNA11. Mutations that alter glutamine at amino acid 209 (Gln209) in GNAQ or GNA11 were found in all tested frozen-tissue samples from eight CHs collected during a clinically indicated procedure, at frequencies ranging from 3 to 33 percent [12]. The same mutations were found in three out of seven paraffin-embedded samples. The authors noted that since the same mutations are found in both NICH and RICH, other factors must account for the differing clinical presentations and postnatal behaviors. Mutations in this allele are also common in uveal melanoma and blue nevi and have been shown to constitutively activate mitogen-activated protein kinase (MAPK) and/or Yes-associated protein (YAP) signaling [13].
Mutations in GNAQ and GNA11 are a common cause of port wine stains, but they are typically found in other alleles, such as R183Q. In one case report, an infant with a very large CH, as well as innumerable small vascular tumors, was found to have a GNA11 mutation in the same allele in both the large tumor and in two of the smaller tumors [14]. Two other types of hemangiomas have also been described as being due to Gln209 GNAQ mutations: congenital hepatic hemangiomas [12] and anastomosing hemangiomas, a type of vascular tumor with adult onset that occurs mostly in the genitourinary tract and paravertebral soft tissues [15].
These genomic findings, together with immunohistochemical differences between CHs and infantile hemangiomas (IHs), as well as differing biomarker expression, confirm that they are distinct entities rather than variants of the same vascular tumor [16-18]. At the same time, they link together RICH, NICH, and PICH as related entities, but the reasons they differ in clinical behavior are not well understood.
HISTOPATHOLOGY — RICH, NICH, and PICH consist of small to large lobules of capillary proliferations lined by endothelial cells, with variably shaped lumen (round, slit shaped, or indistinct). In some cases, admixed fibrosis or sheets of compact endothelial cells and pericytes can be seen. Many of the endothelial cells show "hobnailed" features. Virtually all have extralobular vessels, which are usually large, tortuous, and irregular (picture 2) [3,16,19]. Focal thromboses and calcifications may be seen; stromal hemosiderin deposits are common. Immunohistochemical staining for glucose transporter protein-1 (GLUT-1) is negative. This finding helps distinguish congenital hemangiomas (CHs) from infantile hemangiomas (IHs), whose endothelial cells uniformly express GLUT-1 [17]. The lobular components of NICH and RICH stain for Wilms tumor 1 (WT1) protein, a transcription factor activated during angiogenesis, but the larger dysplastic vessels are WT1 negative [2,3,16,20-22]. There are no significant correlations between CH subtype and histopathology [19].
CLINICAL FEATURES
Overview — Congenital hemangiomas (CHs) are present and fully grown at birth. They usually present as solitary, plaque-like or exophytic lesions of size varying from a few centimeters to more than 10 cm (picture 1A-D). The observation of a rapid involutive process beginning in the first days or weeks of life allows the distinction between RICH and NICH.
Rapidly involuting congenital hemangiomas — RICH are most often located in the head and neck region (picture 1A) or lower extremities [1,23,24]. They vary in size from a few centimeters to 10 cm or more and are nearly always solitary, although multifocal lesions have been reported [25]. Several morphologic variants have been described, including:
●A raised, violaceous, soft-tissue mass with prominent peripheral veins (picture 1A)
●A soft-tissue mass with overlying prominent, coarse telangiectasias admixed with a blanched skin, including a halo of blanched skin at the periphery of the tumor (picture 1C)
●A pink to violaceous tumor with a deeper dermal or subcutaneous infiltration
●Exophytic vascular tumor with central ulceration [26]
●A vascular tumor with overlying pustules [27]
On palpation, lesions are typically warmer than the surrounding skin. Occasionally, a bruit can be heard, or a thrill can be felt. Rare examples of multifocal RICH have also been reported [28].
The involution of RICH typically starts a few days to a few weeks after birth and, in most cases, is complete in 6 to 14 months. In rare instances, involution may occur in utero [29]. Involution leaves areas of skin redundancy with dermal or subcutaneous atrophy, textural and color changes, and persistent telangiectasias or scattered veins (picture 3). Other local sequelae include permanent alopecia, superficial scarring, and milia formation [1,20,30].
In a small proportion of patients with RICH, involution is incomplete and leaves a vascular plaque with coarse telangiectasias on the surface and a peripheral, bluish-white border indistinguishable from NICH [2]. (See 'Partially involuting congenital hemangiomas' below.)
Hepatic rapidly involuting congenital hemangioma — RICH can also occur in the liver [31-33]. They present as an abdominal mass in an otherwise healthy infant. Transient thrombocytopenia and anemia are observed in some infants, presumably due to intralesional thrombosis. Arteriovenous shunting may result in heart failure and pulmonary hypertension. Although rare, these lesions can lead to instability in some neonates, with progressive liver failure and death. Significant bleeding can occur in some lesions thought to be secondary to the central areas of necrosis. There are reports of two infants developing significant ascites needing surgical intervention after the start of regression [34]. Ultrasonography demonstrates a well-circumscribed vascular mass with large feeding and draining vessels; coarse subcapsular calcifications can be seen after involution. On magnetic resonance imaging (MRI), the lesions are hyperintense on T2 imaging and hypointense on T1 imaging, with postcontrast imaging demonstrating early peripheral enhancement with eventual diffuse enhancement.
Noninvoluting congenital hemangiomas — NICH are well-circumscribed, round to oval, plaque-like or bossed, soft-tissue masses; the color varies from pink to blue-red or purple [2]. Overlying telangiectasias and a rim of pallor are characteristic. NICH are typically flatter than RICH. Two morphologic variants have been described: a patch type, characterized by a flat or slightly atrophic surface, resembling a vascular stain (picture 4), and a nodular/plaque type, associated with prominent tissue swelling (picture 1B, 1D) [3].
The tumor size ranges from a few centimeters to more than 10 cm. In many cases, peripheral enlarged draining veins can be seen in the surrounding skin (picture 4). Ulceration, scarring, and atrophy are uncommon. On palpation, lesions are typically warm and may have areas of induration with well-circumscribed borders.
NICH do not resolve spontaneously but tend to enlarge proportionately with the child's growth (picture 5). Doppler examination shows persistent fast flow. Nearly half of patients in one retrospective case series reported some degree of pain within their lesions [3]. Although classically thought of as static in size, some do expand over time.
In one case series, 9 of 80 NICHs (11 percent) had postnatal growth, after an initial stable period, at ages 2 to 10 years (mean 5.3 years) [4]. Other reports have also described expansion of NICH lesions usually during childhood [5-7] but even later in rare cases [7]. Surface changes (eg, development of papules or pyogenic granuloma), bleeding, and pain from focal ulceration have also been described [4,5].
Partially involuting congenital hemangiomas — In rare instances, CH involute rapidly during the first year of life, fulfilling the criteria for RICH, but fail to regress completely (picture 6). The residual tumor has the clinical, ultrasonographic, and histologic features of NICH [8,35]. In a series of eight patients with PICHs followed up for 2.5 to 10 years, the hemangiomas decreased in size and thickness in 12 to 30 months and then stabilized [8]. The residual lesions appeared as red-purple patches or plaques with telangiectasias, prominent veins, and a pale rim; Doppler ultrasound imaging revealed low-resistance, high-velocity arterial flow.
Complications
●Ulceration – Ulceration with minor bleeding is a frequent complication of large CH, particularly during the involution phase. Severe bleeding due to ulceration involving large feeding vessels or trauma is a rare occurrence and may require early surgical excision [26,36-38]. Liver CHs can have life-threatening bleeding.
●Thrombocytopenia and coagulopathy – Transient thrombocytopenia and coagulopathy with hypofibrinogenemia and anemia are potential complications of RICH, especially in liver lesions. The thrombocytopenia is usually mild and typically resolves without treatment in a few weeks [39,40]. In a case series of seven patients with large RICH presenting with thrombocytopenia, low fibrinogen, and high fibrin split products, only one developed petechiae [39]. In all cases, thrombocytopenia and coagulopathy resolved in two weeks.
Occasionally, transient thrombocytopenia in combination with coagulopathy and hypofibrinogenemia mimics the Kasabach-Merritt phenomenon (KMP), a life-threatening complication of other rare congenital vascular tumors (eg, tufted angioma [TA], hemangioendothelioma) characterized by profound and protracted thrombocytopenia and disseminated intravascular coagulation [39,41,42]. (See "Tufted angioma, kaposiform hemangioendothelioma (KHE), and the Kasabach-Merritt phenomenon".)
●Heart failure – High-output heart failure from arteriovenous shunting and cardiac overload has been reported in a small number of infants with large CH (>7 cm) [40,43]. Heart failure is present at birth or develops in the first days of life. There are rare reports of prenatal diagnosis of heart failure in association with a CH [40]. (See "Heart failure in children: Etiology, clinical manifestations, and diagnosis", section on 'Diagnostic evaluation'.)
●Pain – A minority of NICH cause local pain, occasionally to the point where treatment is needed [3,4].
DIAGNOSIS — In most cases, the diagnosis of congenital hemangioma (CH) is based upon history and physical examination. Imaging studies (eg, ultrasonography, magnetic resonance imaging [MRI], arteriography) may be helpful when the diagnosis is unclear. A biopsy for histologic examination is necessary if a malignant lesion is suspected. Additional evaluation, including laboratory and cardiac evaluation, may be needed in infants with suspected coagulopathy or congestive heart failure.
Clinical examination — In most cases, the diagnosis of CH is made clinically in a newborn presenting with a fully grown soft-tissue mass with overlying telangiectasias and peripheral vasoconstriction (picture 1A-D). The presence of the tumor at the time of birth is an important clue to the diagnosis and an important criterion for differentiating CH from infantile hemangiomas (IHs), which are usually not clinically evident at the time of delivery [1,44]. The observation of a rapid involution starting a few days after birth is usually sufficient to differentiate RICH from NICH.
Imaging studies — Imaging studies (eg, ultrasonography, MRI, arteriography) may be needed when the diagnosis is unclear:
●On ultrasonography, both RICH and NICH show a predominantly heterogeneous sonographic structure, diffuse vascularity, high vessel density, and, occasionally, calcifications [45]. Doppler examination reveals a high-flow vascular lesion [3]. In involuting lesions, long, tortuous, and compressible channels with a venous flow signal become a dominant feature [46]. The presence on B-mode and Doppler ultrasound of visible vessels, venous ectasia, venous lakes, and arteriovenous shunting in patients with RICH suggest increased risk of ulceration, bleeding, and, in the case of arteriovenous shunting, high-output congestive heart failure [47].
●On MRI, RICH and NICH show heterogeneous enhancement, hyperintensity on T2-weighted sequences, flow voids, and absence of peripheral edema. Less defined borders and more fat stranding help distinguish CH from IH [45].
●Angiography reveals heterogeneous parenchyma; large, irregular feeding arteries; venous ectasia; and, in some cases, micro-shunting [48]. Arterial aneurysms and arteriovenous shunts can also be seen.
Prenatal diagnosis — Because of their volume and vascularity, RICH are increasingly detected during prenatal ultrasound investigations, usually during the third trimester of pregnancy. Sonographic features include solid appearance with a homogeneous or slightly heterogeneous pattern isoechoic with the placenta, venous lakes mimicking a cystic component, hemosiderin deposits, and small calcifications [49].
If a RICH is noted on prenatal ultrasound, an antenatal MRI is recommended to define better the characteristics of the tumor. On fetal MRI, the signal of the lesion is slightly hyperintense or hypointense on T2-weighted sequences [49].
Biopsy — A biopsy for histopathologic examination should be performed if the diagnosis is uncertain or there is a clinical suspicion of malignant tumor (firmness on palpation, rapid growth, ulceration, and fixation to the fascia). Both RICH and NICH show similar histopathologic findings. The histologic finding of lobules of capillary proliferations embedded in a fibrous stroma containing hemosiderin deposits and surrounded by large dysplastic vessels confirms the diagnosis of CH. In contrast with IH, both RICH and NICH are glucose transporter protein-1 (GLUT-1) negative, but lobular areas may show positive staining for Wilms tumor 1 (WT1) protein [3]. Gene testing on frozen or paraffin-embedded tissue for GNAQ or GNA11 mutations can help to confirm the diagnosis in uncertain cases [12,14].
Additional evaluation — Laboratory blood tests are not routinely performed in infants with small, uncomplicated CH. However, a complete blood cell count with platelet count and evaluation of the coagulation status (eg, fibrinogen concentration, fibrin split products [d-dimer]) may be warranted in newborns with large tumors at risk of thrombocytopenia and coagulopathy, especially tumors of the liver. (See "Neonatal thrombocytopenia: Clinical manifestations, evaluation, and management", section on 'Diagnostic evaluation to identify underlying cause'.)
Comprehensive cardiac evaluation should be performed in infants with clinical signs of heart failure. (See "Heart failure in children: Etiology, clinical manifestations, and diagnosis", section on 'Diagnostic evaluation'.)
DIFFERENTIAL DIAGNOSIS — The differential diagnosis of congenital hemangioma (CH) includes other benign vascular tumors, vascular malformations, and benign and malignant nonvascular tumors [44]:
●Infantile hemangioma – A diagnostic dilemma often arises when the patient's parents report that a certain vascular growth "has been there since birth." It is particularly important to ascertain whether a palpable mass (suggesting CH) or just a flat area of discoloration was present at birth, since the latter could represent a premonitory mark of infantile hemangioma (IH) (picture 7). A history of rapid postnatal growth usually differentiates IH from CH. When the diagnosis is uncertain, a tissue biopsy for immunohistochemical staining for glucose transporter protein-1 (GLUT-1) can clarify the diagnosis. (See "Infantile hemangiomas: Epidemiology, pathogenesis, clinical features, and complications", section on 'Clinical presentation'.)
●Tufted angioma – Tufted angioma (TA) is an uncommon, benign vascular tumor that usually develops in early infancy but may be present at birth. It presents as an infiltrated, firm, dusky, red to violaceous plaque or nodule with typical, overlying hypertrichosis (picture 8). Histology shows vascular tufts of tightly packed capillaries, randomly dispersed throughout the dermis in a typical "cannonball distribution." TA is GLUT-1 negative. (See "Tufted angioma, kaposiform hemangioendothelioma (KHE), and the Kasabach-Merritt phenomenon", section on 'Tufted angioma'.)
●Kaposiform hemangioendothelioma – Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor that can be present at birth. KHE appears as a slightly raised, subcutaneous mass with a purpuric, bruised appearance (picture 9). It is often associated with the Kasabach-Merritt phenomenon (severe thrombocytopenia and coagulopathy). Histologically, KHE is characterized by spindled cells with minimal atypia and infrequent mitoses lining slit-like or crescentic vessels containing hemosiderin. The tumor is GLUT-1 negative. (See "Tufted angioma, kaposiform hemangioendothelioma (KHE), and the Kasabach-Merritt phenomenon", section on 'Kaposiform hemangioendothelioma'.)
●Vascular malformations – Venous malformations are often present at birth as soft, compressible, blue soft-tissue mass or plaque. Doppler ultrasonography demonstrates a slow-flow, ill-defined vascular lesion that is compressible on palpation. Histology shows ectatic venous-like channels with anomalies in mural cells. In contrast to CH, vascular malformations do not stain for cytoplasmic Wilms tumor 1 (WT1) [22]. (See "Capillary malformations (port wine stains) and associated syndromes".)
●Infantile myofibromatosis/hemangiopericytoma – Infantile myofibromatosis is a rare, fibrous tissue tumor of infancy. In most cases, it presents at birth as solitary or multiple nodules from one-half to several centimeters in diameter of firm or rubbery consistency and variable colors (brown, blue, purple, or red) (picture 10) [50]. A biopsy is necessary to differentiate infantile myofibromatosis from CH. Histopathology shows a biphasic pattern of peripheral smooth muscle-like fascicles of spindle cells and central areas resembling hemangiopericytoma with rounded pericytes. Given their considerable clinical and histologic overlap, infantile myofibromatosis and infantile hemangiopericytoma are thought to be related entities or identical conditions in different maturation stages [51-53]. (See "Skin nodules in newborns and infants", section on 'Infantile myofibromatosis'.)
●Lipoblastoma and lipoblastomatosis — Lipoblastoma is a rare, benign, infantile tumor that may be present at birth [54]. It presents as a well-circumscribed soft-tissue mass located in the subcutaneous tissue on the extremities, trunk, and head and neck area. Histology shows an admixture of mature and immature adipocytes.
●Malignant tumors — Highly vascular malignant tumors (eg, infantile fibrosarcoma, rhabdomyosarcoma, dermatofibrosarcoma protuberans, metastatic neuroblastoma, angiosarcoma) may be present at birth and mimic CH [50]. Clinical signs that raise the suspicion of a malignant lesion and prompt a biopsy for histopathologic examination include firmness on palpation, rapid growth, ulceration, fixation to the fascia, and an atypical appearance on imaging. (See "Skin nodules in newborns and infants", section on 'Malignant tumors'.)
MANAGEMENT
Approach — The approach to the management of congenital hemangioma (CH) must be individualized based upon the tumor size and location, tendency to spontaneous involution, and presence of local or systemic complications. Consultation with a specialist or multidisciplinary group with special expertise in vascular anomalies may be warranted for infants with large tumors that are at increased risk of complications (eg, bleeding, thrombocytopenia, high-output cardiac failure) or tumors that do not show a tendency to regress in the first weeks of life [55]. Large, complicated liver CH should be evaluated by an interdisciplinary vascular anomaly team.
It is important to educate the family about the natural history of CH, potential complications, and treatment options for noninvoluting lesions or residual skin changes after involution.
Rapidly involuting congenital hemangiomas — RICH typically involute by age 14 months. Because RICH are self-resolving, treatment is usually not necessary, unless there are complications such as ulceration or bleeding. (See 'Treatment of complications' below.)
Periodic clinical examinations are performed until complete involution has taken place and to assess for possible persistence (ie, PICH). The time to involution has not been precisely determined. Evidence from small case series and case reports indicates that most RICH undergo complete regression in 12 to 14 months [1,11,23]. It is our practice to examine infants with RICH for at least 12 to 18 months, usually at monthly intervals, to confirm the expected decrease in size. Further assessment may be needed to evaluate any unexpected complications, such as pain, ulceration, or infection, and to assess whether involution is complete or partial. We do not recommend routine laboratory monitoring, unless abnormalities (eg, thrombocytopenia) were previously noted. However, once thrombocytopenia has resolved, laboratory testing is no longer necessary.
During the rapid involution phase, the application of petrolatum to the lesion surface multiple times per day may be helpful in preventing ulceration [11]. Persistently ulcerated lesions or lesions with a history of repeated bleeding may require early surgical excision. (See 'Treatment of complications' below.)
After involution is completed, residual skin changes can be corrected surgically. Pulsed-dye laser treatment may be helpful in reducing the appearance of superficial telangiectasias [56]. (See "Laser and light therapy for cutaneous vascular lesions", section on 'Involuting and resolved lesions'.)
Noninvoluting congenital hemangiomas — NICH do not resolve over time, but treatment is not necessarily needed if they remain asymptomatic and do not bother the patient [2]. Pain or increased size may increase the need for treatment. In cases where treatment is indicated, pulsed-dye laser may help diminish superficial discoloration. For larger, thicker, or more symptomatic lesions, surgical excision is the treatment of choice.
In a series of 53 patients (age 2 to 30 years) with NICH, 28 underwent surgical excision [2]. Preoperative arterial embolization was performed in seven patients. No intraoperative or postoperative complications were reported.
The optimal timing for surgical excision has not been determined. Most experts recommend surgical excision during the preschool age, when the child begins to manifest a facial or body image [56].
Treatment of complications
●Ulceration – Superficial ulcerations can be treated conservatively with meticulous wound care, which involves the use of topical antibiotics (eg, mupirocin 2% cream), barrier creams (eg, petrolatum), and nonadherent dressings [57]. Crusting should be debrided with diluted hydrogen peroxide or saline soaks two to three times daily since crusting prevents re-epithelization and favors infection. Pulsed-dye laser treatment may be a treatment option for ulcerations that do not respond to topical therapy.
Pain associated with ulceration may be severe. Appropriate analgesia with oral acetaminophen or a topical anesthetic agent (ie, lidocaine hydrochloride 2% ointment) may be warranted. (See "Prevention and treatment of neonatal pain" and "Prevention and treatment of neonatal pain".)
●Bleeding – Minor bleeding can be controlled with compression. Persistent or major bleeding from ulceration or trauma may require arterial embolization and/or surgical excision of the tumor [26,37,38]. In two reported cases, control of minor bleeding from superficial ulcerations was successfully achieved with topical tranexamic acid and compression [36].
●Thrombocytopenia and coagulopathy – The thrombocytopenia associated with CH is transient and usually resolves in a few weeks without treatment. Systemic corticosteroids have been used in a small number of infants with RICH and thrombocytopenia, but their role in the normalization of the platelet count remains uncertain [39].
●Heart failure – For infants who develop CH-induced heart failure, embolization and/or surgical excision of the tumor in addition to medical treatment is required in most cases [40]. The medical management of congestive heart failure in infants and children is discussed separately. (See "Heart failure in children: Management".)
PROGNOSIS — Infants with uncomplicated RICH have a favorable prognosis. The prognosis is guarded for infants with severe bleeding and infants who develop heart failure. In a review of 17 patients with large congenital hemangioma (CH) and heart failure, four died in the first weeks of life despite embolization or excision of the tumor and medical treatment of heart failure [40]. The prognosis of NICH is generally favorable. Lesions that are not excised most often grow in proportion with the child and persist unchanged throughout adulthood [2]. However, a subset expand over time, albeit slowly, and recurrent discomfort or pain can occur in some cases [7].
SUMMARY AND RECOMMENDATIONS
●Congenital hemangiomas (CHs) are rare, benign vascular tumors that are present and fully grown at birth. Based upon their natural history, two major subtypes have been recognized: rapidly involuting congenital hemangioma (RICH) and noninvoluting congenital hemangioma (NICH). A third intermediate subtype, called partially involuting congenital hemangioma (PICH), shows overlapping features of RICH and NICH. (See 'Introduction' above.)
●RICH usually present as solitary, violaceous, soft-tissue masses with coarse telangiectasias and prominent peripheral veins, most often located on the head, neck, or lower extremities (picture 1A, 1C). Involution typically starts a few days to weeks after birth and, in most cases, is complete in 6 to 14 months (picture 3). In some patients, involution may be incomplete, and the residual lesion is indistinguishable from NICH. (See 'Rapidly involuting congenital hemangiomas' above and 'Partially involuting congenital hemangiomas' above.)
●NICH present as well-circumscribed, plaque-like or bossed, soft-tissue masses with overlying telangiectasias and a characteristic rim of pallor (picture 1B, 1D). NICH do not resolve spontaneously, but tend to grow proportionately with the child’s somatic growth. (See 'Noninvoluting congenital hemangiomas' above.)
●The diagnosis of CH is made clinically in a newborn presenting with a fully grown, soft-tissue mass with overlying telangiectasias and peripheral vasoconstriction (picture 1A-D). The rapid involution starting a few days after birth differentiates RICH from NICH. Imaging studies (eg, ultrasonography, magnetic resonance imaging [MRI], arteriography) and/or a biopsy for histopathologic examination may be warranted if the diagnosis is unclear or a malignant tumor is suspected. (See 'Diagnosis' above.)
●Because RICH are self-resolving, treatment is usually not necessary, unless there are complications. Periodic clinical examinations are performed until complete involution has taken place. (See 'Rapidly involuting congenital hemangiomas' above and 'Treatment of complications' above.)
●NICH do not resolve over time, but treatment may be not necessary if they remain asymptomatic and do not bother the patient. For larger, thicker, or more symptomatic lesions, surgical excision is the treatment of choice. Most experts recommend surgical excision during the preschool age, when the child begins to manifest a facial or body image. (See 'Noninvoluting congenital hemangiomas' above.)
