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Clinical study (Prospective, Retrospective, Case Series)

Ulceration in Congenital Hemangiomas

A Life-Threatening Complication

Cordisco, María Rosaa,b; Fernández Faith, Estebanc; Lanoel, Agustinab; Manunza, Francescab; Stefano, Paolab; Castro, Carlad; Durán-McKinster, Carolae; Velasquez, Felipef; Henostroza, Katiaf; Harfmann, Katyac; Baselga Torres, Eulàliag

Author Information
Journal of Vascular Anomalies: September 2021 - Volume 2 - Issue 3 - p e015
doi: 10.1097/JOVA.0000000000000015
  • Open

Abstract

Introduction

Congenital hemangiomas (CH) are a distinct type of benign vascular tumor, which follow a variable postnatal course. In contrast to infantile hemangiomas, CH are fully developed at birth and lack the typical postnatal proliferation phase of infantile hemangiomas. Three distinct types of CH have been characterized based on the postnatal course: rapidly involuting CH (RICH), noninvoluting CH (NICH), and partially involuting CH (PICH). Most patients with CH follow an uncomplicated course. Reported complications include coagulopathy, thrombocytopenia, ulceration, bleeding, and high-output cardiac failure.1–4 Our knowledge of ulceration in CH has been limited to case reports and small case series to date. Here we report on a large cohort of patients with CH ulceration to expand our understanding of this potentially life-threatening complication.

Methods

A retrospective chart review of patients with CH who developed ulceration was performed. Cases were identified in 7 tertiary referral centers with expertise in vascular anomalies. Data were collected on demographics, clinical presentation, imaging characteristics, laboratory reports, histopathology, complications, and treatment course.

Results

Demographics and clinical features

A total of 20 patients were identified, 11 males and 9 females. Two patients (cases 7 and 10) were previously reported as part of a case series of coagulopathy in CH.1 Details are summarized in Table 1. Available clinical photographs are presented in Figures 1–4. Most patients were born at term, 4 patients were born prematurely. A prenatal diagnosis of CH was made in 4 patients. The most common location of the CH was the extremities (n = 12, 60%), followed by the head and neck (n = 7, 35%), with a single case on the trunk. The average diameter was 7.8 cm, ranging from 1.5 to 14 cm. Three main morphological variants were recognized: exophytic tumors, subcutaneous tumors, and vascular plaques. Exophytic tumor was defined as a vascular mass with outward projection, with a wide or narrow base. Subcutaneous tumors were characterized by dermal and/or subcutaneous infiltration with almost circumferential involvement when located on an extremity. The third category was used for broad, elevated vascular plaques, similar to the morphology of indeterminate or segmental infantile hemangiomas. The most common pattern was exophytic tumors (n = 14, 70%). Subcutaneous tumors were present in 3 patients and vascular plaques in 3 patients. Of the 3 CH presenting as a vascular plaque, the diagnosis was confirmed by characteristic imaging features in all 3 cases, and by histologic findings in 2 patients. Imaging was performed in 18 patients (90%), with ultrasound being the most common modality of imaging (18 patients), followed by magnetic resonance imaging (5 patients), angiography (2 patients), and computerized tomography scan (3 patients). Details of imaging findings are summarized in Table 2. Histopathological evaluation was performed in 8 patients. This was done to confirm the diagnosis in 5 patients, and as part of surgical intervention in 3 patients. Common histologic findings included lobular vascular proliferations with plump endothelial cells and GLUT-1 negativity.

Table 1. - Clinical Features, Treatment Course, and Complications
Case No. Gender/GA Location/Size (cm) Clinical Biopsy Imaging Age at Ulceration (wk) Location of Ulceration Complications Treatment Time to Heal (wk) Postnatal Course
1 F/40 wk Left parietal scalp/6 × 7 × 1.5 Exophytic tumor with coarse telangiectasias and pale halo No US, CT scan Birth Central Normal coagulation studies None 4 PICH (partial regression at 5 mo leaving central depression and telangiectasias)
2 M/37 wk Left forehead/8 × 3.5 Purple plaque with pale halo No US, MRI, angiography Birth Central - Vaseline 6 PICH (partial regression leaving violaceous patch with prominent veins)
3 M/39 wk Left parietal scalp/6 × 7 × 1.5 Purple plaque with pale halo Yes, Glut 1 (-) US Birth Central Thrombocytopenia (90,000), recurrent bleeding, sepsis Systemic antibiotic 8 Lost to follow up
4 F/37 wk Occipital scalp/5 × 5 Exophytic tumor with coarse telangiectasias and pale halo No US Birth Central Normal coagulation studies None 6 PICH (partial regression over 2 mo leaving central depression with pale halo)
5 M/36 wk Left upper extremity/8 × 9 × 2 Purple lobulated exophytic tumor Yes, Glut1 (-) US Birth Central Normal coagulation studies Topical and oral antibiotic 16 RICH (complete involution at 17 mo leaving depressed area)
6 M/36 wk Right upper extremity/10 × 11 Large subcutaneous tumor with tense purple skin and fine telangiectasias Yes, Glut 1(-) US, CT scan Birth Central Thrombocytopenia (74,000), sepsis Prednisone, systemic antibiotic 4 PICH (partial regression at 12 mo leaving residual loose skin and scar)
7 F/37 wk Right upper extremity/6 × 4 Exophytic tumor with coarse telangiectasias, central depression, and pale halo Yes, Glut 1(-) US, angiography Birth Central Thrombocytopenia (62,000), elevated D-dimer, hypofibrinogenemia Prednisone, embolization, surgical resection of PICH 28 PICH (regression over 15 mo, residual ovoid plaque with telangiectasias and pale rim)
8 F/37 wk Abdomen/4 × 3.5 × 2.2 Exophytic tumor with pale halo and large draining veins Yes, Glut 1 (-)
GNAQ/GN11(-)
US 3 Central Mild thrombocytopenia (100,000), bleeding Surgical resection N/A Surgical resection at 1 mo of age
9 M/39 wk Left lower extremity/7.5 × 7 Violaceous subcutaneous tumor with pale halo No US Birth Lateral Normal coagulation studies Fucidic ointment 8 PICH (partial regression for 2 mo leaving central depression and telangiectasias)
10 M/38 wk Left lower extremity/11 × 13 Large subcutaneous purple tumor No None Birth Lateral (multifocal) Thrombocytopenia (30,000), elevated D-dimer, hypofibrinogenemia Prednisone 8 RICH (complete involution at 16 mo leaving hypertrophic scar and prominent dilated veins)
11 F/37 wk Left lower extremity/9 × 7.5 Exophytic tumor with coarse telangiectasias, central depression, and pale halo No US, MRI Birth Central Normal coagulation studies Prednisone, systemic antibiotic 8 PICH (partial regression ceased at 8 mo leaving central scar with residual telangiectasias with pale halo)
12 M/38 wk Left lower extremity/5 × 6 × 1 Exophytic purple tumor with fine telangiectasias and pale halo No US Birth Central Elevated D-dimer None 4 PICH (regression at 18 mo leaving hypopigmented central area with telangiectasias, pale halo, and prominent veins)
13 F/37 wk Left lower extremity/6 × 5 × 0.5 Exophytic violaceous tumor with pale halo No US Birth Central Normal coagulation studies Topical collagenase 2 RICH (complete involution over 5 mo leaving central depressed scar, pale halo)
14 F/30 wk Right lower extremity/10 × 9 × 5 Exophytic tumor with coarse telangiectasias Yes, Glut 1(-) US 2 Lateral Thrombocytopenia (54,000), bleeding Prednisone, propranolol, surgical resection N/A Surgical resection at 2 mo of age
15 M/39 wk Right lower extremity/5 × 4 × 1.5 Exophytic purple tumor with coarse telangiectasias and pale halo No US Birth Central Sepsis, severe bleeding, anemia, thrombocytopenia (179,000/9000/4000), leukopenia, elevated D-dimer, hypofibrinogenemia, superior mesenteric artery thrombosis Systemic antibiotics, blood products N/A Death at 1 mo of age
16 F/37 wk Vertex scalp/1.5 × 1.5 Small exophytic violaceous tumor with pale halo No US Birth Central - Vaseline 2 RICH (complete involution at 12 mo)
17 F/35 wk Right occipital scalp/14 × 13 × 3.5 Large plaque with tense purple skin and pale halo Yes, Glut 1 (-) US, MRI, CT scan Birth Central Normal coagulation studies Topical antibiotic 16 RICH (complete involution at 13 mo leaving residual loose skin)
18 M/37 wk Left lower extremity/9 × 7 Purple exophytic tumor with coarse telangiectasias and pale halo No US, MRI Birth Central - None 2 PICH (rapid involution at 4 mo leaving plaque with telangiectasias and pale halo)
19 M/38 wk Right lower extremity/6 × 7 × 1.5 Exophytic purple tumor with coarse telangiectasias and pale halo No None Birth Central - None 1 PICH (regression at 8 mo leaving telangiectatic plaque with pale halo)
20 M/37 wk Left temporal scalp/6 × 5 × 2 Exophytic purple tumor with pale halo Yes, Glut 1 (-) US, MRI Birth Lateral Normal coagulation studies None 8 RICH (complete regression over 14 mo leaving depressed central area)
Abbreviations: CT, computerized tomography; F, female; GA, gestational age; M, male; MRI, magnetic resonance imaging; PICH, partially involuting congenital hemangioma; RICH, rapidly involuting congenital hemangioma; US, ultrasound.

Table 2. - Imaging Features
Case No. Imaging Features
1 Ultrasound: heterogeneous mass with fast flow vessels
3 Ultrasound: fast flow large vessels and arteriovenous shunting
4 Ultrasound: highly vascularized mass
Angiography: enlarged axillary and brachial arteries and veins, and intralesional arteriovenous shunts
6 Ultrasound: mass with high vascularization and intralesional arteriovenous fistulae
7 Ultrasound: intercommunicating central anechoic areas. Peripheral echogenic areas with arterial and venous flow
CT scan: heterogeneous vascular subcutaneous lesion without bone or intracranial compromise
8 Ultrasound: heterogeneous hypervascular mass which appears to infiltrate underlying muscle. Predominant venous flow with large caliber vessel draining in the common femoral vein
9 Ultrasound: heterogeneous, solid, echogenic mass with anechoic areas corresponding to calcified channels and arteriovenous flow
CT scan: mass with homogeneous enhancement organized in a lobular pattern
10 Ultrasound: heterogeneous structure with visible vessels.
MRI: expansive extracranial lesion with remodeling of cranial vault (T1 hypointense, T2 hyperintense). Enhances with contrast
CT scan: expansive extracranial lesion on right parietotemporal region with intracranial projection
11 Ultrasound: fast flow mass with venous and arterial component
MRI: stir MRI show large hypervascular mass with flow voids
12 Ultrasound: richly vascularized lesion with large venous and arterial vessels
13 Ultrasound: hypervascular heterogeneous mass with large vessels
MRI: mass with T2 high intensity signal with arteriovenous shunts and flow void
15 Ultrasound: heterogeneous mass with arteriovenous shunts and calcifications
16 Ultrasound: highly vascularized mass with arterial and venous component
MRI: axial T2 weighted scan with hyperintense mass with flow voids
17 Ultrasound: fast flow vascular tumor with large dilatation of superficial veins with calcifications
18 Ultrasound: superficial soft tissue mass with scattered punctate echogenic foci (calcifications) and cystic areas. Vascular structures course through the mass
19 Ultrasound: multiple serpiginous vessels within the subcutaneous tissue with robust venous flow.
MRI/angiography: large vascular mass extending into the left orbit with arteriovenous shunting
20 Ultrasound: heterogeneous mass with arterial and venous components.
Abbreviations: CT, computerized tomography; MRI, magnetic resonance imaging.

F1
Figure 1.:
Case 1 at birth (A) and partial involution at 5 mo of age (B). Case 2 at birth (C) and partial involution with residual erythema and prominent vasculature at 16 mo of age (D). Case 3 with ulcerated plaque at birth (E). Case 4 with ulcerated exophytic tumor at birth (F).
F2
Figure 2.:
Case 5 at birth (A) and complete involution at 17 mo of age leaving residual loose skin (B). Case 6 at birth (C) and at 12 mo of age (D). Case 7 at birth (E) and 3 y of age (F). Case 8 at birth (G) and 3 wk of age (H).
F3
Figure 3.:
Case 9 at birth (A) and 1 mo of age (B). Case 10 at 2 mo of life (C) and complete involution at 5 y of age with prominent veins and hypertrophic scarring (D). Case 11 at birth (E) and partial involution at 8 mo of age (F). Case 12 at birth (G) and partial involution at 2 y of age (H).
F4
Figure 4.:
Case 13 at birth (A) and complete involution at 5 mo of age (B). Case 14 at birth (C) and 2 wk of age (D). Case 15 at birth (E) and 1 mo of age (F).

Ulceration characteristics

The ulceration was present at birth in 18 patients (90%). The remainder 2 patients developed ulceration at 2 and 3 weeks of age. The ulceration developed centrally in 16 patients (80%), while lateral location of the ulceration was noted in 4 patients. The average size of the ulceration was 2 cm (largest diameter), ranging from 0.5 to 4.5 cm.

Treatment and time to heal

Topical therapy was used in 6 patients (30%), including petrolatum-based ointment, topical antibiotic, and collagenase. Systemic therapy was used in 8 patients (40%), including systemic corticosteroids (n = 5, 25%), systemic antibiotic (n = 5, 25%), and propranolol in 1 patient. Embolization was performed in 1 patient and surgical resection in 2 patients. Another patient (case 7) underwent surgical resection of residual tissue after partial involution. The time to heal of the ulceration ranged from 1 to 28 weeks, with a median time to heal of 8 weeks.

Complications

Additional complications were reported in 7 patients (35%). Coagulation studies were performed in 16 subjects, with normal results in 8 patients. Thrombocytopenia occurred in 7 patients. One patient had more severe and complex coagulopathy (case 15, details below). Bleeding occurred in 4 patients (20%) and sepsis in 3 patients (15%). One of our patients had a fatal outcome (case 15). This patient presented with an exophytic CH on the thigh with ulceration present at birth without bleeding. At 1 month of age, the hemorrhagic crust was accidentally detached by a caregiver resulting in profuse bleeding. He was admitted with signs of hypovolemic shock, with initial laboratory findings of severe anemia, prolonged prothrombin time, and normal platelet count. His course was complicated with sepsis, abdominal distension, and worsening coagulopathy (thrombocytopenia, elevated D-dimer, and hypofibrinogenemia). He received blood product transfusions and an exploratory laparotomy showed superior mesenteric artery thrombosis with transmural ischemic necrosis. Multiorgan failure ensued leading to his demise.

Postnatal course

Information on postnatal course was available in 16 patients. Of patients with information on postnatal course, 10 patients (62.5%) were categorized as PICH, while the course in 6 patients (37.5%) was characteristic of a RICH. One patient was lost to follow up, 2 patients were treated with surgical resection in early infancy and 1 patient died (see Complications).

Discussion

Reports of ulceration and bleeding of CH are limited to case reports and small case series. Table 3 summarizes previous reports of ulceration or bleeding in CH.3–10 Included in this table are 2 patients with severe hemorrhage reported with a clinical diagnosis of hemangioma, although their description is consistent with CH.11,12 These reports were published before the characterization of CH. Other case series of CH include patients who developed ulceration as a complication.13–17 Our study represents a large cohort of patients with ulcerated CH and expands our knowledge of this complication.

Table 3. - Previously Reported Cases of Ulceration and Severe Bleeding in Congenital Hemangiomas
Study No. Patients (Gender) Location Age at Ulceration Complications Treatment Course
Leung et al (2019)5 1 (M) Knee Birth - None RICH
Al Malki et al (2018)6 1 (F) Scalp 4 wk Severe bleeding - RICH
Vildy et al (2015)7 2 (F, M) Knee, thigh Birth Severe bleeding Propranolol, embolization, surgery -
Scalise et al (2014)8 1 Scalp Birth - Wound care RICH
Weitz et al (2013)4 1 (F) Neck Birth Cardiac failure, coagulopathy Prednisolone, propranolol, embolization, surgery -
Powell et al (2012)3 2 (F, M) Forehead, knee 1, 3 wk Bleeding Tranexamic acid PICH,RICH
Nolan et al (2012)9 1 (M) Flank Birth Thrombocytopenia, bleeding Surgery -
Agesta et al (2003)10 1 Thigh 34 d Severe bleeding Prednisone, surgery -
Boussemart et al (1995)11 1 (F) Neck 5 d Severe bleeding Sclerotherapy -
Nakada et al (1993)12 1 (M) Arm 19 d Severe bleeding Surgery -
Abbreviations: F, female; M, male; PICH, partially involuting congenital hemangioma; RICH, rapidly involuting congenital hemangioma.

Ulceration developed at birth in the majority of our patients, and before 4 weeks of age in the entire cohort. Previously reported cases also document the development of CH ulceration early in life, particularly in the first month. This highlights the importance of adequate counseling of parents and need for close monitoring early on. At the same time, it may provide reassurance about a lower risk of ulceration after 1 month of age.

The pathophysiology of ulceration in CH is not clear. As documented in this cohort and previous reports, this complication occurs in RICH, but not in NICH. The process of involution appears to be an inherent determinant of ulceration in CH. This is in contrast to infantile hemangiomas in which ulceration occurs most commonly during the proliferation phase. A central area of regression with marked depression is a characteristic finding in RICH, which aids in the clinical and radiologic diagnosis. A centrally located ulceration was present in 90% of our patients supporting the direct role of involution in the development of ulceration. Its association with the active involution process in CH may suggest rapid or uncontrolled apoptosis of tumoral tissue which may lead to necrosis and skin breakdown. The association of thrombocytopenia in some patients, and less commonly more severe coagulopathy, also suggest a localized intravascular coagulopathy with formation of microthrombi as a potential associated factor in the development of CH ulceration. As an alternative hypothesis, one may also speculate that proliferation of CH leads to ulceration, and this complication starts to develop in utero when CH are thought to proliferate. The presence of ulceration at birth in 85% of our cohort would support this theory.

Ulceration in CH carries the risk of other complications including pain, infection, and life-threatening bleeding. The majority of our patients experienced a favorable course without other complications. Nonetheless, an important minority experienced additional complications, with a fatal outcome in 1 patient. Apart from ulceration, thrombocytopenia was the most common complication in this cohort. A transient coagulopathy has been described in patients with RICH.1 It is characterized by thrombocytopenia, low fibrinogen, and elevated D-dimers. This coagulopathy is less severe and transient, which differentiates it from Kasabach-Merritt phenomenon seen in kaposiform hemangioendotheliomas and tufted angiomas. One of the patients in this cohort (case 20) developed severe thrombocytopenia and coagulopathy. These complications developed in the setting of sepsis, which likely contributed in a significant manner to the severity of his coagulopathy and eventual demise. Screening laboratory studies to include platelet count and coagulation studies in newborns with CH may assist in determining the risk of ulceration or potential for severe bleeding.

While a rare complication, life-threatening bleeding has been reported in CH.7,9–12 Severe bleeding is thought to occur in CH as a result of high flow large caliber blood vessels and arteriovenous shunting in close proximity to the skin. The occurrence of skin breakdown exposes these large caliber vessels resulting in difficult to control hemorrhage. The presence of thrombocytopenia and abnormal coagulation enhances the severity of bleeding. The unfortunate course of one of our patients and other reports of life-threatening hemorrhage highlight the importance of avoiding active debridement of the hemorrhagic crust, providing gentle wound care and ensuring close monitoring and adequate education to the parents and providers involved in their care. The accidental detachment of the hemorrhagic crust with the resultant severe hemorrhage lead to hypovolemic shock in our patient (case 15). Sepsis with ensuing coagulopathy and multiorgan failure resulted in his fatal outcome.

The clinical features of RICH are variable, typically described as raised or hemispheric violaceous tumors or plaques with variable presence of a pale rim, overlying telangiectasias, large radial veins, and central depression.2,14 A clinical classification such as the one used for infantile hemangiomas has not been established for CH. We recognize the clinical classification we proposed has not been previously used for CH. Our proposed classification demonstrates the variability in the clinical presentation of CH and may aid in preventing a delay in clinical diagnosis particularly for high risk patients. Larger studies are needed to verify and establish a clinical classification of CH. The majority of our patients presented with an exophytic tumor. A pale halo was noted in most exophytic tumors and all vascular plaques in our cohort. A subcutaneous tumor was noted in 3 of our patients, in which the CH involved an extremity circumferentially (cases 2, 5, and 9). The deeper infiltration and circumferential pattern of CH is not well described in the literature and may pose a challenge for clinical diagnosis.

Imaging studies are often performed as part of the evaluation of CH. Ultrasonographic features of CH include high blood flow, presence of arterial and venous vessels, along with calcifications.18,19 An ultrasound was performed in the vast majority of patients in our cohort, showing features consistent with previous reports (Table 2).

Other neoplasms, including vascular and soft tissue tumors, may have a similar presentation with ulceration, coagulopathy, and equivocal features on imaging studies. Diagnostic considerations include kaposiform hemangioendothelioma, tufted angiomas, congenital rhabdomyosarcoma, congenital fibrosarcoma, teratoma, and infantile myofibroma.1,20,21 Biopsy for tissue confirmation should be considered when the diagnosis is not clear. On histology, RICH are characterized by a lobular architecture of capillaries in the dermis and subcutis, with prominent lobular draining channels.14 Hemosiderin granules, calcifications, and thrombi may also be present. Characteristically, endothelial cells in CH stain negative for GLUT-1.

Management of ulceration in CH is complex and often requires a multidisciplinary approach. The majority of our patients were successfully treated with wound care. Systemic therapy was used in 10 patients, most commonly systemic antibiotics and systemic corticosteroids. Propranolol was used in 1 patient, who also received oral corticosteroids. Of note, this patient subsequently underwent surgical resection. Evidence is lacking for effective therapies for CH ulceration. Although systemic corticosteroids and propranolol were used in some of our patients, it is difficult to determine the benefits or efficacy of these treatment interventions and cannot be recommended based on our cases. Risks and benefits must be carefully weighed, and the decision to start systemic therapy must be considered on a case by case basis. Petrolatum-based barrier ointments are often recommended to prevent detachment of the hemorrhagic crust. Topical tranexamic acid has also been reported in 2 patients with adequate control of bleeding.3 Surgical resection is of particular benefit particularly in patients with exophytic lesions or with severe bleeding. Embolization, alone or in combination with surgery may be considered in patients with severe bleeding or high output cardiac failure.

In contrast to infantile hemangiomas, propranolol does not appear to have the same benefits for CH. A successful pharmacological treatment for CH is yet to be identified. Uncomplicated CH are typically monitored initially to determine their postnatal course. Treatment options for residual changes include surgery, sclerotherapy, or vascular lasers for surface changes. The identification of a pharmacological intervention could play a crucial role in patients who experience severe complications. The discovery of GNAQ/GNA11 mutations in CH makes this molecular pathway a potential therapeutic target.22

This study is limited by its retrospective design. The patients did not undergo a systematic work up and management; rather their evaluation was dictated by the features at time of presentation. Histology was available in 8 patients. The remaining patients did not have a biopsy, but were considered to present the clinical, imaging features, and course with complete or partial involution consistent with a diagnosis of CH. Moreover, the institutions involved in this study are tertiary referral centers, possibly introducing a selection bias for more complex cases.

This report represents the largest study of ulceration in CH to date. It highlights the clinical presentation and potential complications associated with ulceration. This is a serious, potentially life-threatening complication which requires a multidisciplinary approach.

Acknowledgments

The authors thank the patients and their families for their collaboration.

References

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        Keywords:

        Congenital hemangioma; Hemangioma; Thrombocytopenia; Ulceration

        Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc. on behalf of The International Society for the Study of Vascular Anomalies.