Home About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Subscribe Contacts Login 
Home Print this page Email this page Small font size Default font size Increase font size Users Online: 321

 Table of Contents  
Year : 2019  |  Volume : 20  |  Issue : 1  |  Page : 57-59

Rapidly involuting congenital hemangioma with complete resolution In Utero

Department of Dermatology, Doncaster Royal Infirmary, Doncaster, England, UK

Date of Web Publication14-Dec-2018

Correspondence Address:
Dr. Sabitha Lakshminarayanan
Department of Dermatology, Lincoln Suite, Armthorpe Lane, Doncaster Royal Infirmary, Doncaster, DN2 5LT
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpd.IJPD_122_17

Rights and Permissions

There are three types of congenital hemangioma - rapidly involuting congenital hemangioma (RICH), partially involuting congenital hemangioma, and noninvoluting congenital hemangioma. We report a case of RICH on the back of a 4-month-old Caucasian male infant, based on ultrasound and magnetic resonance imaging findings in the antenatal period, skin changes at birth, and a normal ultrasound after birth. He was born with an atrophic plaque at the site of the vascular swelling depicted on the scans, suggesting the complete involution of the lesion in the antenatal period.

Keywords: Antenatal resolution, hemangioma, magnetic resonance imaging, ultrasound

How to cite this article:
Lakshminarayanan S. Rapidly involuting congenital hemangioma with complete resolution In Utero. Indian J Paediatr Dermatol 2019;20:57-9

How to cite this URL:
Lakshminarayanan S. Rapidly involuting congenital hemangioma with complete resolution In Utero. Indian J Paediatr Dermatol [serial online] 2019 [cited 2020 Sep 18];20:57-9. Available from: http://www.ijpd.in/text.asp?2019/20/1/57/247543

  Introduction Top

Hemangioma is an abnormal collection of blood vessels in the skin. They are common in Caucasian female infants, premature babies, twins, advanced maternal age, and families with infantile hemangioma. The exact cause is not known and may have a hereditary component.

Hemangioma is of different types. Capillary hemangioma, cavernous hemangioma, compound hemangioma, lobular hemangioma (pyogenic granuloma), and infantile hemangioma (IH). IH are the most common soft-tissue tumors of infancy, which are seen in 10%–12% of infants in the 1st year of life. They appear in the first few weeks and go through a proliferative phase up to 1 year. They then involute over a period of 3–10 years. The majority of hemangioma does not need any treatment unless they affect breathing, vision and are large in size.

Congenital hemangioma is seen at birth and behaves differently to infantile hemangioma. They are thought to arise from the placental tissue. Rapidly involuting congenital hemangioma (RICH) is a rare entity and is included in the Orphanet. A few case reports have been published from different parts of the world. Our case of RICH was detected in the antenatal period, but a diagnosis was made retrospectively.

  Case Report Top

A 11-week-old male Caucasian infant was referred to Dermatology Outpatient Department by a pediatrician for a lesion on the back, present since birth. It had remained stable and was asymptomatic. A routine ultrasound in the antenatal period at 20 weeks gestation showed a soft-tissue swelling on the back of the fetus[1] [Figure 1]. An magnetic resonance imaging (MRI) was carried out at 22 weeks of gestation, which revealed a soft-tissue swelling suggestive of hemangioma[2] [Figure 2]. A repeat ultrasound at 32 weeks did not reveal the swelling[3] [Figure 3]. He was delivered by cesarean section due to breech presentation. At birth, he had a skin-colored plaque on the back at the site of the soft-tissue swelling depicted on ultrasound and MRI. A repeat ultrasound performed 3 months after birth was normal.
Figure 1: Ultrasound at 20 weeks showing soft-tissue lesion adjacent but separate to spine measuring 1.7 cm × 0.7 cm × 1.6 cm. It is located in the thorax. The spine appears to be intact

Click here to view
Figure 2: Magnetic resonance imaging T2-weighted parasagittal images through the area of concern. There is a subcutaneous mixed signal intensity lesion

Click here to view
Figure 3: Ultrasound at 32 weeks of gestation showing no swelling

Click here to view

Clinical examination revealed 3 cm × 1 cm skin-colored atrophic plaque on the back with a rim of white scarring[4] [Figure 4]. Dermatoscopy did not reveal any residual telangiectasia. He was reviewed at 3 and 6 months later. The plaque had flattened and became less conspicuous.
Figure 4: Atrophic plaque on the back

Click here to view

  Discussion Top

In 2014, the International Society for the Study of Vascular Anomalies classified vascular tumors into benign, locally aggressive, and malignant.[1] The benign lesions are the hemangioma of infancy, congenital hemangioma, tufted angioma, spindle cell hemangioma, and pyogenic granuloma. Congenital hemangioma is a rare, benign vascular tumor that shows significant intrauterine growth and rapidly involutes in the 1st year of life. Histologically, they are GLUT1 negative in contrast to infantile hemangioma. They are usually cutaneous but can be found in the viscera. Complications are hemorrhage, transient heart failure, and transient coagulopathy.[2] They are typically purplish with a halo around the lesion and fully formed at birth. Ultrasound imaging shows a high flow within the lesion. RICH that has regressed completely is seen as a pale atrophic patch with dilated veins.[3] They only increase in size if there is bleeding into the tumor. Somatic activating mutations of GNAQ and GNA11 have been associated with congenital hemangioma.[4]

Congenital hemangiomas are of three types: Partially involuting congenital hemangioma is fully formed at birth and involutes only partially. Noninvoluting congenital hemangioma is formed at birth and never involutes. RICH is rare. The prevalence and etiology are unknown.[5] RICH is seen equally in boys and girls. Intrauterine growth may be demonstrated by Doppler ultrasonography which reveals a highly vascular and fast -flow mass as early as 12 weeks of gestation. Ultrasound may also show calcifications.[6] Involution of the lesion in the follow-up ultrasound confirms the diagnosis of RICH.[7] RICH has area of inhomogeneity and large flow voids on the MRI,[8] When clinical and radiological data are ambiguous, a biopsy is indicated in the newborn. Histology shows small-to-large lobules of capillaries with moderately plump endothelial celia and pericytes. The lobules may be surrounded by abundant fibrous tissue.[9] The prognosis is good and masterly inactivity is the usual management. The residual lesion may need lipofilling depending on the site. When they cause functional impairment, may need to be removed surgically.[10] A similar case has been reported in 2010 by Ozcan from Turkey.[7]


We would like to acknowledge Dr. E. Whitby, Senior Lecturer/Honorary Consultant, University of Sheffield, for her help with MRI and Dr. M. Kaduthodil Consultant Radiologist, Doncaster Royal Infirmary, Doncaster, for his help with ultrasound images.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

National Cancer Institute US. Childhood Vascular Tumors Treatment (PDQ). 2002 Health Professional Version PDQ Paediatric Treatment Editorial Board. Bethesda MD: National Cancer Institute US; 2017.  Back to cited text no. 1
Vildy S, Macher J, Abasq-Thomas C, Le Rouzic-Dartoy C, Brunelle F, Hamel-Teillac D, et al. Life-threatening hemorrhaging in neonatal ulcerated congenital hemangioma: Two case reports. JAMA Dermatol 2015;151:422-5.  Back to cited text no. 2
Maguiness S, Uihlein LC, Lieang MG, Kozakewich H, Mulliken JB. Rapidly involuting congenital hemangioma with foetal involution. Paediatr Dermatol 2015;32:321-6.  Back to cited text no. 3
Ayturk UM, Couto JA, Hann S, Mulliken JB, Williams KL, Huang AY, et al. Somatic activating mutations in GNAQ and GNA11 are associated with congenital hemangioma. Am J Hum Genet 2016;98:789-95.  Back to cited text no. 4
Orphanet: Rapidly Involuting Congenital Hemangioma. Available from: http://www.orpha.net/consort/cgi-bin/OC. [Last accessed on Sep 2017].  Back to cited text no. 5
Rogers M, Lam A, Fischer G. Sonographic findings in a series of rapidly involuting congenital hemangiomas (RICH). Pediatr Dermatol 2002;19:5-11.  Back to cited text no. 6
Ozcan UA. Rapidly involuting congenital hemangioma: A case of complete prenatal involution. J Clin Ultrasound 2010;38:85-8.  Back to cited text no. 7
Berenguer B, Mulliken JB, Enjolras O, Boon LM, Wassef M, Josset P, et al. Rapidly involuting congenital hemangioma: Clinical and histopathologic features. Pediatr Dev Pathol 2003;6:495-510.  Back to cited text no. 8
Lee PW, Frieden IJ, Streicher JL, McCalmont T, Haggstrom AN. Characteristics of noninvoluting congenital hemangioma: A retrospective review. J Am Acad Dermatol 2014;70:899-903.  Back to cited text no. 9
Enjolras O, Mulliken JB, Boon LM, Wassef M, Kozakewich HP, Burrows PE, et al. Noninvoluting congenital hemangioma: A rare cutaneous vascular anomaly. Plast Reconstr Surg 2001;107:1647-54.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

  In this article
Case Report
Article Figures

 Article Access Statistics
    PDF Downloaded89    
    Comments [Add]    

Recommend this journal