|Year : 2019 | Volume
| 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 Publication||14-Dec-2018|
Dr. Sabitha Lakshminarayanan
Department of Dermatology, Lincoln Suite, Armthorpe Lane, Doncaster Royal Infirmary, Doncaster, DN2 5LT
Source of Support: None, Conflict of Interest: None
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 2019 Apr 24];20:57-9. Available from: http://www.ijpd.in/text.asp?2019/20/1/57/247543
| Introduction|| |
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|| |
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 [Figure 1]. An magnetic resonance imaging (MRI) was carried out at 22 weeks of gestation, which revealed a soft-tissue swelling suggestive of hemangioma [Figure 2]. A repeat ultrasound at 32 weeks did not reveal the swelling [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|
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|Figure 2: Magnetic resonance imaging T2-weighted parasagittal images through the area of concern. There is a subcutaneous mixed signal intensity lesion|
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Clinical examination revealed 3 cm × 1 cm skin-colored atrophic plaque on the back with a rim of white scarring [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.
| Discussion|| |
In 2014, the International Society for the Study of Vascular Anomalies classified vascular tumors into benign, locally aggressive, and malignant. 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. 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. 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.
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. 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. Involution of the lesion in the follow-up ultrasound confirms the diagnosis of RICH. RICH has area of inhomogeneity and large flow voids on the MRI, 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. 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. A similar case has been reported in 2010 by Ozcan from Turkey.
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.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]