Benign Neonatal Hemangiomatosis

Alexander K. C. Leung, M.B.B.S., F.R.C.P.(U.K. and Irel.), F.R.C.P.C.H., F.R.C.P.C.,

and Massoud Rafaat, M.D., F.R.C.P.C., D.T.M.

Department of Pediatrics, University of Calgary, and Alberta Children’s Hospital, Calgary, Alberta, Canada

Abstract: An infant with benign neonatal hemangiomatosis is described.Examination for internal hemangiomas was negative. The patient had com-plete resolution of the lesions by 2 years of age.

Hemangiomas occur in approximately 2% of newb-orns and up to 10% of infants by 1 year of age (1). Themajority of these are solitary (2). Benign neonatalhemangiomatosis is a rare self-limited disease charac-terized by multiple hemangiomas occurring exclusively inthe skin (3). This is in contrast to diffuse neonatalhemangiomatosis, another rare disorder, characterizedby multiple cutaneous and visceral hemangiomas andassociated with a poor prognosis (4). Most of thereported cases of neonatal hemangiomatosis are com-plicated by visceral involvement (5). We describe a childwith benign neonatal hemangiomatosis whose cutaneouslesions resolved by 2 years of age.

CASE REPORT

An infant girl was born at term to a gravida 2, para 1,24-year-old mother following an uncomplicated preg-nancy and normal spontaneous vaginal delivery. Thefather was also healthy. There was no history of consan-guinity. Family history was unremarkable. Apgar scoreswere 7 at 1 minute and 9 at 5 minutes. Birth weight was3420 g, length 51 cm, and head circumference 35.5 cm.

On examination, the rectal temperature was 37 �C,heart rate 102 beats/minute, and respiratory rate 38/minute. Approximately 80 red, superficial hemangiomasranging from 0.5 to 3 mm in diameter were scattered overthe trunk, extremities, scalp, and face (Figs. 1 and 2).None were seen in the intraoral, genital, or conjunctivalareas. The anterior fontanelle measured 2 cm · 3 cm

and the posterior fontanelle 1 cm · 1 cm; both fonta-nelles were soft. Fundoscopic examination was normal.There was no hepatosplenomegaly. The rest of the ex-amination was unremarkable.

The following laboratory test results were normal:complete blood cell count, serum electrolytes, urinalysis,and stool guaiac. Other investigations including chestradiograph, electrocardiogram, abdominal ultrasound,and computed tomography (CT) of the brain, thoraciccavity, and abdomen were all normal.

No treatment was implemented. The patient wasfollowed at monthly intervals up to 6 months of age andonce every 2–3 months thereafter. The lesions increasedin size and number until 3 months of age, at which timethe infant had more than 100 hemangiomas ranging from0.5 to 6 mm in diameter. The lesions started to involute at6 months of age. By 2 years of age, no hemangiomascould be found. The child was growing and developingnormally.

DISCUSSION

Benign neonatal hemangiomatosis is a nonheritabledisorder characterized by numerous cutaneous heman-giomas following a benign course, with spontaneousresolution and without clinically evident visceral lesions(5). Girls are more often affected (3,6). Typically theselesions have their onset at birth (61%) or within the firstmonth of life (86%) (7). They increase rapidly in size andnumber for the first few months and then start to involute

Address correspondence to Alexander K. C. Leung, M.B.B.S.,#200, 233 16th Ave. NW, Calgary, Alberta T2M 0H5, Canada, ore-mail: aleung@ucalgary.ca.

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(7). In most cases the hemangiomas resolve spontane-ously during the first 2–3 years of life (8,9). The lesionsare bright red to purple and range in size from that of apinhead to 20 mm (5,10). Histologically they are mul-tilobular vascular proliferations with thin-walled vascu-lar channels lined by normal endothelium (5).

It is important to differentiate benign neonatalhemangiomatosis from diffuse neonatal hemangioma-tosis, which has a high mortality rate (1,11). Extensivevisceral involvement is a poor prognostic sign (2,5).Death usually occurs within a few months of life. Themost common causes of death are high-output conges-tive heart failure as a result of arteriovenous shunting inthe liver and lungs, and hemorrhage from hemangiomasin the upper respiratory or gastrointestinal tract (2,4,5).Other serious complications include obstructive jaundiceand central nervous system sequelae caused by masseffect (8,9,11,12). The skin (100%), liver (64%), brain(52%), gastrointestinal tract (52%), lungs (52%), oralcavity (44%), and eyes (32%) are frequently affected (3).

The spleen, pancreas, heart, kidneys, and adrenal glandsmay also be involved (2).

In infants with neonatal hemangiomatosis, clinicalevaluation should be done to determine the presence ofvisceral involvement. Clinically the cutaneous featuresof diffuse neonatal hemangiomatosis cannot be distin-guished from those seen in benign neonatal hemangio-matosis. The majority of children with diffuse neonatalhemangiomatosis have numerous lesions (3). Althoughuncommon, visceral involvement may occur with a fewcutaneous hemangiomas (6). Although involvement ofthe oral, genital, and conjunctival mucous membranesfavors a diagnosis of diffuse neonatal hemangiomatosis,benign neonatal hemangiomatosis with involvement ofthe vaginal and oral mucous membranes has been re-ported (13). The presence of gastrointestinal bleeding,hematuria, anemia, petechiae, jaundice, hepatomegaly,or abnormal neurologic signs suggests the diagnosis ofdiffuse neonatal hemangiomatosis (3).

Diagnostic evaluation of an infant with hemangio-matosis may include complete blood cell count to detect

Figure 2. Multiple superficial hemangiomas over the lowerextremities and lower abdomen.Figure 1. Numerous superficial hemangiomas scattered

over the trunk, face, and extremities characteristic of neonatalhemangiomatosis.

162 Pediatric Dermatology Vol. 20 No. 2 March/April 2003

anemia and thrombocytopenia, urinalysis to detecthematuria, stool guaiac test to check for gastrointestinalbleeding, chest radiography and electrocardiogram ifheart failure is suspected, abdominal ultrasonographyfor organomegaly, and CT scan or magnetic resonanceimaging (MRI) to detect intracranial lesions if involve-ment of the central nervous system is a possibility (14).Physicians must use their clinical acumen in the choice ofdiagnostic studies.

Our patient had benign neonatal hemangiomatosis, asevidenced by the lack of visceral involvement, the rapidspontaneous regression of cutaneous hemangiomas, andthe excellent outcome. Although the literature containsa paucity of case reports on benign neonatal hemangio-matosis (1,2,5,10,13), the condition is probably morecommon than is presently appreciated.

ACKNOWLEDGMENTS

The authors are grateful to Dianne Leung for her expertsecretarial assistance and Sulakhan Chopra of the Uni-versity of Calgary Medical Library for help in the pre-paration of this article.

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