Dermatology Online Journal
Multiple cutaneous hemangiomas accompanied by hepatic hemangioma
- Author(s): Douri, Thaer
- et al.
Multiple cutaneous hemangiomas accompanied by hepatic hemangiomas
Ministry of Health, Syria-Hama. firstname.lastname@example.org
Dermatology Online Journal 11 (1): 21
Hemangioma of infancy is the most common tumor in infancy. The incidence in the newborn nursery is between 1.0 percent and 2.6 percent in white, black, and Japanese infants. An incidence of 10-12 percent has been reported in white children by age 1 . It is characterized by an initial phase of rapid proliferation, followed by slow involution, often leading to complete regression.
Although most of the tumors are small and innocuous, some may be life- or function-threatening, or have associated structural congenital anomalies . We describe a case of multiple hemangiomas with an extracutaneous manifestation.
|Figure 1||Figure 2|
A 3-month-old girl has onset of multiple hemangiomas at age 1 month. The lesions begin as red papules that then enlarge. They develop on widespread body surfaces (scalp, face, chest, abdomen, back, and extremities).
Physical examination reveals the presence of approximately fifty cutaneous hemangiomas. She is also noted to have moderate hepatomegaly but an otherwise-normal examination. An ultrasound examination of the abdomen reveals multiple hepatic hemangiomas (Fig. 3). She is also noted to have a hemoglobin of 8. Her stool is negative for occult blood; the chest x-ray and other routine laboratory tests are normal.
The patient is diagnosed with multiple cutaneous hemangiomas accompanied by hepatic hemangiomas; she is treated with prednisone (2 mg per kg per day) without benefit. She develops congestive heart failure 2 weeks later and expires.
Hemangioma of infancy (HOI) is the most common benign tumor of childhood. It is characterized by an initial phase of rapid proliferation followed by slow involution, often leading to complete regression.
On the basis of clinical features, HOI can usually be classified as localized, segmental, indeterminate, or multifocal. Although 80 percent of HOI occur in isolation, 20 percent are multiple. The term hemangiomatosis has been used to describe the uncommon presentation of few to hundreds of small, generalized hemangiomas, often with visceral (usually hepatic) involvement .
The presentation of multiple neonatal hemangiomas may be further divided into benign and disseminated forms. Stern et al. originally proposed the term benign neonatal hemangiomatosis to described infants with multiple cutaneous hemangiomas lacking visceral involvement [2, 3]. The presence of visceral hemangiomas can signify a relatively poor prognosis for the affected infant, especially hemangiomas of the liver. The liver is the most common extracutaneous site of hemangiomas, but virtually any organ may be affected, including brain, intestine, oral mucosa, eyes, spleen, and kidney. In 87 percent of affected neonates, cutaneous hemangioma are the first sign of disease.
Our patient has multiple hepatic hemangiomas. Hepatic hemangiomas may occur without cutaneous involvement. A common presentation of hepatic hemangiomas is hepatomegaly and congestive heart failure. Anemia or thrombocytopenia secondary to the increased blood flow may also be seen. Yoon et al. described 115 patients with hepatic hemangiomas; nearly half were asymptomatic . In symptomatic patients, abdominal pain or discomfort are the most common presenting symptom. At our institution, the diagnosis of hemangioma is established by ultrasonographic studies in 57 percent of patients, by CT scan in 73 percent of patients, and by MRI in 84 percent of patients tested. In patients with large tumors considered for resection, direct angiography or, more recently, CT angiography, confirmed the diagnosis in twenty-seven of twenty-nine patients (93 %) . Our patient initially has only anemia, but 2 weeks later she developed congestive heart failure and expired.
Systemic corticosteroid therapy is the treatment of choice for neonatal hepatic hemangiomas (HHE), but a significant number of infants fail to respond. Other reported pharmacologic options include α-interferon, vincristine, and cyclophosphamide. Surgical interventions include embolization and hepatic artery ligation. Bachmann et al. described a case of a neonate with congestive heart failure associated with a localized HHE treated successfully by selective ligation of the left hepatic artery branch supplying the tumor under perioperative-ultrasound Doppler control . A review of the literature showed thirty-five cases of HHE treated by hepatic artery ligation with a survival rate of 80 percent . This approach is very difficult for our patient because she has multiple liver hemangiomas. Other options include hepatic resection or even liver transplantation. Enucleation is performed in thirty-one (60 %) of the fifty-two patients who underwent surgical resection ; sixty-three patients were observed. Postoperative complications occurred in thirteen patients (25 %), and there were no perioperative deaths. Of the patients with symptoms before resection, 96 percent had resolution of symptoms after operation . Without treatment, mortality with hepatic hemangioma is reported to be as high as 80 percent, but early aggressive treatment can lower mortality to approximately 20 percent . Our attempt to treat with prednisone is unsuccessful, perhaps because it is started late in her course.
References1. Dermatology in General Medicine. Fitzpatrick (CD ROM fifth edition)
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3. Stern JK, Wolf JE Jr, Jarratt M. Benign neonatal hemangiomatosis. J Am Acad Dermatol. 1981 Apr;4(4):442-5. PubMed
4. Yoon SS, Charny CK, Fong Y, Jarnagin WR, Schwartz LH, Blumgart LH, DeMatteo RP. Diagnosis, management, and outcomes of 115 patients with hepatic hemangioma. J Am Coll Surg. 2003 Sep;197(3):392-402. PubMed
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