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DIFFUSE NEONATAL HEMANGIOMATOSIS: IMAGING FINDINGS IN TWO PATIENTS

Philip Goodman *+, Rodrigo Dominguez*, and Mauricio Castillo* *University of Texas Medical Branch, Department of Radiology, Galveston, TX 77550, and *The University of Texas Medical School at Houston, Department of Radiology, 643 1 Fannin, 2.134 MSB, Houston, TX 77030

(Received 18 March 1991; Revised 18 November 1991)

Abstract-Diffuse neonatal hemangiomatosis is a rare condition in which cutaneous and visceral hemangiomas may coexist. Intra-abdominal involvement occurs commonly and, if untreated, may result in high-output cardiac failure and other life-threatening conditions. We report two neonates with this disease and discuss the radiographic findings and the role of vascular embolization in the treatment of hepatic hemangiomatosis.

Key Words: Hemangioma, Hemangiomatosis, Ultrasound

INTRODUCIION

Diffuse neonatal hemangiomatosis (DNH) is an often fatal disease characterized by multiple cutaneous and visceral hemangiomas. The most commonly involved visceral organs are the liver, gastrointestinal tract, lungs, and CNS ( 1). Extensive arteriovenous shunting through the hemangiomas may lead to high-output cardiac fail- ure. Other complications relate to the specific organs affected and include gastrointestinal bleeding, dyspnea, and seizures (2). Imaging studies are important in eval- uating the extent of visceral involvement in this disease. We present two cases of intra-abdominal hemangio- matosis, discuss the radiographic findings, and briefly review the role of embolization in one of our patients with hepatic involvement.

CASE REPORTS

Case # 1 A 2-mo-old girl with cutaneous hemangiomas was

transferred to our institution for evaluation of multiple hemangiomas of the liver found at exploratory lapa- rotomy. She had been well until 2 wk previously when she developed feeding difficulty and became dehydrated and febrile.

Due to increasing cyanosis and massive hepato- megaly, embolization of the common hepatic and in- ferior phrenic arteries using gelfoam and Gianturco coils was performed. Although arteriovenous shunting decreased immediately after this procedure (Fig. IA and 1 B), the patient soon developed disseminated in- travascular coagulation (DIC), ventricular fibrillation, decreased urine output, and seizures. Hepatic artery

+ To whom correspondence should be addressed.

ligation was performed 2 days after the embolization, but the coagulopathy persisted and the patient died the next day.

Autopsy revealed an enlarged liver with multiple capillary hemangiomas, focal hemorrhage, and infarc- tion. Capillary hemangiomas were also noted in the skin, pancreas, uterus, and right ovary. Hemorrhage was present in the lungs, small and large intestine, kid- neys, adrenals, urinary bladder, and peritoneal cavity.

Case #2 A 24-wk gestation stillborn fetus was born to a

24-yr-old mother. Ultrasound performed 1 wk earlier showed fetal ascites, pleural effisions, small pericardial effusion, diffuse soft tissue thickening, scalp edema, and bilateral bowing of the femurs (Fig. 2A and 2B). A plain radiograph of the stillborn fetus confirmed these findings and also revealed giantism of the left upper extremity (Fig. 2C).

Autopsy showed numerous hemangiomas involv- ing the gastrointestinal tract, mesentery, pancreas, kid- neys, retroperitoneum, pelvis, mediastinum, right pulmonary hilum, axillae, and extremities. No he- mangiomas were present in the liver, brain or skin.

DISCUSSION

Hemangiomas occur in 10% of newborns and are most often solitary cutaneous lesions (3). Multiple he- mangiomas limited to the skin are less common and are referred to as benign neonatal hemangiomatosis. These hemangiomas usually regress spontaneously after a limited period of growth and may involute completely by several months to 2 yr of age (4).

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118 Computerized Medical Imaging and Graphics March-April/ 1992, Volume 16, Number 2

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0.8 Fig. 1. Parenchymal phase of hepatic arteriogram. (a) Pre- embolization film shows pooling of contrast in multiple he- patic hemangiomas. (b) Postembolization film shows marked decrease in pooling. Gianturco coils are seen in the hepatic

artery (arrows).

Rarely, multiple hemangiomas involve both the skin and visceral organs. This condition, known as dif- fuse neonatal hemangiomatosis (DNH), is defined as the presence of papular cutaneous and visceral heman- giomas in the neonatal period, involvement of three or more organ systems, and absence of malignancy in the hemangiomas (5). Although any area of the body may be affected, the most commonly involved visceral organs are the liver (60- 100% of cases), gastrointestinal tract (50%), lungs (SO%), and CNS (30-50%). Rarely, the liver may be spared (Case #2) (5).

Cutaneous hemangiomas are often a clue to the presence of visceral hemangiomas. However, heman- giomatosis of visceral organs may rarely present with- out skin lesions (Case #2) (6).

In contrast to the favorable prognosis of solitary or multiple benign cutaneous hemangiomas in the newborn, DNH is potentially lethal if untreated. Death most often is related to high-output cardiac failure sec- ondary to the arteriovenous shunting in the heman- giomas (7). Consumption coagulopathy within the he- mangiomas (Kasabach-Merritt syndrome) may lead to thrombocytopenia and fatal hemorrhage (8). Other complications relate to local rather than systemic effects of hemangiomas and include gastrointestinal bleeding, dyspnea, hydrocephalus, and seizures (2).

It is important to note that congestive heart failure in DNH is usually not relieved by conservative medical therapy alone. For this reason, treatment must be aimed at decreasing hepatic arteriovenous shunting in the first few months of life until growth of the hemangiomas ceases and spontaneous involution occurs (9). To accomplish this, various methods have been used including partial hepatectomy, radiation therapy, hepatic artery ligation, and steroid therapy which presumably acts by increasing sensitivity of end arterioles to circulating vasoconstrictive agents ( 10, I 1). Hepatic artery embolization is another way to treat these lesions. Embolization with gelfoam and Gianturco coils was performed in one of our patients (Case # 1). Regretfully, the patient developed DIC follow- ing this procedure, either as a result of the extensive he- mangiomatosis or as a complication of the procedure itself The DIC persisted despite subsequent hepatic artery ligation, and the patient died.

Imaging studies useful in the assessment of visceral hemangiomas include computed tomography (CT), ul- trasound, radionuclide scans, and magnetic resonance imaging (MRI) (12, 13). Since visceral hemangiomas rarely contain phleboliths, plain films are not sensitive in their detection. On CT, hepatic hemangiomas may demonstrate the classical pattern of peripheral enhance- ment with central filling-in. This finding is not seen in all hemangiomas, though when seen it is suggestive but not pathognomonic of hemangioma (14). Liver heman- giomas are echogenic by ultrasound, but again this finding

Diffuse neonatal hemangiomatosis ??P. GOODMAN, R. D~MINGUEZ, and M. CASTILLO 119

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(b)

Fig. 2. (a) Transverse plane of obstetrical sonogram with computer enhancement of the fetal abdominal wall (dotted line). This shows edematous thickening of soft tissues (arrows) and ascites (curved arrow) surrounding the liver and bowel. (b) Coronal plane of the fetal pelvic region demonstrates foreshortening and bowing of the right femur (arrows). (c) Plain film of the stillborn fetus shows generalized edema, giantism of the left upper extremity, bilateral femoral bowing,

and diffuse bony demineralization.

120 Computerized Medical Imaging and Graphics March-April/ 1992, Volume 16, Number 2

is nonspecific. Doppler analysis of hemangiomas shows low-frequency arteriaI and venous signals ( 15). Color flow imaging is also promising, but at this time experience is limited ( 15). On nuclear studies utilizing technetium-la- belIed red blood cells, increased uptake can be seen in hemangiomas on both conventional planar scans and single photon emission computed tomography (SPECT) ( 14). The high signal intensity of hemangiomas on heavily TZweighted magnetic resonance (MR) images makes them easy to identify. This finding is relatively specific. Unfortunately, MR studies am often degraded by artifacts related both to respiratory and bowel motion and to the long acquisition times.

Since most untreated infants with DNH will die in the first months of life, early diagnosis is crucial. Imaging studies demonstrating hepatic and other visceral lesions in an infant with cutaneous hemangiomas, hepatomegaly, and congestive heart failure should suggest the diagnosis of DNH and treatment should be started accordingly.

SUMMARY

Diffuse neonatal hemangiomatosis is a rare life- threatening disease consisting of multiple heman- giomas of the skin and visceral organs. We have pre- sented two patients with this condition and reviewed the clinical and radiographic findings.

Acknowledgments-We gratefully acknowledge the assistance of Kim Sickler, M.D., in obtaining clinical and pathological correlation for the cases presented in this manuscript. We would also like to thank Lila Hecker, Katie Isch, and Vicki McDowell, medical secretaries, for their assistance in the preparation of the manuscript.

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Ahout the Author--PHILIP GOODMAN was born in Binghamton, NY, in 1954. He received his B.A. degree from Cornell University in 1976 and his M.D. degree from the University of Rochester in 1980. He was a resident in diagnostic radiology at Rochester General Hospital in Rochester, NY, from 198 1 to 1984 and a fellow in gastrointestinal radiology at the Hospital of the University of Pennsylvania in Phil- adelphia during 1986 and 1987.

From 1987 to 1990, Dr. Goodman served as an Assistant Pro- fessor of Radiology at the University of Texas Health Science Center at Houston, and Chief of Gastrointestinal Radiology at Hermann Hospital. He is currently an Assistant Professor of Radiology and Director of Abdominal Imaging at the University of Texas Medical Branch in Galveston, TX.

About the Author-RODRtcio DOMINGUEZ-MAYORAL, a native of Spain, is an Associate Professor of Pediatrics and Radiology at the Universitv of Texas Health Science Center at Houston. He is the last disciple of the founder of pediatric radiology, Dr. John Caffey.

Dr. Dominguez is mostly involved in neonatal imaging and he has just published a book on this field of radiology about the diagnosis of diseases in premature infants.

About the Author-MAURICIO CASTILLO was born in Guatemala City, Guatemala. He received his M.D. degree at the University of San Carlos in Guatemala. From 1983 to 1984 he was a visiting fellow at the University of Texas M.D. Anderson Cancer Center. He did his residency in Diagnostic Radiology at the University of Miami School of Medicine and then went on to do a fellowship in Neuro- radiology at Emory University. Currently, he is an Assistant Professor of Radiology at the University of Texas Medical School at Houston and Assistant Chief of Radiology Service at LBJ General Hospital in Houston, TX.