Fetal Hemangioma
Sonographic and Color Flow Doppler Findings Dorothy I. Bulas, MD, Dennis Johnson, MD; Jill Fonda Allen, MS, Sudesh Kapur, MD
Hemangiomas are the most common of all congenital anomalies.1 The sonographic appearance and Doppler characteristics are variable, depending on the location, size of vessels, amount of arteriovenous shunting, and degree of endothelial proliferation. We report a case of an extracranial mass followed prenatally by sonogra~ phy and color Doppler flow imaging. At delivery and subsequent surgical resection, a mixed capillary cavernous hemangioma was confirmed pathologically.
Case Report A 26 year old woman (gravida 2, para 1) had an initial sonogram at 15 weeks of gestation for dating. The examination was reported to be normal. A repeat examination of the fetus at 30 weeks identified a mass arising from the right occipital region. The diagnosis of an encephalocele was made, and she was referred to our laboratory for amniocentesis and further evaluation. The prenatal course had been uncomplicated. Her medical history was not significant for any diseases, and she denied any use of medications or exposure to drugs during the pregnancy. The family history also was unremarkable. Sonogram at our institution showed a single intrauterine pregnancy with an estimated gestational age of 31 weeks. A
Received November 11, 1991, from the Departments of Diagnostic Imaging and Radiology (D.l.B.), Pediatrics (D.l.B., S.K.), Neurosurgery (D.J.), Genetics (J.F.A.), and Pathology (S.K.), Children's National Medical Center and the Ceorge Washington University School of Medicine and Health Sciences, Washington, DC. Revised manuscript accepted for publication March 5, 1992. Address correspondence and reprint requests to Dorothy 1. Bulas, MD, Department of Diagnostic Imaging, Children's National Medical Center, 111 Michigan Avenue, N.W., Washington, DC 20010.
heterogeneous echogenic mass measuring 3 X 2 x 1.6 cm was identified in the right occipital region (Fig. 1). Color Doppler flow imaging identified vessels at the periphery of the mass. The center of the mass, however, was avascular (Fig. 2). No skull or spinal defect could be identified, and no intracranial abnormalities were noted. Similarly, there was no evidence of a cleft lip, and the kidneys were free of cystic lesions. Fetal size was appropriate for dates, and no other fetal abnormalities were identified. Amniocentesis at 31 weeks documented a normal 46,XY karyotype and normal amniotic fluid alpha-fetoprotein (AFP) levels. The results of the sonogram, together with the normal cellular and bio· chemical findings, were thought to be most consistent with a benign tumor. Differential diagnosis included a dermoid, hemangioma, or lipoma. Follow-up examinations demonstrated no change in size of the echogenic mass. At 39 weeks a cesarean section was performed. A full-tenn male infant weighing 3.8 kg was delivered without complications. A 3 cm soft, compressible, mobile mass was found in the right occiput. The skin over the mass was slightly mottled. The infant's face was sym· metric, without midline defects. No other anomalies were identified. Neurologic examination was within normal limits. At 3 months after birth the mass was removed. The external surface of the mass was smooth, with the cut surface revealing prominent vascular spaces (Fig. 3). Microscopic sections revealed lobules of capillary-like vascular structures intermixed with dilated venous channels, consistent with a hemangioma. Extensive endothelial proliferation was present in focal regions.
DISCUSSION Hemangiomas, which arise from venules, arterioles, or capillaries, are one of the most common tumors of childhood. Any organ can be affected, but the skin is
© 1992 by the American Institute of Ultrasound in Medicine• J Ultrasound Med 11:499-501, 1992 • 0278-4297/92/$3.50
500
FETAL HEMANGJOMA
Figure 1 Transverse image o( the cranium demonstrates an echogenic mass adjacent to the occiput. Apparent skull defect (arrow) is actually an artifact caused by reflective shadowing of the acoustic beam.
the most common site. Appearance can vary, depending on the type of vessels present and the amount of endothelial proliferation. Capillary hemangiomas usually lie in the epidermal layer, whereas cavernous hemangiomas lie deeper in the dermis and contain large venous channels lined with smooth muscle endothellum. Sonographic features range from a homo· geneousJy echogenic pattern similar to that of the placenta to large, thick-walled cystic lesions.M Calcifications may be present. 6 Classification of angiomas is complicated by the variety of vascular patterns and diverse hemodynamics. Doppler flow patterns vary depending on the size of the vessels and degree of arterial venous shunting. Several cases of hemangioma examined prenatally with Doppler sonography have been reported previously.3·4·6 Using continuous wave Doppler velodmetry, both Smith and colleagues and Lasser and coworkers noted a low resistance wave pattern in cystic areas within a cavernous hemangioma identified pre• natally. 4 •6 Using a pulsed Doppler (duplex) system, arterial and venous pulsations were reported in a he· mangioendothelioma examined prenatally by McGahan and Schneider. 7 Pennell and Baltarowkh described central pulsating vessels within confluent cystic areas in a fascia] hemangioma. 3 Color flow Doppler
J Ultrasound Med 11:499- 501, 1992
Figure 2 Color flow Doppler imaging demonstrated flow at the base of the lesion (arrow), with an avascular center.
Figure 3 Gross specimen demonstrates a solid-appearing mass containing several prominent vascular spaces.
imaging has not yet been described in the evaluation of hemangioma prenatally. Its use, however, has been described with orbital hemangiomas, ocular capillary hemangiomas being highly vascular and cavernous hemangiomas being relatively avascular. 8•9 When an extracranial mass is identified, the diagnosis of an encephalocele must be excluded. An encephalocele may appear sonographically as a thin-
J Ultrasound Med 11:499-501, 1992
walled cyst, as a solid mass with a gyral pattern continuous with the cranium, or as a combined cystic and solid mass. 10 When no calvarial defect is identified, the differential diagnosis for a juxtacranial mass should include teratomas, hemangiomas, mesenchymal sarcomas, and cystic hygromas. 11 Teratomas may be solid or heterogeneous, with no demonstrable gyral pattern. Cystic hygroma and lymphangioma are more typically cystic and have septa. Although previous investigators speculated that the Doppler demonstration of low vascular resistance could differentiate vascular lesions from neoplasms prenatally, this was not true in our case. 4 Understanding the variable sonographic and Doppler characteristics of hemangioma allows for more accurate prenatal diagnosis and management.
REFERENCES 1. Edgerton MT: The treatment of hemangiomas. Ann Surg 183:517, 1976 2. Bell RL: US of hemangioma of the neck of a newborn. J Tenn Med Assoc 71:289, 1978 3. Pennell RG, Baltarowich OH: Prenatal sonographic di-
BULAS ET AL 501
agnosis of a fetal facial hemangioma. J Ultrasound Med 5:525, 1986 4. Smith LG, Carpenter RJ, Gonsoulin W, et al: Prenatal diagnosis of a chest wall mass with ultrasonogr aphy and Dopplervelocimetry. AmJ Obstet Gynecol 163:567, 1990 5. Grundy H, Glasmann A, Burlbaw J, et al: Hemangiom a presenting as a cystic mass in the fetal neck. J Ultrasound Med 4:127, 1985 6. Lasser D, Preis 0, Dor N, et al: Antenatal diagnosis of giant cystic cavernous hemangioma by Doppler velocimetry. Obstet Gynecol 72:476, 1988 7. McGahan JP, Schneider JM: Fetal neck hemangioe ndo¥ thelioma with secondary hydrops fetalis: Sonographic diagnosis. J Clin Ultrasound 14:384, 1986 8. Folley WO: Color Doppler Flow Imaging. Boston, An· dover Medical Publishers, 1991, 57 9. Sklar EL, Quencer RM, Byrne SF, et al: Correlative study of the computed tomographic, ultrasonographic, and pathological characteristics of cavernous versus capillary hemangiomas of the orbit. J Clin Neuro-Oph thalmol 6:14, 1986 10. Nyberg DA, Mahony BS, Pretorius DH: Diagnostic Ultrasound of Fetal Anomalies: Text and Atlas. Chicago, Year Book, 1990, p 153 11. Sabbagha RE, Tamura RK, Dal Compo S, et al: Fetal cranial and craniocervical masses: Ultrasound characteristics and differential diagnosis. Am J Obstet Gynecol 138:511, 1980
Sonographic and Color Flow Doppler Findings Dorothy I. Bulas, MD, Dennis Johnson, MD; Jill Fonda Allen, MS, Sudesh Kapur, MD
Hemangiomas are the most common of all congenital anomalies.1 The sonographic appearance and Doppler characteristics are variable, depending on the location, size of vessels, amount of arteriovenous shunting, and degree of endothelial proliferation. We report a case of an extracranial mass followed prenatally by sonogra~ phy and color Doppler flow imaging. At delivery and subsequent surgical resection, a mixed capillary cavernous hemangioma was confirmed pathologically.
Case Report A 26 year old woman (gravida 2, para 1) had an initial sonogram at 15 weeks of gestation for dating. The examination was reported to be normal. A repeat examination of the fetus at 30 weeks identified a mass arising from the right occipital region. The diagnosis of an encephalocele was made, and she was referred to our laboratory for amniocentesis and further evaluation. The prenatal course had been uncomplicated. Her medical history was not significant for any diseases, and she denied any use of medications or exposure to drugs during the pregnancy. The family history also was unremarkable. Sonogram at our institution showed a single intrauterine pregnancy with an estimated gestational age of 31 weeks. A
Received November 11, 1991, from the Departments of Diagnostic Imaging and Radiology (D.l.B.), Pediatrics (D.l.B., S.K.), Neurosurgery (D.J.), Genetics (J.F.A.), and Pathology (S.K.), Children's National Medical Center and the Ceorge Washington University School of Medicine and Health Sciences, Washington, DC. Revised manuscript accepted for publication March 5, 1992. Address correspondence and reprint requests to Dorothy 1. Bulas, MD, Department of Diagnostic Imaging, Children's National Medical Center, 111 Michigan Avenue, N.W., Washington, DC 20010.
heterogeneous echogenic mass measuring 3 X 2 x 1.6 cm was identified in the right occipital region (Fig. 1). Color Doppler flow imaging identified vessels at the periphery of the mass. The center of the mass, however, was avascular (Fig. 2). No skull or spinal defect could be identified, and no intracranial abnormalities were noted. Similarly, there was no evidence of a cleft lip, and the kidneys were free of cystic lesions. Fetal size was appropriate for dates, and no other fetal abnormalities were identified. Amniocentesis at 31 weeks documented a normal 46,XY karyotype and normal amniotic fluid alpha-fetoprotein (AFP) levels. The results of the sonogram, together with the normal cellular and bio· chemical findings, were thought to be most consistent with a benign tumor. Differential diagnosis included a dermoid, hemangioma, or lipoma. Follow-up examinations demonstrated no change in size of the echogenic mass. At 39 weeks a cesarean section was performed. A full-tenn male infant weighing 3.8 kg was delivered without complications. A 3 cm soft, compressible, mobile mass was found in the right occiput. The skin over the mass was slightly mottled. The infant's face was sym· metric, without midline defects. No other anomalies were identified. Neurologic examination was within normal limits. At 3 months after birth the mass was removed. The external surface of the mass was smooth, with the cut surface revealing prominent vascular spaces (Fig. 3). Microscopic sections revealed lobules of capillary-like vascular structures intermixed with dilated venous channels, consistent with a hemangioma. Extensive endothelial proliferation was present in focal regions.
DISCUSSION Hemangiomas, which arise from venules, arterioles, or capillaries, are one of the most common tumors of childhood. Any organ can be affected, but the skin is
© 1992 by the American Institute of Ultrasound in Medicine• J Ultrasound Med 11:499-501, 1992 • 0278-4297/92/$3.50
500
FETAL HEMANGJOMA
Figure 1 Transverse image o( the cranium demonstrates an echogenic mass adjacent to the occiput. Apparent skull defect (arrow) is actually an artifact caused by reflective shadowing of the acoustic beam.
the most common site. Appearance can vary, depending on the type of vessels present and the amount of endothelial proliferation. Capillary hemangiomas usually lie in the epidermal layer, whereas cavernous hemangiomas lie deeper in the dermis and contain large venous channels lined with smooth muscle endothellum. Sonographic features range from a homo· geneousJy echogenic pattern similar to that of the placenta to large, thick-walled cystic lesions.M Calcifications may be present. 6 Classification of angiomas is complicated by the variety of vascular patterns and diverse hemodynamics. Doppler flow patterns vary depending on the size of the vessels and degree of arterial venous shunting. Several cases of hemangioma examined prenatally with Doppler sonography have been reported previously.3·4·6 Using continuous wave Doppler velodmetry, both Smith and colleagues and Lasser and coworkers noted a low resistance wave pattern in cystic areas within a cavernous hemangioma identified pre• natally. 4 •6 Using a pulsed Doppler (duplex) system, arterial and venous pulsations were reported in a he· mangioendothelioma examined prenatally by McGahan and Schneider. 7 Pennell and Baltarowkh described central pulsating vessels within confluent cystic areas in a fascia] hemangioma. 3 Color flow Doppler
J Ultrasound Med 11:499- 501, 1992
Figure 2 Color flow Doppler imaging demonstrated flow at the base of the lesion (arrow), with an avascular center.
Figure 3 Gross specimen demonstrates a solid-appearing mass containing several prominent vascular spaces.
imaging has not yet been described in the evaluation of hemangioma prenatally. Its use, however, has been described with orbital hemangiomas, ocular capillary hemangiomas being highly vascular and cavernous hemangiomas being relatively avascular. 8•9 When an extracranial mass is identified, the diagnosis of an encephalocele must be excluded. An encephalocele may appear sonographically as a thin-
J Ultrasound Med 11:499-501, 1992
walled cyst, as a solid mass with a gyral pattern continuous with the cranium, or as a combined cystic and solid mass. 10 When no calvarial defect is identified, the differential diagnosis for a juxtacranial mass should include teratomas, hemangiomas, mesenchymal sarcomas, and cystic hygromas. 11 Teratomas may be solid or heterogeneous, with no demonstrable gyral pattern. Cystic hygroma and lymphangioma are more typically cystic and have septa. Although previous investigators speculated that the Doppler demonstration of low vascular resistance could differentiate vascular lesions from neoplasms prenatally, this was not true in our case. 4 Understanding the variable sonographic and Doppler characteristics of hemangioma allows for more accurate prenatal diagnosis and management.
REFERENCES 1. Edgerton MT: The treatment of hemangiomas. Ann Surg 183:517, 1976 2. Bell RL: US of hemangioma of the neck of a newborn. J Tenn Med Assoc 71:289, 1978 3. Pennell RG, Baltarowich OH: Prenatal sonographic di-
BULAS ET AL 501
agnosis of a fetal facial hemangioma. J Ultrasound Med 5:525, 1986 4. Smith LG, Carpenter RJ, Gonsoulin W, et al: Prenatal diagnosis of a chest wall mass with ultrasonogr aphy and Dopplervelocimetry. AmJ Obstet Gynecol 163:567, 1990 5. Grundy H, Glasmann A, Burlbaw J, et al: Hemangiom a presenting as a cystic mass in the fetal neck. J Ultrasound Med 4:127, 1985 6. Lasser D, Preis 0, Dor N, et al: Antenatal diagnosis of giant cystic cavernous hemangioma by Doppler velocimetry. Obstet Gynecol 72:476, 1988 7. McGahan JP, Schneider JM: Fetal neck hemangioe ndo¥ thelioma with secondary hydrops fetalis: Sonographic diagnosis. J Clin Ultrasound 14:384, 1986 8. Folley WO: Color Doppler Flow Imaging. Boston, An· dover Medical Publishers, 1991, 57 9. Sklar EL, Quencer RM, Byrne SF, et al: Correlative study of the computed tomographic, ultrasonographic, and pathological characteristics of cavernous versus capillary hemangiomas of the orbit. J Clin Neuro-Oph thalmol 6:14, 1986 10. Nyberg DA, Mahony BS, Pretorius DH: Diagnostic Ultrasound of Fetal Anomalies: Text and Atlas. Chicago, Year Book, 1990, p 153 11. Sabbagha RE, Tamura RK, Dal Compo S, et al: Fetal cranial and craniocervical masses: Ultrasound characteristics and differential diagnosis. Am J Obstet Gynecol 138:511, 1980
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