Demie Senayit, Bossak Michael
Department of Pediatrics, Willett Children's Hospital of Savannah, Memorial Health University Medical Center, Savannah, GA.
HCA Healthc J Med. 2021 Aug 29;2(4):267-272. doi: 10.36518/2689-0216.1250. eCollection 2021.
Hemangiomas are benign vascular tumors that are common during infancy. They are most commonly noted as superficial bright red lesions on the skin but can also be found deeper as subcutaneous lesions. Patients with multifocal cutaneous hemangiomas are at risk of visceral involvement with the liver being most commonly affected. Most hemangiomas can be monitored clinically as they are self-limiting. Despite this, hepatic hemangiomas can have serious complications including large arteriovenous shunts leading to cardiac compromise as well as severe hepatomegaly which can cause abdominal compartment syndrome, impaired ventilation and renal vein compression.
A six-month-old female, born full term and previously healthy, presented due to worsening abdominal distention and hepatomegaly. On examination, abdominal distention, hepatomegaly and three superficial hemangiomas on her torso and scalp were appreciated.
The patient had an extensive workup which showed an elevated AFP and TSH. An abdominal ultrasound revealed numerous rounded regions of hypoechogenicity throughout the hepatic parenchyma. These findings were consistent with diffuse infantile hemangiomas; however, metastasis could not be ruled out with ultrasound alone. An MRI of the abdomen was obtained which confirmed infantile hemangiomas. The patient's MRI and lab findings are the classical findings of infantile hepatic hemangiomas. She had elevated blood pressures during the hospital course and was subsequently evaluated by cardiology for concern of cardiac compromise. Treatment with propranolol was initiated and continued upon discharge. A six month follow-up ultrasound showed significant decrease in size of the hemangiomas.
Hepatic hemangiomas should be monitored closely for serious complications. Although rare, it is important to identify which patients with multifocal cutaneous hemangiomas should be worked up for hepatic hemangiomas and their complications. It is recommended that infants younger than 6 months of age with 5 or more cutaneous hemangiomas undergo early evaluation with abdominal ultrasound.
血管瘤是婴儿期常见的良性血管肿瘤。它们最常表现为皮肤表面的鲜红色病变，但也可更深地作为皮下病变被发现。患有多灶性皮肤血管瘤的患者有内脏受累的风险，其中肝脏最常受到影响。大多数血管瘤可通过临床监测，因为它们是自限性的。尽管如此，肝血管瘤可出现严重并发症，包括导致心脏功能不全的大型动静脉分流以及可引起腹腔间隔室综合征、通气障碍和肾静脉受压的严重肝肿大。
一名足月出生且既往健康的6个月大女性因腹胀和肝肿大加重就诊。检查时，发现腹部膨隆、肝肿大以及躯干和头皮上有3个浅表血管瘤。
患者接受了全面检查，结果显示甲胎蛋白（AFP）和促甲状腺激素（TSH）升高。腹部超声显示肝实质内有许多圆形低回声区。这些发现符合弥漫性婴儿血管瘤；然而，仅靠超声不能排除转移。进行了腹部磁共振成像（MRI），证实为婴儿血管瘤。患者的MRI和实验室检查结果是婴儿肝血管瘤的典型表现。她在住院期间血压升高，随后因担心心脏功能不全由心脏病学专家进行评估。开始使用普萘洛尔治疗并出院后继续用药。6个月后的随访超声显示血管瘤大小显著减小。
应密切监测肝血管瘤的严重并发症。虽然罕见，但确定哪些患有多灶性皮肤血管瘤的患者应检查是否患有肝血管瘤及其并发症很重要。建议6个月以下有5个或更多皮肤血管瘤的婴儿早期进行腹部超声检查。
