PDF(Pubmed)
Abstract：
To explore the clinical characteristics, postnatal treatment and prognosis of giant fetal hepatic hemangioma (GFHH).
Retrospective analysis was performed on children with giant fetal hepatic hemangioma (maximum tumor diameter > 40 mm) diagnosed by prenatal ultrasound and MRI from December 2016 to December 2020. These patients were observed and treated at the Children\'s Hospital of Fudan University after birth. The clinical data were collected to analyze the clinical characteristics, treatment, and prognosis of GFHH using independent sample t tests or Fisher\'s exact tests.
Twenty-nine patients who were detected by routine ultrasound in the second and third trimester of pregnancy with giant fetal hepatic hemangiomas were included. The first prenatal ultrasound diagnosis of gestational age was 34.0 ± 4.3 weeks, ranging from 22 to 39 weeks. Of the patients, 28 had focal GFHHs and 1 had multifocal GFHHs. Surgery was performed, and the diagnosis was confirmed histopathologically in two patients. There were 8 cases with echocardiography-based evidence of pulmonary hypertension, 11 cases had a cardiothoracic ratio > 0.6, and 4 cases had hepatic arteriovenous fistula (AVF). The median follow-up time was 37 months (range: 14-70 months). During the follow-up, 12 patients received medical treatment with propranolol as the first-line therapy. The treatment group had a higher ratio of cardiothoracic ratio > 0.6 (P = 0.022) and lower albumin levels (P = 0.018). Four (14.8%) lesions showed postnatal growth before involuting. Complete response was observed in 13 (13/29) patients, and partial response was observed in 16 (16/29) patients.
Fetal giant hepatic hemangioma is mainly localized, and its clinical outcome conforms to RICH (rapidly involuting) and PICH (partially involuting), but some fetal giant hepatic hemangiomas will continue to grow after birth and then gradually decrease. For uncomplicated giant fetal hepatic hemangioma, postnatal follow-up is the main concern, while those with complications require aggressive medical treatment. Propranolol may have no effect on the volume change of GFHH.
Retrospective analysis was performed on children with giant fetal hepatic hemangioma (maximum tumor diameter > 40 mm) diagnosed by prenatal ultrasound and MRI from December 2016 to December 2020. These patients were observed and treated at the Children\'s Hospital of Fudan University after birth. The clinical data were collected to analyze the clinical characteristics, treatment, and prognosis of GFHH using independent sample t tests or Fisher\'s exact tests.
Twenty-nine patients who were detected by routine ultrasound in the second and third trimester of pregnancy with giant fetal hepatic hemangiomas were included. The first prenatal ultrasound diagnosis of gestational age was 34.0 ± 4.3 weeks, ranging from 22 to 39 weeks. Of the patients, 28 had focal GFHHs and 1 had multifocal GFHHs. Surgery was performed, and the diagnosis was confirmed histopathologically in two patients. There were 8 cases with echocardiography-based evidence of pulmonary hypertension, 11 cases had a cardiothoracic ratio > 0.6, and 4 cases had hepatic arteriovenous fistula (AVF). The median follow-up time was 37 months (range: 14-70 months). During the follow-up, 12 patients received medical treatment with propranolol as the first-line therapy. The treatment group had a higher ratio of cardiothoracic ratio > 0.6 (P = 0.022) and lower albumin levels (P = 0.018). Four (14.8%) lesions showed postnatal growth before involuting. Complete response was observed in 13 (13/29) patients, and partial response was observed in 16 (16/29) patients.
Fetal giant hepatic hemangioma is mainly localized, and its clinical outcome conforms to RICH (rapidly involuting) and PICH (partially involuting), but some fetal giant hepatic hemangiomas will continue to grow after birth and then gradually decrease. For uncomplicated giant fetal hepatic hemangioma, postnatal follow-up is the main concern, while those with complications require aggressive medical treatment. Propranolol may have no effect on the volume change of GFHH.
摘要：
目的：探讨临床特点，巨大胎儿肝血管瘤（GFHH）的产后治疗和预后。
方法：回顾性分析2016年12月至2020年12月通过产前超声和MRI诊断的巨大胎儿肝血管瘤（最大肿瘤直径>40mm）儿童。这些患者出生后在复旦大学附属儿童医院观察和治疗。收集临床资料,分析其临床特点,治疗,使用独立样本t检验或Fisher精确检验对GFHH进行预测。
结果：29例妊娠中期和中期通过常规超声检查发现巨大胎儿肝血管瘤的患者。首次产前超声诊断胎龄为34.0±4.3周,从22到39周不等。在患者中，28人患有局灶性GFHHs，1人患有多灶性GFHHs。进行了手术，两名患者的组织病理学证实了诊断。有8例以超声心动图为基础的肺动脉高压的证据，11例患者心胸比>0.6，4例肝动静脉瘘（AVF）。中位随访时间为37个月（范围：14-70个月）。在后续行动中,12例患者接受了普萘洛尔作为一线治疗。治疗组的心胸比率>0.6的比率较高（P=0.022），白蛋白水平较低（P=0.018）。四个（14.8％）病变在消退前显示出产后生长。在13（13/29）患者中观察到完全缓解，16例（16/29）患者出现部分缓解。
结论：胎儿巨大肝血管瘤主要是局限性的，其临床结果符合RICH（快速消退）和PICH（部分消退），但是一些胎儿巨大肝血管瘤在出生后会继续生长，然后逐渐减少。对于无并发症的巨大胎儿肝血管瘤，产后随访是主要问题，而那些有并发症的人需要积极的治疗。普萘洛尔可能对GFHH的体积变化没有影响。
方法：回顾性分析2016年12月至2020年12月通过产前超声和MRI诊断的巨大胎儿肝血管瘤（最大肿瘤直径>40mm）儿童。这些患者出生后在复旦大学附属儿童医院观察和治疗。收集临床资料,分析其临床特点,治疗,使用独立样本t检验或Fisher精确检验对GFHH进行预测。
结果：29例妊娠中期和中期通过常规超声检查发现巨大胎儿肝血管瘤的患者。首次产前超声诊断胎龄为34.0±4.3周,从22到39周不等。在患者中，28人患有局灶性GFHHs，1人患有多灶性GFHHs。进行了手术，两名患者的组织病理学证实了诊断。有8例以超声心动图为基础的肺动脉高压的证据，11例患者心胸比>0.6，4例肝动静脉瘘（AVF）。中位随访时间为37个月（范围：14-70个月）。在后续行动中,12例患者接受了普萘洛尔作为一线治疗。治疗组的心胸比率>0.6的比率较高（P=0.022），白蛋白水平较低（P=0.018）。四个（14.8％）病变在消退前显示出产后生长。在13（13/29）患者中观察到完全缓解，16例（16/29）患者出现部分缓解。
结论：胎儿巨大肝血管瘤主要是局限性的，其临床结果符合RICH（快速消退）和PICH（部分消退），但是一些胎儿巨大肝血管瘤在出生后会继续生长，然后逐渐减少。对于无并发症的巨大胎儿肝血管瘤，产后随访是主要问题，而那些有并发症的人需要积极的治疗。普萘洛尔可能对GFHH的体积变化没有影响。
