儿童脾海绵状血管瘤23例报告

目的　了解脾海绵状血管瘤患儿的临床特点及诊治要点。方法　总结了 1997～ 2 0 0 3年深圳市儿童医院和 1980～ 2 0 0 3年中南大学湘雅二院收治的 2 3例脾海绵状血管瘤患儿的临床表现、实验室检查、影像学检查、病理学检查的特点及其治疗经过和预后情况。结果　脾海绵状血管瘤患儿的主要临床表现为腹部包块、腹痛、腹部不适 ,B超多表现为增强回声 ,彩色多普勒多显示其内部有静脉血流信号 ,CT平扫多表现为均质的低密度灶 ,增强扫描多表现为均质性显著强化。脾海绵状血管瘤患儿进行脾切除术效果良好。结论　脾海绵状血管瘤的诊断主要依靠影像学检查 ,治疗可根据临床表现的有无及时或择期行脾切除术等干预措施。     

Sclerosing therapy with bleomycin emulsion for lymphangioma in children

Forty-seven children with lymphangioma were treated by sclerosing therapy with bleomycin (BLM) emulsion between may 1976 and March 1988. Injection of 0.3 to 0.6 mg/kg body weight BLM emulsion into the cavity was repeated at intervals of 4 to 6 weeks. The lymphangioma regressed in 41 (87%) and almost disappeared in 20 (43%). The response was greater when the lymphangioma was cystic rather than cavernous. In the cases that responded, the total injected dose was 0.6 to 4 mg/kg. This therapy proved to be dangerous for large cervicomediastinal lymphangiomas in young infants because the cystic mass enlarged transiently after local injection and airway compression resulted. Pulmonary fibrosis was not observed. There was no recurrence of the residual induration after BLM emulsion therapy. Antenatal ultrasonography and magnetic resonance imaging have been shown to be useful diagnostic aids. Offprint requests to: K. Tanaka     

Imaging characteristics of two subtypes of congenital hemangiomas: rapidly involuting congenital hemangiomas and non-involuting congenital hemangiomas

Background: Common infantile hemangiomas (COMMON) occur in approximately 10% of infants by the age of 1 year, with a female predominance. Some hemangiomas can be fully developed at birth and are thus called congenital hemangiomas (CH). Within this population, two courses have been identified: rapidly involuting CH (RICH) and non-involuting CH (NICH). Little has been reported on the clinical prognosis and imaging features of these entities. Objective: To describe the imaging characteristics of two subtypes of CH, i.e. RICH and NICH, and to compare them with COMMON. Materials and methods: We retrospectively gathered data on 26 children presenting with CH, i.e. lesions fully developed at birth. These lesions were divided into two groups according to the clinical course: suspected RICH (n=8) and suspected NICH (n=18). We used US, CT or MRI and angiography to identify the gross anatomy and structure and the vascularization. Imaging findings were compared with the clinical course and pathology results, when available. The imaging findings in these patients were compared retrospectively with those in 26 patients with COMMON randomly chosen from the database of our multidisciplinary clinic. Results: When compared with COMMON imaging characteristics, NICH and RICH had distinctive features on US such as being heterogeneous (72% of NICH and 62.5% of RICH vs 42.3% of COMMON), visible vessels (72% of NICH and 62.5% of RICH vs 15.4% of COMMON), calcifications (17% of NICH and 37.5% of RICH vs no case of COMMON). On CT and/or MRI, we compared imaging features such as well-defined limits (67% of NICH and 60% of RICH vs 100% of COMMON), and fat stranding (29.4% of NICH and RICH vs 7.7% of COMMON). Conclusion: Distinctive imaging characteristics are observed in cases of CH with US findings of visible vessels and calcifications statistically significant.     

Intralesional bleomycin injection (IBI) treatment for haemangiomas and congenital vascular malformations

Successful treatment of vascular anomalies has eluded the physician until now, despite various treatments utilised. Bleomycin has been successfully used in intralesional injection treatment of cystic hygromas and haemangiomas, based specifically on a high sclerosing effect on vascular endothelium. In a prospective study of 95 patients, the effectiveness of intralesional bleomycin injection (IBI) treatment in haemangiomas and vascular malformations was evaluated and documented. Complete resolution or significant improvement occurred in 80% of all patients treated. Complete resolution occurred in 49% of haemangiomas, 32% of venous malformations, and 80% of cystic hygromas. Significant improvement occurred in 38% of haemangiomas, 52% of venous malformations, 13% of cystic hygromas and 50% of lymphatic malformations. Of the six patients who presented with a painful lesion, four experienced complete resolution and two had significant improvement to treatment. Local complications encountered were superficial ulceration occurring in 2 patients, and cellulitis in 1 of the 95 patients. Systemic complications were flu-like symptoms in three patients and partial, transient hair loss in two patients. None of the patients presented with haematological toxic effects or signs of pulmonary involvement (fibrosis, hypertension). IBI is an effective treatment in haemangiomas and vascular malformation lesions, obviating the need for invasive primary surgery or systemic treatment regimens in 80% of cases, and allowing for limited need of secondary surgical or adjunctive procedures in cases with a moderate result.     

普萘洛尔片剂口服治疗婴幼儿血管瘤68例临床分析

目的探讨口服普萘洛尔片剂方法治疗婴幼儿血管瘤的临床疗效及安全性。方法2011年11月-2015年6月在门诊就诊的68例血管瘤患儿,接受普萘洛尔片剂治疗(每日总量1.5~2 mg/kg,分3~4次口服,一周加至足量);观察记录血管瘤大小、颜色的变化以及相关副作用,以评价其疗效及安全性。结果服药3个月、6个月、1年治疗有效率分别为88.2%、98.1%、98.1%;疗效达V级及以上者分别为33.8%、80.8%、100%。多数患儿在治疗12个月时已经停药,继续用药的患儿,其疗效分级均为Ⅵ级。疗效与性别、开始服药月龄、病灶部位均无关(P0.05)。本组3例患儿出现一过性普洛萘尔相关不良反应,停药后恢复。结论口服普萘洛尔方法治疗婴幼儿血管瘤疗效显著、副作用少。     

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血管瘤和血管畸形是婴幼儿常见疾病。传统分类方法依据形态学分类将血管瘤和血管畸形统称为血管瘤,并分为鲜红斑痣、草莓状血管瘤、海绵状血管瘤及混合性血管瘤。20世纪80年代以来,Mulliken等提出了生物学分类方法,将传统意义血管瘤划分为血管瘤与脉管畸形两大类。根据国际血管性疾病研究协会(ISSVA)对血管瘤的最新分类,血管瘤又分为婴儿血管瘤、先天性血管瘤、Kaposi样血管内皮瘤、丛状血管瘤、化脓性肉芽肿、梭形细胞血管瘤、血管外皮细胞瘤、上皮样血管瘤,其中先天性血管瘤又分为快速消退先天性血管瘤和无消退     

Efficacy and safety of propranolol as first-line treatment for infantile hemangiomas

Beta-blockers are a highly promising treatment modality for complicated infantile hemangiomas (IH). However, data on propranolol as first-line treatment, objective outcome measures and impact on hemodynamics in young infants is limited. We retrospectively evaluated a homogenous group of infants with proliferating complicated IH treated with propranolol (2 mg/kg/day). Outcome was assessed by blinded evaluation of clinical photographs by visual analogue scale (VAS), ultrasound examination and ophthalmological review (if appropriate). Tolerance and hemodynamic variables were recorded over time, including a 2-day in-patient observation at the initiation of therapy. Twenty-five infants (median age 3.6 (1.5–9.1) months) were included in the study. The median follow-up-time was 14 (9–20) months and 14 patients completed treatment at a median age of 14.3 (11.4–22.1) months, after a duration of 10.5 (7.5–16) months. In all patients, there was significant fading of colour (with a VAS of −9 (−6 to −9) after 7 months) and significant decrease in size of the IH (with a VAS of −8 (−3 to −10) after 7 months). Median thickness of the lesions assessed by ultrasound at baseline and after 1 month was 14 (7–28) mm and 10 (5–23) mm, respectively (p < 0.01). In children with periocular involvement, astigmatism and amblyopia resolved rapidly within 8 weeks. The overall tolerance of propranolol was good, and no relevant hemodynamic changes were noted. Conclusion: Our report supports the excellent effect and good tolerance of this novel therapy, and we propose the use of propranolol as first-line treatment for IH.     

CCNU and bleomycin in the treatment of cancer: A southwest oncology group study

CCNU and Bleomycin, alone or in combination, were used to treat 397 evaluable cases of advanced cancer. Responses defined as 50% or greater reduction in all tumor masses occurred in 13/35 lymphomas, 8/50 brain cancers, and 28/312 other solid tumors. Hematologic toxicity of a life-threatening degree occurred in 33/298 patients evaluable for CCNU toxicity; in an additional 50 it was considered severe. Pulmonary toxicity was severe in 4 patients. Drug-associated deaths (4) included one each due to leukopenia and thrombocytopenia after CCNU; one due to pulmonary toxicity after Bleomycin; and one due to shock after the 20th dose of Bleomycin. There was no suggestion of synergism or additive activity with the two drugs.     

Clinically significant ascites as an indication for resection of rapidly involuting congenital hepatic hemangiomas

Hepatic hemangiomas are the most common benign liver tumor of infancy and are divided into two main types: rapidly involuting congenital hemangiomas (RICH) and non‐involuting congenital hemangiomas. RICH typically involute by 12 months and are often asymptomatic. Surgical resection is rare. Indications for surgical resection include rupture, rapid growth, consumptive coagulopathy, and abdominal pain. We present two patients from different institutions who both developed clinically significant ascites as the RICH involuted, prompting surgical resection. This is a new indication for resection.     

儿童颞叶症状性癫痫的手术治疗

目的：探讨儿童颞叶症状性癫痫的原因和临床特征并总结手术治疗经验。方法：对我院自1994年1月至2002年8月手术治疗的17例儿童颞叶症状性癫痫，男11例，女6例，年龄3～14岁，平均9岁，均以癫痫为首发症状，病程13d到7年，平均为4．3年。手术方式为：单纯病灶切除6例，病灶加前颞叶切除11例，其中13例再行皮层脑电极检查，12例仍发现致病波，则加大病灶切除范围和／或病灶周围皮质区多处软膜下横纤维切断。结果：17例患儿无手术死亡及并发症。随访16例，时间为6个月～7年，术后症状完全消失者12例(占70．6％)，术后癫痫发作显著减少者(发作频率不到原来的25％)3例(17．6％)，术后癫痫发作减少者(发作频率不到原来的50％)2例(11．8％)。结论：在皮层脑电极监测下，多种手术方法结合治疗颞叶症状性癫痫患儿安全有效。     

Necrotizing fasciitis in a newborn with an enormous cavernous hemangioma

Necrotizing fasciitis (NF) is a rapidly progressive, invasive softtissue infection with a high mortality that is rare in the pediatric population. Of the reported pediatric cases, 50% involved neonates; the most common cause was neonatal omphalitis. The cornerstone of therapy is early, aggressive surgical treatment with boradspectrum antibiotics and nutritional support. In this case report, a large, contaminated cavernous hemangioma in the gluteal area associated with NF is reported. The patient's condition was severely compromised due to toxicity and sequestration of blood in the hemangioma. Effective treatment relied on a multimodal approach.     

Extensive pneumothorax, pneumomediastinum and surgical emphysema as a complication of bleomycin therapy

Bleomycin is a commonly used chemotherapeutic agent and one of the commonest cytotoxic drugs leading to pulmonary parenchymal damage. It generally leads to interstitial pneumonitis and fibrosis, hypersensitivity reactions and acute respiratory distress syndrome. We describe an 8-year-old boy who, following prolonged bleomycin therapy, demonstrated extensive air dissection and extrapulmonary air, an unusual and fatal complication.     

Evaluation of plasma tissue factor and tissue factor pathway inhibitor levels in childhood hemangiomas

BACKGROUND: The purpose of the study was to evaluate the plasma levels of tissue factor (TF), an angiogenic marker, and tissue factor pathway inhibitor (TFPI), an antiproliferative protein, in the childhood hemangiomas at proliferative and regressive stages. PROCEDURE: The study included 30 patients with hemangiomas and 30 healthy children. Localization, number, stage, type, duration of growth, and complications of the hemangiomas were determined. Venous blood samples from all individuals were collected into citrated tubes and centrifuged. Supernatant plasma was separated, aliquated, and stored at -70 degrees C until samples could be assayed. Plasma levels of TF and TFPI were measured with quantitative ELISA kits. RESULTS: Plasma TF and TFPI levels did not show any significant difference between the study and control groups. When plasma TF and TFPI levels of the children in the control group and in the study group who were in proliferative and regressive stage were compared to each other, no statistically significant difference could be detected. CONCLUSIONS: Plasma TF and TFPI levels of our patients with hemangiomas were not different from healthy children and they did not show any significant difference in proliferative and regressive stages.     

Interferon-α for alarming hemangiomas in infants: Experience of a single institution

Background:  Hemangioma is the most common tumor in infancy. 'Alarming hemangiomas' refer to the lesions that potentially impair vital structures or cause life-endangering complications, and which warrant vigorous treatment. Interferon-α has been used for alarming hemangiomas at Mackay Memorial Hospital, Taipei, Taiwan, since 1994.
Methods:  The records of 21 consecutive infants treated between January 1994 and December 2005 were retrospectively reviewed. The initial dose of interferon-α was 50 000 IU/kg per day, which was increased to 100 000 IU/kg per day in the second week of therapy if tolerated. It was tapered depending on response, with total treatment lasting no longer than 12 months. Treatment response was evaluated depending on the size of the lesion and resolution of complications.
Results:  The duration of therapy ranged from 6 to 12 months. Six patients (29%) had a reduction in mass of ≥25% after 1 month of therapy. Twenty patients (95%) had achieved a decrease in size of 50% by 12 months, and 15 (71%) had total involution of lesions by a median age of 13.5 months (range 7–50 months). Only mild and transient adverse effects were encountered. No neurologic complications occurred.
Conclusions:  Interferon-α appears to be an effective and well-tolerated treatment for alarming hemangiomas in infancy.     

采用Evans跟骨延长术治疗儿童特发性痛性扁平足

目的 评价儿童痛性扁平足Evans跟骨延长术的矫正作用和效果.方法 患儿入选标准:负重及非负重情况下足内侧纵弓均降低或消失且合并疼痛症状者.本组自2006年1月至2008年7月共治疗18人23足,男11人,女7人,年龄11～14岁,平均12岁8个月,采用Evans跟骨截骨延长术进行治疗,平均随诊2年5个月,范围7个月至3年3个月,均为特发性病例,以前均进行过保守治疗且无效.术前、术后均拍照负重下前后位及侧位X线片,临床评价采用Dogan标准,X线评价包括7个参数,对术前、术后各项均值进行成组的t检验,P<0.05被认为差异有统计学意义.结果 临床评价优22足(96%),良1足(4%).术前与术后、术前与末次随访时各项X线参数的均值差异都存在统计学意义,且术后各项指标均数都在相应年龄段的正常值范围内.术后8周跟骨延长处开始愈合,术后1年塑形完成.跟骨平均延长8.0 mm(范围5～10 mm).未发生植骨块移动,无过度矫正,术后所有患儿均恢复正常跖行足负重行走.结论 对于有症状的儿童扁平足,保守治疗无效时,采用跟骨截骨延长术可以从临床和X线两个方面有效地矫正后足和前足畸形并缓解疼痛.     

血管内皮生长因子在增生性血管瘤组织和血清中表达的意义

目的　探讨血管内皮生长因子 (VEGF)在增生性血管瘤组织、邻近组织和外周血中的表达水平和分布规律 ,进一步了解血管瘤的发生机制。方法　取增生性血管瘤患儿外周血清和术中瘤组织及其邻近组织标本 ,以相同年龄非相关患儿作对照 ,用酶联免疫法测定血清VEGF水平 ;对肿瘤组织及其相邻组织行cDNA合成和PCR扩增 ,提取VEGFmRNA ,并进行定量分析。结果　血管瘤患儿血清VEGF水平明显高于对照组 (P <0 .0 5 ) ;血管瘤组织的VEGFmRNA电泳阳性率为 10 0 % (9/ 9) ,邻近组织为 88.9% (8/ 9) ,对照组为 11.1% (1/9) ,经检验血管瘤及其邻近组织与对照组有显著性差异 (P均 <0 .0 1)。荧光定量测定mRNA在血管瘤及其邻近组织无显著差异 (P <0 .0 5 ) ,而两者与对照组均有显著差异 (P均 <0 .0 1)。结论　增生性血管瘤组织及患儿外周血VEGF明显增高 ,对血管内皮细胞增生和分化成血管瘤细胞有促进作用 ,检测血清VEGF水平有助于血管瘤分型和增生情况的监测     

Diagnosis and treatment of blue rubber bleb nevus syndrome in children

Background  Blue rubber bleb nevus syndrome (BRBNS) is characterized by distinctive vascular malformations of skin and the gastrointestinal tract, often leading to chronic anemia and intestinal bleeding. It usually presents right after birth or during early infancy. Though the disease is inherent, its occurrence is sporadic. Thus it is usually not timely diagnosed. We analyzed the clinical characteristics and treatment of this disorder in order to improve the diagnosis and treatment. Methods  Three patients with BRBNS treated at our hospital during 2002–2003 and 39 patients from the literature reported during 1965–2003 were reviewed in terms of the diagnosis and treatment. BRBNS may be diagnosed as cutaneous cavernous hemangioma associated with the same lesion of the gastrointestinal tract and other organs. Results  Our 3 patients suffered from cutaneous angioma and gastrointestinal hemangioma. In 39 patients reported in the literature, cutaneous angioma was observed in all of them, and gastrointestinal hemangioma in 31. Additionally, the lesions were also found in other organs such as the brain (7 patients), joint (2), liver (2), eye (1), kidney (1) and spleen (1). Cutaneous angioma was located on the surface of the skin, including body (93%), limbs (86%), hip (36%) and face (26%). Gastrointestinal hemangioma was more common in the small intestine (100%) than in the colon (74%) and stomach (26%). When the joint was involved by hemangioma, pathologic fracture or overgrowth of bone needed traction and amputation (1 patient respectively). For significant gastrointestinal bleeding, endoscopic techniques (8 patients), surgical excision (5), or both (1) were performed. Recurrent bleeding was successfully treated by endoscopic laser combined with steroid or interferon in one patient. Conclusions  BRBNS in children presents atypical symptom and systemic complications. It should be dealt with seriously if gastrointestinal bleeding or orthopedic complication occurs. Treatment includes conservative, endoscopic and surgical options. Its recurrence with new angioma in the gastrointestinal tract needs laser-steroid therapy.