Revisit on congenital bronchopulmonary vascular malformations: a haphazard branching theory of malinosculations and its clinical classification and implication.

We propose a haphazard branching theory to support the concept of bronchopulmonary malinosculations, and we apply this theory to classify congenital bronchopulmonary vascular malformation (BPVM) based on the anatomical results we have found. Between January 1990 and December 1997, a total of 22 pediatric patients (10 male and 12 female), aged 2 days to 14 years (median, 19.6 months), with congenital BPVM were enrolled in this retrospective study. Study modalities include the clinical features and plain chest films (n = 22) plus at least two of the following: echocardiography (n = 13), barium esophagraphy (n = 2), bronchoscopy (n = 4), contrast bronchography (n = 8), high-resolution direct coronal CT (n = 1) and electron beam or ultrafast CT (n = 1) of the chest, MRI (n = 10), MRA (n = 1), contrast cineangiocardiography (n = 9), surgery (n = 11), or autopsy (n = 2). The salient clinical features were recurrent lung infections in 14 patients, acute respiratory distress in 13, associated cardiovascular malformations in 8, dextroversion in 7, congestive heart failure in 7, dextrocardia in 4, and complex congenital heart diseases in 4. There were abnormal openings (malinosculations) of the pulmonary airway in 20 patients: to an artery in 12, to a vein in 8, and to the lung parenchyma in 9. These 22 patients with congenital BPVM can be classified into bronchial malinosculation (10 cases), arterial malinosculation (2 cases), and bronchoarterial malinosculation (10 cases). Congenital BPVM can be classified in terms of bronchopulmonary malinosculation based upon a haphazard branching theory, in which abnormal communications between two independent systems (primitive foregut system and aortic-pulmonary arch system) occurred coincidentally rather than causally.     

Haemoglobin A1 and congenital malformation

Two hundred and thirty pregnancies were studied in 196 diabetic women. Seven women with babies found to have major malformations had a higher median first trimester haemoglobin A1 (12.9%) than the median HbA1 (10.8%) in those with normal babies (p = 0.06). No relationship was found between the occurrence of minor malformations and first trimester maternal haemoglobin A1. Two of the seven congenital malformations were diagnosed antenatally at a time when therapeutic abortion could be offered. Expert antenatal ultrasound scanning should be offered to all pregnant diabetic women as poor glycaemic control at the time of conception and organogenesis, as evidenced by raised first trimester HbA1, predisposes to congenital malformation.     

Arnold-Chiari畸形合并脊髓空洞症的外科治疗(附32例报告)

目的：探讨Chiari畸形合并脊髓空洞症的手术治疗方法。方法：分析我科收治的32例Chiari畸形合并脊髓空洞症患者进行手术治疗，以后颅窝减压、小脑扁桃体切除、脊髓空洞穿刺、松解四脑室正中孔蛛网膜粘连为主，达到解除后脑受压，解除粘连，恢复枕大孔区CSF循环通畅的目的。结果：随访24例患者中，临床症状完全消失18例，好转2例，术前有头痛、颈痈或肩臂病症状者均有显著减轻，3例自觉症状无变化，复查MRI显示21例空洞明显缩小。结论：后颅窝减压、松解四脑室正中孔蛛网膜粘连，恢复枕大孔区CSF循环通畅是手术治疗Chiari畸形合并脊髓空洞症的关键。     

350例脊髓血管畸形介入治疗的护理分析

目的探讨护理工作在脊髓血管畸形介入治疗中的价值。方法通过对我院介入中心350例脊髓血管畸形患者介入治疗,针对脊髓血管畸形患者的临床特点和介入治疗方法的适应证、操作过程和预后等特点,从护理学的角度对术前、术中、术后等阶段进行讨论分析。结果术前进行有效的心理护理和健康教育;术中与介入医生及技术操作人员进行有机的配合、熟练使用输液微量泵、准确使用相关药物及认真观察病情变化;术后对患者进行严密的护理观察,可减少并发症的发生,将有助于该手术的成功。结论介入护理工作对脊髓血管畸形介入治疗手术的成败起着至关重要的作用。     

Intra-articular arteriovenous malformation of the knee

Two young men with longstanding suprapatellar masses are presented. The initial clinical diagnosis was of a synovial or soft tissue tumour. Magnetic resonance imaging showed the features of an arteriovenous malformation (AVM). Surgical exploration and biopsy confirmed the diagnosis. AVMs, although rare, should be considered as a possible diagnosis of a peri-articular swelling of the knee.     

Hematospermia associated with congenital arteriovenous malformation of internal iliac vessels

The presence of blood in the ejaculate is called hematospermia or hemospermia. While often perceived as a symptom of little significance, hematospermia can cause great concern to men who experience it. We report an unusual case of hematospermia associated with pelvic arteriovenous malformation (AVM). A 60-year-old man who visited our hospital complaining of hematospermia and pollakisuria was found to have AVM and aneurysmal changes in the left side of the pelvis using computed tomography (CT). The patient was treated with steel coil embolization of the left inferior gluteal artery, and after the procedure the hematospermia and pollakisuria remained absent without flare-ups.     

Bilateral Ulnar Agenesis: Case Report and Review of the Literature

Bilateral ulnar agenesis is a rare abnormality. A total of 36 cases are analyzed: 35 of these are documented in the literature and 1 stillborn male is presented in this study. Most patients had one of the three conditions: Al-Awadi/Raas-Rothschild syndrome, syndrome of ulnar aplasia with split hand/split foot deformity, or the Brachmann—de Lange syndrome. Fifty percent of all cases with bilateral ulnar agenesis were associated with lower limb defects and these cases, for the most part, also belonged to the aforementioned syndromes. Nonskeletal, internal organ malformations were identified in 34% of all patients. Nine patients presented with isolated bilateral ulnar agenesis. The Al-Awadi/Raas-Rothschild syndrome and the split hand/split foot deformity are heritable disorders. There was no evidence for genetic etiology in most of the other cases. Bilateral ulnar agenesis in our fetus was part of the Brachmann—de Lange syndrome with associated cardiac defect, diaphragmatic hernia, and umbilical artery agenesis.     

Basilar arterio-venous pseudoparallelism due to persistence of embryonal venous pattern

Summary A vascular malformation, consisting of a venous vessel bridgeing the right inferior petrosal sinus and the anterior spinal veins, was found in the posterior fossa. The vessel presented a ring-like course around the right trigeminal root, and it was parallel and dorsal to the basilar artery. The malformation was associated with cutaneous and hepatic angiomas and peri-osteal lipomas. It had been clinically silent for 52 years, when it thrombosed causing death. The authors think that, within a general mesenchymopatic state, this is a result of the persistence of an embryonal cerebral venous pattern.     

新生儿先天性畸形与孕妇乙型肝炎病毒感染关系的研究

为探讨孕妇乙型肝炎病毒(HBV)感染和某些因素对先天性畸形的致病作用,本文进行了96对新生儿先天畸形的配对(1:1)病例对照研究,报道先天畸形的频率和构成,单因素和多因素的条件Logistic回归分析,表明孕妇HBsAg阳性等6个因素对先天畸形呈阴性结果,而与接触农药和孕周的因素有明显联系,值得进一步探讨。     

低位无肛术术中完整保留瘘口组织的必要性和可行性

目的探讨低位肛门直肠畸形术术中保留瘘口组织的必要性和可行性。方法对67例无肛前庭及会阴瘘患儿的临床资料进行回顾性分析。其中女童无肛前庭瘘59例,男童无肛会阴瘘8例。年龄3个月~16岁,平均10个月。患儿多以排便困难或肛门位置异常就诊。4例无肛前庭瘘患儿曾在婴儿期行瘘口后切术。患儿均采用完整保留瘘口的前矢状入路肛门成形术治疗。结果患儿术后3及6个月常规来院复诊,最长随访8年。65例(97.0%)患儿会阴部矢状切口一期愈合,会阴及肛门外观良好;另2例(男、女各1例)术中直肠破损患儿,自修补处穿孔导致会阴部矢状切口感染(占直肠破损修补术的40%),最终形成直肠会阴瘘,但肛门外观良好。67例均采用肛门功能临床评分标准评估患儿排便功能,64例(95.5%)患儿排便功能良好,总评分为5~6分;另3例(男1例,女2例)顽固便秘,需开塞露协助排便。结论低位无肛术中完整保留瘘口组织非常必要。