Pulmonary artery banding revisited.

Pulmonary artery (PA) banding to reduce pulmonary blood flow was described by Muller and Dammann in 1952. This review describes the outcome of 170 children who had PA banding at the University of Virginia Medical Center between 1955 and 1988. One hundred and one of the patients were banded between 1958 and 1970; fewer bands were placed in later years because early total correction was feasible for certain conditions. When analyzed by preoperative diagnoses, the data reveal that children with a single ventricle undergoing banding had a significantly lower 30-day mortality rate of 12% compared to other preoperative diagnoses, including atrioventricular canal, truncus arteriosus, and ventricular septal defect (VSD) at 30% (p less than 0.05). The late overall mortality for all patients was approximately 10%, an attrition rate of 1% per year. PA banding still has a role in management of patients with congenital heart disease, particularly for infants with a single ventricle. Actuarial survival at 10 years for patients with this condition is 92%. Interestingly, this indication for pulmonary banding is the same one cited in the original report.     

Renal peripelvic multicystic lymphangiectasia

Renal peripelvic multicystic lymphangiectasia is an unusual benign peripelvic fluid collection. The sonographic appearance, etiology, and differential diagnosis of peripelvic fluid collections are reviewed.     

肾淋巴管扩张症临床分析

目的 总结肾淋巴管扩张症的临床特点及治疗方法。 方法 分析2008年12月、2010年6月收治的2例肾淋巴管扩张症患者临床资料。例1,女,37岁。右腰腹部痛8d。B超检查示双肾周混合性回声,与肾脏分界欠清。CT检查示双肾被膜下低密度影,内混有散在点状高密度影。保守治疗3周后疼痛缓解。3个月后右侧腰痛加重,CT检查示右肾周被膜下大量积液,B超引导下穿刺置管引流后积液消失;2个月后复查B超示左侧肾周少量积液,穿刺抽液后行积液常规及瘤细胞检查。例2,女,32岁。左腰部酸痛不适3年。B超检查示左肾周围不规则囊性病变,CT检查示左肾后外侧分叶状囊性病变,与肾实质分界不清,左肾受压前移。诊断为左肾淋巴管瘤,硬膜外麻醉下行淋巴管瘤切除术。 结果 例1囊液涂片见大量淋巴细胞和少量中性粒细胞,考虑为淋巴液,临床诊断为肾淋巴管扩张症,随访2个月未见复发。例2术后病理检查囊壁衬以扁平上皮细胞伴淋巴细胞浸润,淋巴管呈囊状扩张,诊断为肾囊性淋巴管瘤。术后随访9个月未见复发。 结论 B超、CT检查有助于肾淋巴管扩张症的诊断,穿刺细胞学和组织病理学检查可确诊。无症状者可密切随访,有症状者可穿刺引流,但复发率较高;也可手术切除扩张淋巴管+无水乙醇破坏内皮细胞治疗,复发率低,但可能发生淋巴漏。     

Recurrent hemolytic uremic syndrome associated with intestinal lymphangiectasia

A 17-year-old boy was admitted to the hospital twice in a year for 2 episodes of hemolytic uremic syndrome (HUS). During these 2 HUS episodes he had diarrhea, decreased serum complement, decreased total protein and decreased serum albumin concentrations. We suggest that protein-losing enteropathy and hypocomplementemia due to intestinal lymphangiectasia is may be a rare cause of atypical HUS.     

Intestinal lymphangiectasia and colonic polyps: surgical intervention

A 36-mo-old boy with Milroy's Disease, intestinal lymphangiectasia, and an exudative enteropathy (EE), was shown to have four colonic polyps. A large adenomatous polyp was excised from the transverse colon in an effort to control his EE and hypoalbuminemia (1.95 g/dl). His clinical status then stabilized until age 50 mo when there was a marked exacerbation of his EE. Medical management resulted in a temporary stabilization of his condition. A partial resection (40 cm) of the visually worse affected jejunum was performed. There was no improvement in the EE as measured by 51Cr-tagged albumin study; however, his clinical response was dramatic. In the 10 mo since surgery, he has been well and has shown catchup in linear growth.     

Generalised lymphangiectasia: pulmonary presentation in an adult.

A 25 year old man presented with dyspnoea and was found to have generalised, but predominantly pulmonary, lymphangiectasis without gastrointestinal symptoms. This is an unusual presentation of a disorder previously diagnosed only in childhood.     

Cystic renal lymphangiectasia presenting as renal insufficiency in childhood

Cystic renal lymphangiectasia is an unusual cause of cystic renal disease in childhood. We present a case of bilateral cystic renal lymphangiectasia in a 7-year-old boy who presented with asymptomatic renal insufficiency and anemia with decreased erythropoietin production. The clinical features of this condition and the diagnostic approach are reviewed. Although rare, this disorder should be considered in the differential diagnosis of cystic renal disease. Received: 2 September 1999 / Revised: 26 April 2000 / Accepted: 27 April 2000     

End-stage renal disease in a patient with congenital lymphangiectasia and lymphedema

Congenital lymphangiectasia with lymphedema is a disorder constituting the main defect in many different genetic syndromes. Herein we describe a 23-year-old male patient with congenital lymphangiectasia and severe lymphedema of the right leg, scrotum, and abdominal wall, who presented with end-stage renal disease, presumably due to cystic renal lymphangiectasia, and is undergoing chronic hemodialysis treatment. Received: 19 April 2000 / Revised: 4 October 2000 / Accepted: 5 October 2000