Biomechanical parameters for planning Chiari's pelvic osteotomy

A Chiari osteotomy was performed in a macerated female pelvis specimen and the medialization of the distal pelvic segment bearing the hip joint was simulated within a range from 0-2.5 cm. In relation to the medialization different biomechanical hip-parameters were measured as well from the specimen as from the x-ray. For practical purpose the Wiberg CE angle towards the edge of the original acetabulum and the CE' angle towards the edge of the acetabular shelf are important parameters for preoperative calculation of the required amount of medialization.     

Dupuytren's disease in Marfan's syndrome

We report a case of histologically confirmed Dupuytren's disease of the hand in a young man with Marfan's syndrome.     

盆底松弛综合征的病理生理与外科治疗的研究

目的研究盆底松弛综合征（ＲＰＦＳ）的病理生理特征，并探讨盆腔紧固术对本征的治疗价值。方法对１１例诊断为ＲＰＦＳ患者的临床症状进行了分析，并行标准化盆腔紧固术，术后平均随访１９个月。结果患者的主要症状与体征为排便困难、排便不全、会阴坠胀、排便时会阴下凸、子宫后倾及直肠松弛。肛肠动力学检测有静息压、收缩压下降，收缩时间缩短，直肠感觉功能减退。排粪造影示盆腔多种松弛性病变同时存在。术中可见结肠、直肠、子宫的固定松弛及腹腔位置降低。术后７例症状完全消失；４例明显改善。结论ＲＰＦＳ的病理生理特征是以盆腔脏器为主的多部位、多系统、多脏器松弛性改变。盆腔紧固手术对本征的治疗具有一定价值     

Pandemic planning and its relevance to surgeons

This article will briefly discuss pandemic planning and its relevance to surgeons. It will cover principally the UK response to the severe acute respiratory syndrome coronavirus 2 (SARS-Cov-2), although it will also compare and contrast other diseases and reference more general principles of major incident planning. Areas that individual surgeons and departments can, and should, influence are discussed.     

Acute aortic dissection in a patient with Marfan's syndrome and severe pectus excavatum--emergency surgical repairs of ascending aortic dissection and aortic regurgitation complicated by pectus excavatum in Marfan's syndrome]

A case of acute aortic dissection Stanford Type A occurring in a patient with Marfan's syndrome, complicated with aortic regurgitation and severe pectus excavatum, is reported with successful surgical correction. The patient was a 33-year-old woman, who suffered from severe back pain riding to work. An emergency operation consisting of Cabrol's procedure for aortic dissection Stanford Type A and sternal turnover for pectus excavatum was performed simultaneously. Because of the postoperative mediastinal bleeding the implanted sterno-costal complex was removed on the second POD and the prosthetic sternum was reimplanted on the tenth POD. The two-stage operation seems preferable in such an emergency case to avoid postoperative bleeding and infection.     

Iliac artery aneurysm in Marfan's syndrome

A case of iliac artery aneurysm in a young male patient with Marfan's Syndrome is described. The aneurysm was managed by a simple exclusion technique. The occurrence of such an aneurysm suggests that the inherent mural weakness in the syndrome is more widespread in the arterial tree than is generally appreciated. The association of iliac artery aneurysm and Marfan's Syndrome does not seem to have been previously reported.     

Biliary atresia-polysplenia syndrome: surgical and clinical relevance in liver transplantation.

OBJECTIVE: To review a single center's 10-year experience with liver transplantation (LTx) for the biliary atresia-polysplenia syndrome (BA-PS) and to define surgical and clinical guidelines for its management. SUMMARY BACKGROUND DATA: BA is the most common indication for pediatric liver transplantation (LTx) and is associated with PS in 12% of cases. Only a few studies of LTx for BA-PS have been reported, and the optimal management of BA-PS patients undergoing LTx has yet to be determined. METHODS: From July 1985 to September 1995, 166 liver transplants were performed in 130 patients with BA and were included in the study. The malformations most commonly associated with BA-PS, surgical techniques used to overcome these anomalies, and surgical pitfalls that could have contributed to the outcome were characterized. Actuarial 10-year patient and graft survival for patients undergoing LTx for BA-PS were calculated and compared to those with isolated BA. RESULTS: Ten patients (7.8%) with BA had associated PS. An additional patient with PS without BA was included in the study. The diagnosis of PS was unknown before the transplantation in 72% of cases. Thirteen liver transplants were performed in these 11 patients. Modifications of the usual surgical technique were used to overcome the complex anatomy encountered. There was no association between the type of anomaly and the outcome, nor were there any significant differences in patient survival (72% vs. 73.5%, p = 0.79) or graft survival (56.4% vs. 54.6%, p = 0.54). CONCLUSIONS: The association of BA with various anomalies should be considered a spectrum that may vary widely from patient to patient. The finding of two or more of these malformations in a patient awaiting transplantation should lead the surgeon to look systematically for other associated anomalies. With some special surgical considerations, the outcome in BA-PS patients should not differ from those with isolated BA.     

Internal carotid artery aneurysm and Marfan's syndrome

Aneurysms of the extracranial carotid artery are most commonly caused by atherosclerosis or trauma but may also have unusual causes, such as Marfan's syndrome. Although aneurysmal changes in the extracranial carotid vessels usually are due to extension of aortic dissection into the carotid system, isolated aneurysms may occasionally complicate Marfan's syndrome. The authors report a case of Marfan's syndrome in which the patient, a 45-year-old woman, presented with an asymptomatic mass in the right side of the neck at the level of the carotid bifurcation. An isolated internal carotid artery aneurysm was identified. The aneurysm was resected using an interposition vein graft over an outlying shunt. Histologic examination confirmed the typical cystic medial necrosis with loss of elastic fibres and an increase of mucoid material in the media. The patient's recovery was smooth and at 2-year follow-up there were no signs of recurrent aneurysm formation.     

Hand deformities in Freeman-Sheldon syndrome and their surgical treatment

The Freeman-Sheldon-Syndrome is a rare complex of malformations with typical deformities of face, hands and feet. The individual variability of the characteristics seems to be very big, as could see in our own patients (9 cases) as well as in literature (43 cases). The most frequent and most important characteristics are discussed, mainly the deformity of the hand. Six hands have been operated on for functional improvement; different methods were used. The primary objective of our treatment was the correction of thumb deformity.