Hypoplasia of the descending thoracic and abdominal aorta: a report of two cases and review of the literature

Hypoplasia of the thoracic and abdominal aorta, referred to as atypical, elongated, or diffuse coarctation, is an exceedingly rare cardiovascular anomaly. Congenital, acquired, inflammatory, and infectious etiologies have been described. Symptoms typically occur within the first three decades of life and include hypertension, lower extremity claudication, and mesenteric ischemia. The condition is considered a life-threatening emergency as a result of the complications associated with severe hypertension. Diagnosis is best made with angiography. Surgical bypass grafting is the optimal method of treatment and must be tailored depending on the distribution of disease. We report two cases of diffuse hypoplasia involving the thoracic and abdominal aorta treated with thoracic aorta to abdominal aorta bypass.     

Penetrating ulcers of the descending thoracic aorta: Diagnosis and surgical management

In this paper two patients with penetrating ulcers of the descending thoracic aorta are presented, a clinical condition recognized only recently, that may have a severe or even fatal outcome, due to rupture of the aorta. It is a peculiar form of the atherosclerotic disease, and its clinical and imagiologic presentation may mimics the classic aortic dissection of the distal type type. Computed tomography, nuclear magnetic resonance and transesophageal ecocardiography are the most apropriate methods for studying and diagnosis and the surgical management, aimed at the prevention or treatment of the aortic rupture, and based on the prosthetic aortic replacement, is the only alternative allowing the definite cure of the disease. The main features of pathology, clinical manifestations, diferential diagnosis and surgical management of this entity are described and discussed, according to the most recently articles published in the literature, on the subject.     

Traumatic rupture of the thoracic aorta: ten years of delayed management

OBJECTIVE: Traumatic rupture of the thoracic aorta is a highly fatal condition in which patient outcome is strongly conditioned by other associated injuries. Delayed aortic treatment has been proposed to improve results. METHODS: The charts of 69 patients with traumatic rupture of the thoracic aorta observed between 1980 and 2003 were reviewed. Patients were grouped according the timing of repair: group I, immediate repair (21 patients); and group II, delayed repair (48 patients). In group II, 45 patients were treated surgically or by endovascular procedure. RESULTS: In-hospital mortalities were 4 of 21 patients (19%) in group I and 2 of 48 patients (4.2%) in group II. There were 3 cases of paraplegia in group I and none in group II. CONCLUSION: Improvement of patient outcome with traumatic rupture of the thoracic aorta can be achieved by delaying surgical repair until after management of major associated injuries if there are no signs of impending rupture. Endovascular treatment is feasible and safe and may represent a valid alternative to open surgery in selected cases.     

Thrombosed tricuspid valves presenting some 20 years after the surgical correction of Ebstein's anomaly: Report of two cases

We present herein the cases of two patients who were diagnosed as having thrombosed tricuspid valves by electrocardiography after undergoing valve replacement with Starr-Edwards caged ball valves for Ebstein's anomaly 19 and 27 years earlier. In both patients, the valves were found to be attached to the right ventricular septum and covered with massive thrombus, the balls being completely jammed in a half-open position in the cages. Re-replacement of the valves was successfully performed with Hancock II porcine heterograft prostheses and both patients are now classified as being in Functional Class I or II.     

Syphilitic aneurysm of the abdominal aorta: report of two cases

We have described two histologically confirmed syphilitic abdominal aortic aneurysms arising below the renal arteries. Serological tests were negative and the patients had in the past been treated with penicillin and bismuth. We consider that there may be a correlation between atherosclerosis and syphilis in the natural history of some abdominal aortic aneurysms and the hazard of indiscriminate use of antibiotics for the treatment of other inflammatory vessel disease may allow syphilis to develop insidiously.     

Coarctation of the abdominal aorta: current options in surgical management.

Coarctation or hypoplasia of the abdominal aorta is a rare cause of life-threatening hypertension. In most cases the mechanism of hypertension is elevated blood renin levels secondary to associated renal artery stenosis. Medical control of the hypertension is often difficult, and thus patients usually require renal artery revascularization combined with aortic bypass or replacement early in life. Current surgical management should optimize the use of autogenous methods of renal artery reconstruction including saphenous vein aortorenal bypass, splenorenal arterial anastomosis, hepatorenal saphenous vein bypass, and renal autotransplantation. In selected patients the reconstruction can be staged by correction of the renal artery stenosis and postponement of definitive repair of the aortic coarctation until it becomes hemodynamically significant.     

Dissecting thoracic aorta and fusiform aneurysm of the abdominal aorta

A 59-year-old woman with dissection of the thoracic aorta and a fusiform aneurysm of the abdominal aorta without evidence of Marfan's syndrome underwent aneurysmorrhaphy with a bifurcated expanded polytetrafluoroethylene graft. Histological specimens of the aneurysmal wall revealed the presence of idiopathic cystic medial necrosis. As typical findings of idiopathic cystic medial necrosis in the aortic wall are very rare except in cases of Marfan's syndrome, the present case is reported and the implications of this condition are discussed.     

Secondary surgical interventions after endovascular stent-grafting of the thoracic aorta.

OBJECTIVE: The objective of the study was to evaluate mid-term durability and need for reinterventions after endovascular stent-grafting (ESG) in descending aortic aneurysms and dissections. PATIENTS AND METHODS: Between November 1996 and February 2003 a total of 80 patients underwent ESG for the following indications: atherosclerotic aneurysms (50/80; 63%), type B dissections (20/80; 25%), penetrating ulcers (6/80; 8%), traumatic aneurysms (4/80; 5%). Two types of commercially available ESG (Talent, Medtronic, Santa Rosa, CA and Excluder, WL GORE, Flagstaff, AZ) were inserted via the femoral artery in 53 patients, via the iliac artery in 21 patients and via the abdominal aorta in 6 patients. RESULTS: In-hospital mortality was 3.8% (n=3). Type I endoleak formation requiring endovascular reintervention was observed in 3.8% (n=3). Surgical reintervention became necessary in 4 patients (4/80; 5%). One patient experienced a retrograde type A dissection, detected in the 3 month control after ESG of an acute type B dissection, consecutively undergoing frozen elephant trunc repair. Three patients with late type I endoleak formation (mean interval: 62 months) after ESG (two atherosclerotic aneurysms, one penetrating ulcer) underwent open thoraco-abdominal repair in deep hypothermia or left heart bypass technique. All patients had an uneventful postoperative course. CONCLUSIONS: Occurrence of late endoleak formation requiring surgical reintervention after ESG is acceptably low. In atherosclerotic aneurysms the development of endoleaks is assumed to be causative related to progression of the underlying aortic disease. A close follow-up of patients after ESG is crucial.     

Congenital pyloric atresia,presentation, management,and outcome: A report of 20 cases

Background

Congenital pyloric atresia (CPA) is a very rare anomaly. It is usually seen as an isolated condition with excellent prognosis. Few cases are familial. These are usually associated with other hereditary conditions and have a poor prognosis. This is a review of our experience with 20 patients with CPA outlining aspects of diagnosis, associated anomalies and management.

Patients and methods

This is a retrospective analysis of 20 cases seen over a 22 year period (December 1990 to December 2012). Their records reviewed for: age, sex, presentation, prenatal history, associated anomalies, investigations, treatment, operative findings and the outcome.

Results

20 cases (9 Males, 11 Females) were treated. 7 patients were full term and the remaining 13 were prematures. Their mean birth weight was 2.1 kg (1.1 kg to 3.9 kg). Polyhydramnios was seen in 13 patients (65%). Two were brothers and four were members of the same family. Isolated CPA was seen in 7 (35%); 13 had an associated conditions: epidermolysis bullosa (EB) in 8 (40%) and multiple intestinal atresias (MIA) in 5 (25%). Three patients had associated esophageal atresia. All were operated on except two who died early due to unrelenting sepsis. The variety of pyloric atresias encountered were as follows: pyloric diaphragm in 13 including double diaphragms in 2, pyloric atresia with a gap in 4 and pyloric atresia without gap in 3. Ten died postoperatively giving an overall survival of 40%.

Conclusions

CPA is a very rare condition. Isolated CPA carries a good prognosis. Association of CPA with other familial and congenital anomalies like EB and MIA carries a poor prognosis.     

Nonpenetrating trauma of the abdominal aorta with partial vessel occlusion: report of two cases.

Two cases of blunt trauma of the abdominal aorta are reported. In both there was lacreation of the intima and development of intimal flaps with only partial occlusion of the aorta. Immediate recognition in one allowed prompt repair of injured tissue. Delayed recognition in the other, while not life-threatening, did prolong disability and did necessitate vascular tissue replacement with prosthetic graft. Audible bruit and palpable thrill are diagnostic signs that, if sought for in the emergency room and operating room examinations of the traumatized abdomen, will yield early recognition of this lesion.     

Management of injuries of the thoracic and abdominal aorta

Thirty-five patients had surgery for injuries of the aorta at the Los Angeles County-USC Medical Center over a 4 1/2 year period. There were 27 survivors. The principles of management were to operate without delay if there was evidence of continued bleeding after initial fluid replacement as occurred in 11 patients. For the 24 patients who became stable after initial resuscitation, a more deliberate plan of management was used. Blood pressure was carefully monitored and controlled to avoid hypertension. Priorities for associated injuries were established and in several cases, they took treatment precedence over the aortic injury. Delay was sometimes necessary to utilize the more experienced personnel. In no instance did a stabilized patient hemorrhage during the delay. The most common injury seen was a blunt disruption of the proximal descending aorta. The details of the operative technique for this injury have been reported herein, along with a justification for not using either pump bypass or shunt to perfuse the distal aorta during the period of aortic cross-clamping.     

腹部软组织平滑肌肉瘤外科治疗体会(附11例报告)

目的:探讨腹部软组织平滑肌肉瘤的临床表现、外科治疗及预后特点。方法:回顾性分析我院2009年7月至2012年3月收治的11例经手术及病理证实的腹部软组织平滑肌肉瘤的临床资料。结果 :男3例,女8例,中位年龄54(34～66)岁,腹部肿块及腹痛是主要的临床表现。肿瘤的根治切除率为72.7%,联合脏器等切除率为63.6%,10例中3例术后复发。结论:腹部软组织平滑肌肉瘤多见于女性,好发于腹膜后;根治性手术是治疗的主要手段;影像学评估及多学科协作在腹部软组织平滑肌肉瘤的外科治疗中起重要的作用。