Embolization of a hepatoportal fistula in a patient with Ehlers-Danlos syndrome and colon perforation.

Patients with type IV Ehlers-Danlos syndrome (EDS) have defective collagen synthesis, which places them at risk for spontaneous vascular rupture and other clinical sequelae. After treatment with resection and colostomy, a hepatoportal fistula developed in a 16-year-old boy with type IV EDS. The fistula was embolized angiographically by transcatheter coil occlusion. Management options for patients with type IV EDS and these sequelae are reviewed.

Hematuria caused by infrahepatic interruption of the inferior vena cava.

We report a case of infrahepatic absence of the inferior vena cava, which was believed to be the cause of painless gross hematuria. Diagnosis was based on the results of computerized tomography and venacavography. Hemodynamic studies showed normal pressure in the inferior vena cava, superior vena cava and portal vein.

Thrombosis of the renal artery of a small, solitary kidney: successful return of renal function after prolonged anuria.

In the presence of a single functioning kidney, renal artery obstruction produces anuria, which can require hemodialysis. If the problem is diagnosed immediately and surgical intervention is not delayed, revascularization of the ischemic kidney is usually successful. Few authors, however, have reported the return of function to a small solitary kidney after occlusion lasting longer than 2 hours. We describe a case that involved thrombosis of the renal artery to an 8-cm solitary kidney; a successful endarterectomy was performed 29 hours after the onset of anuria. This case shows that the reversibility of renal ischemia is not necessarily determined by either the duration of occlusion or the size of the affected kidney.

Surgical correction of anomalous left coronary artery from the anterior sinus of valsalva.

Origination of the left main coronary artery from the anterior sinus of Valsalva is a rare congenital anomaly that has been associated with sudden death. Anatomical correction of this defect has only rarely been performed by unroofing the intramural segment of the proximal coronary artery or by widening the narrowed angulated origin of this anomalous vessel. This report describes the clinical course of a 33-year-old woman with this anomaly who experienced episodes of chest tightness associated with ischemic electrocardiographic changes. Surgical correction was performed by enlarging the ostium of the anomalous artery with a "sphincteroplasty" technique. Thallium scanning, which was positive prior to surgery, was normal postoperatively. The patient remains asymptomatic 6 months after surgical correction.

Surgical treatment of aneurysm of the ascending aorta with aortic insufficiency. A selective approach.

The selection of an appropriate surgical technique for repair of aneurysm of the ascending thoracic aorta with associated aortic insufficiency is unsettled. Placement of a supracoronary graft (separate G/V) is a compromise if the coronary ostia are displaced cephalad by the aneurysm, whereas insertion of a valved conduit is difficult and unnecessary if the coronary ostia are normally placed. From June, 1979, to December, 1982, 140 patients underwent repair of ascending aortic aneurysm with aortic valve replacement (AVR). Mean age was 46 years. Annuloaortic ectasia was the most common indication for repair (71/140, 50.7%), followed by acute and chronic dissection (47/140, 33.6%). Twelve patients had undergone previous operations on the ascending aorta or aortic valve, including five separate G/V repairs. Eighty-nine patients (63.6%) underwent composite replacement with coronary reimplantation and 51 (36.4%) had separate G/V repair or primary repair of the aneurysm. Cardiopulmonary bypass methods, times, and postoperative complications were comparable between the two groups. Hospital mortality for the whole series was 7.9% (11/140), with 5.6% (5/89) in patients having conduit replacements and 13.7% (7/51) in patients having separate G/V repair. Mortality correlated with separate G/V repair in patients with annuloaortic ectasia (p = 0.005) and with conduit repair of atherosclerotic aneurysms (p = 0.05). Among 90 patients followed up a total of 1,778 patient-months, there were seven late deaths: three new dissections, two sudden deaths without autopsy, and two patients with chronic congestive heart failure unimproved or made worse with the operation. Notably, no patient has required reoperation for conduit malfunction or has required repair of aneurysm or paravalvular leak below a supracoronary graft. Clinical anatomic assessment at operation should determine the technique of repair employed, based on the degree of displacement of the coronary ostia relative to the aortic anulus.

Resection of the aortic arch with moderate hypothermia and temporary circulatory arrest.

Resection of the aortic arch with the use of moderate hypothermia and temporary circulatory arrest was performed in a 63-year-old woman. The simplified technique is described, along with a brief review of the literature. The patient recovered uneventfully. After this manuscript was prepared, two other patients underwent successful replacement of the aortic arch for aneurysm. These cases are also summarized briefly in an illustrated table.

Acute infrarenal aortic thrombosis during aortoiliac reconstruction.

Two patients developed acute infrarenal aortic thrombosis while undergoing aortoiliac reconstruction. This condition was suspected when there was a sudden loss of aortic pulsations that were previously present. Once diagnosed, a prompt and aggressive surgical approach was necessary, as described in this report.