Endarterectomy of carotid artery bifurcation in the setting of a persistent hypoglossal artery and anomalous collateral vascular supply.

Presented is a patient with carotid artery stenosis resulting in crescendo anterior and posterior circulation transient ischemic attacks. Treatment was complicated by a rare persistent hypoglossal artery (HGA) arising from the left internal carotid artery in addition to severe contralateral carotid disease, hypoplastic vertebral arteries, and incomplete circle of Willis. A carotid endarterectomy with shunting was performed, maintaining perfusion of both the proper left internal carotid artery and HGA. This is a rare case of carotid stenosis in the setting of a persistent HGA with contralateral carotid disease and highlights the importance of planning intracranial perfusion before carotid surgery.

A Novel Recurrent COL5A1 Genetic Variant Is Associated With a Dysplasia-Associated Arterial Disease Exhibiting Dissections and Fibromuscular Dysplasia.

OBJECTIVE: While rare variants in the COL5A1 gene have been associated with classical Ehlers-Danlos syndrome and rarely with arterial dissections, recurrent variants in COL5A1 underlying a systemic arteriopathy have not been described. Monogenic forms of multifocal fibromuscular dysplasia (mFMD) have not been previously defined. Approach and Results: We studied 4 independent probands with the COL5A1 pathogenic variant c.1540G>A, p.(Gly514Ser) who presented with arterial aneurysms, dissections, tortuosity, and mFMD affecting multiple arteries. Arterial medial fibroplasia and smooth muscle cell disorganization were confirmed histologically. The COL5A1 c.1540G>A variant is predicted to be pathogenic in silico and absent in gnomAD. The c.1540G>A variant is on a shared 160.1 kb haplotype with 0.4% frequency in Europeans. Furthermore, exome sequencing data from a cohort of 264 individuals with mFMD were examined for COL5A1 variants. In this mFMD cohort, COL5A1 c.1540G>A and 6 additional relatively rare COL5A1 variants predicted to be deleterious in silico were identified and were associated with arterial dissections (P=0.005). CONCLUSIONS: COL5A1 c.1540G>A is the first recurring variant recognized to be associated with arterial dissections and mFMD. This variant presents with a phenotype reminiscent of vascular Ehlers-Danlos syndrome. A shared haplotype among probands supports the existence of a common founder. Relatively rare COL5A1 genetic variants predicted to be deleterious by in silico analysis were identified in ≈2.7% of mFMD cases, and as they were enriched in patients with arterial dissections, may act as disease modifiers. Molecular testing for COL5A1 should be considered in patients with a phenotype overlapping with vascular Ehlers-Danlos syndrome and mFMD.

Gas in the inferior vena cava from severe emphysematous pyelonephritis.

Emphysematous pyelonephritis is a life-threatening, urologic emergency. We present the case of a 54-yearold female who was found to have a large volume of air in her renal vein and inferior vena cava from severe emphysematous pyelonephritis. In this case report, the medical and operative management of this patient are reviewed, and the literature on the management of emphysematous pyelonephritis is summarized.

Renovascular hypertension from the BCR-ABL tyrosine kinase inhibitor ponatinib.

Drug-induced hypertension is one of the commonest causes of secondary hypertension. In the last few years, secondary hypertension due to tyrosine kinase inhibitors, from the vascular endothelial growth factor class, has been recognized to be an important cause of hypertension, as well as proteinuria, and occasionally kidney dysfunction in some cases. Less well-recognized is that BCR-ABL tyrosine kinase inhibitors also have adverse vascular effects. These manifest as vascular stenoses in large vessels, which may sometimes cause renal artery stenosis and subsequent hypertension. We describe a case report which presented as classical bilateral renal artery stenosis, and responded to revascularization. Increased awareness of these effects, as well as research into the pathogenesis, may provide more insight into vascular biology.

Splenosis involving the gastric fundus, a rare cause of massive upper gastrointestinal bleeding: a case report and review of the literature.

Splenosis, the autotransplantation of splenic tissue following splenic trauma, is uncommonly clinically significant. Splenosis is typically diagnosed incidentally on imaging or at laparotomy and has been mistakenly attributed to various malignancies and pathological conditions. On the rare occasion when splenosis plays a causative role in a pathological condition, a diagnostic challenge may ensue that can lead to a delay in both diagnosis and treatment. The following case report describes a patient presenting with a massive upper gastrointestinal bleed resulting from arterial enlargement within the gastric fundus secondary to perigastric splenosis. The cause of the bleeding was initially elusive and this case highlights the importance of a thorough clinical history when faced with a diagnostic challenge. Treatment options, including the successful use of transarterial embolization in this case, are also presented.

Technique for salvage ERCP with gastric bypass anatomy and severe intra-abdominal adhesions.

BACKGROUND: Choledocholithiasis is a complex problem in patients with Roux-en-Y gastric bypass anatomy. Several techniques of biliary clearance have been described, but these can be limited by intra-abdominal adhesions. PATIENT AND METHODS: A 36-French surgical gastrostomy was created and was allowed to mature for 10 weeks. It was exchanged for a 15-mm laparoscopic surgery trocar under fluoroscopic guidance. Endoscopic retrograde cholangiopancreatography (ERCP) was carried out using the trocar as a stable access point. Complete biliary clearance was achieved in one sitting using sphincterotomy, large-diameter biliary orifice balloon dilation, and balloon/basket sweeps. RESULTS: Total endoscopy time was 120 minutes. There were no complications associated with the procedure. The postprocedure length of stay was 2 days. The total bilirubin level at discharge was 1.2 mg/dL (20 µmol/L). CONCLUSIONS: In patients with gastric bypass anatomy and severe adhesions, successful salvage therapeutic ERCP can be achieved using a gastrostomy tract and a large-bore laparoscopy trocar for access to the defunctioned stomach.

Improved strength of early versus late supraspinatus tendon repair: a study in the rabbit.

HYPOTHESIS: The optimal timing for surgical repair of the supraspinatus (SSP) tendon after full-substance tear has not been established. The objectives of this prospective investigation of SSP tendon repair delayed by 1, 2, or 3 months followed by a 3-month postoperative course were to (1) determine the site of failure, (2) measure the tensile strength and stiffness, and (3) assess the ability of computed tomography to predict mechanical strength. MATERIALS AND METHODS: We transected 1 SSP tendon in 36 rabbits and then repaired it with transosseous sutures after a delay of 1, 2, or 3 months. We compared the results with 36 intact shoulders from 18 age-matched control rabbits. RESULTS: Experimental specimens failed at the tendon (n = 26) more often than at the enthesis (n = 10) (P < .05). The mean peak loads to failure 3 months after repair delayed by 1 month and delayed by 2 months were significantly greater than their respective control values (P < .05 for both); there was no difference after a delay of 3 months. There was no association between the presence of hypoattenuation on computed tomography and repair strength (P > .05). CONCLUSIONS: Our findings indicate better mechanical results with earlier repair (1 or 2 months) after SSP tendon than after a delay of 3 months. Early surgical repair may lower the risk of tendon retear.

Extra- and intramuscular fat accumulation early after rabbit supraspinatus tendon division: depiction with CT.

PURPOSE: To quantify the accumulation, progression, and distribution of fat separately in and around the supraspinatus muscle from the onset of tendon detachment and to validate computed tomography (CT) for quantification of fat content by using volumetry and histomorphometry as reference standards. MATERIALS AND METHODS: Institutional animal care committee approval was obtained. The supraspinatus tendon of 30 adult female rabbits (3.0 kg) was detached. Rabbits were sacrificed in groups of 10 after 4, 8, and 12 weeks. Fifteen rabbits that did not undergo surgery served as control subjects. Fat accumulations in and around the proximal, middle, and distal supraspinatus muscle were quantified, volumetrically and histologically, and were compared with extramuscular presence and intramuscular attenuation at CT. Weight, volume, histologic findings, and CT determinations of fat were compared by using one-way analysis of variance. The Pearson test was used to correlate intramuscular fat accumulation with CT observations. RESULTS: Four weeks after tendon detachment significant extra- and intramuscular fat had accumulated (both P < .05) and progressed during 12 weeks, with accumulation of extramuscular fat being three times greater than that of intramuscular fat (both P < .05). An increasing proximal-to-distal gradient existed for both extra- and intramuscular accumulation (both P < .05). CT depicted the onset, progression, and gradient of extra- and intramuscular fat accumulation (all P < .05). CT attenuation correlated strongly with histologic findings (P < .05). CONCLUSION: Fat accumulated early, along an increasing proximal-to-distal gradient, and progressed with time after supraspinatus tendon detachment. CT proved to be a valid tool for monitoring the onset, progression, and gradient separately for extra- and intramuscular fat accumulation.

Supraspinatus tendon repair into a bony trough in the rabbit: mechanical restoration and correlative imaging.

Recurrence of tears is a common complication after rotator cuff surgery. Retearing seems to occur early after surgery and may be attributed to too early or too vigorous exercises. We found no experimental data correlating the strength of the rotator cuff early after surgery and imaging. Our objectives were to measure the peak load to failure of rabbit supraspinatus tendon-bone constructs at early times postoperatively, to determine their mode of failure, and to determine whether computed tomography (CT) can predict their strength. We divided one supraspinatus tendon of 40 adult female white New Zealand rabbits and, after resection of the enthesis, sutured the tendon into a bony trough. Ten rabbits were killed immediately and 10 each at 1, 2, and 6 weeks postoperatively. The explanted tendons of both shoulders were imaged on CT and tested to failure. Compared to normal tendons (mean 210 +/- 42 N), the mean strength was very low at 0 weeks (57 +/- 21 N) and 1 week (86 +/- 33 N) (both p < 0.05); it had recovered by 6 weeks (324 +/- 66 N). Early on, suture pullout was the most common mode of failure, whereas at 6 weeks, mid-substance tears predominated (p < 0.05). Hypoattenuation on CT was associated with increased strength of the tendon-bone construct (p < 0.05). The strength of the surgical construct is very low in the early postoperative period. Therefore, the shoulder should be submitted only to loads not interfering with healing.