Preventing the Catastrophe: Aberrant Carotid in Adenoid and Tonsil Surgery: Two Case Reports

Background: Adenoid and tonsil surgeries are the most commonly done surgical procedures, with haemorrhage being an important complication. Fatalities due to haemorrhage almost never occur, but the occasional devastating outcomes that are life-threatening become a frequent source of litigations and audits. Catastrophic bleeds are usually due to an aberrant vessel or carotid protruding in the pharyngeal airway. The aberrant carotid poses a risk during routine pharyngeal surgeries like adenoidectomy, tonsillectomy, Quincy and Para-pharyngeal abscess drainage, UPPP, pharyngeal biopsies, etc. and injuries during routine pharyngeal surgical procedures can be catastrophic due to massive bleeding. Case report: We report two cases of routine Adeno-tonsillectomy surgeries where aberrant carotid vessels protruding in the adeno-tonsil surgical area were identified by endoscopic transoral evaluation intraoperatively. The surgery in these cases was postponed for further investigation due to the risk of injury and catastrophic bleeding. Conclusion: Aberrant carotids are usually clinically silent, and adenoid removal in most cases is a blind procedure done by curettage. There are no guidelines to identify aberrant vessels pre or intraoperatively, and preventing injury and catastrophic bleeding depends on surgeon’s experience and caution. These cases underline the importance of due vigilance and taking steps before starting the procedures, particularly pre-and intraoperative transoral endoscopic assessment for identifying aberrant vessels in the upper airway area, thus preventing injury and avoiding devastating complications. We report these cases and underline the steps to identify aberrant vessels in the upper airway surgical field before starting surgery to prevent a potentially catastrophic complication.

Kaposi’s sarcoma-associated herpesvirus vFLIP promotes MEndT to generate hybrid M/E state for tumorigenesis

Kaposi sarcoma (KS) is an angioproliferative and invasive tumor caused by Kaposi sarcoma-associated herpesvirus (KSHV). The cellular origin of KS tumor cells remains contentious. Recently, evidence has accrued indicating that KS may arise from KSHV-infected mesenchymal stem cells (MSCs) through mesenchymal-to-endothelial transition (MEndT), but the transformation process has been largely unknown. In this study, we investigated the KSHV-mediated MEndT process and found that KSHV infection rendered MSCs incomplete endothelial lineage differentiation and formed hybrid mesenchymal/endothelial (M/E) state cells characterized by simultaneous expression of mesenchymal markers Nestin/PDGFRA/α-SAM and endothelial markers CD31/PDPN/VEGFR2. The hybrid M/E cells have acquired tumorigenic phenotypes in vitro and the potential to form KS-like lesions after being transplanted in mice under renal capsules. These results suggest a homology of KSHV-infected MSCs with Kaposi sarcoma where proliferating KS spindle-shaped cells and the cells that line KS-specific aberrant vessels were also found to exhibit the hybrid M/E state. Furthermore, the genetic analysis identified KSHV-encoded FLICE inhibitory protein (vFLIP) as a crucial regulator controlling KSHV-induced MEndT and generating hybrid M/E state cells for tumorigenesis. Overall, KSHV-mediated MEndT that transforms MSCs to tumorigenic hybrid M/E state cells driven by vFLIP is an essential event in Kaposi sarcomagenesis.

Surgical strategy for pulmonary sequestration: Focus on precautions for aberrant vessels under minimally invasive surgery

Background Recently, thoracoscopic resection of pulmonary sequestration has become more common, since resection of an aberrant artery using an end-stapler is a safe maneuver in many cases. However, injury of the vessels can lead to major hemorrhage. We reported our surgical experience based on thoracoscopic surgery, with five cases of interlobar pulmonary sequestration, focusing on precautions for aberrant arterial vessels. Object and methods We performed pulmonary resections for five patients with interlobar pulmonary sequestration in a lower lobe (left, n = 4; right, n = 1) between April 2004 and May 2020. All aberrant vessels were derived from the lower thoracic artery. Two patients had a single aberrant artery and three had multiple. In four patients, these vessels were detected before surgery, and pulmonary sequestration was diagnosed in four. In one elderly patient, the aberrant vessel was overlooked, and lung cancer was suspected before surgery. Angiography or multidetector-row computed tomography was subsequently performed in four cases. The surgical plan was determined according to the location and size of the pulmonary lesion and three-dimensional images of aberrant vessels. Result In all patients, approaches were made thoracoscopically. Hemorrhage from an anomalous vessel was encountered in one case. Pulmonary resections included two lobectomies and three limited resections. Angioplasty for the root of anomalous branches was performed following pulmonary resections under converted minimal lateral thoracotomy in two cases. Conclusion Preoperative assessment of the anatomical variations in abnormal vessels is essential to achieve safe surgical procedures. According to the situation of the aberrant vessels, selecting surgical procedures with consideration of potential subsequent complications arising over a long period of time is important.

Abernethy Malformation Type II in a 70-Year-Old Patient with Angina Pectoris

Congenital extrahepatic portosystemic shunts (ECPSS) are rare developmental anomalies in which a variable portion of the portal blood bypasses the liver and is shunted in the systemic circulation via one or more aberrant vessels. We present a clinical case of a 70-year-old man, who was referred to the Cardiology Department because of exertional dyspnea, fatigue, and feeling of heaviness and pressure behind the sternum. MDCT of the aorta was performed and an aberrant vessel was discovered with communication with the left iliac vein on one side and superior mesenteric and splenic veins on the other. The portal vein was hypoplastic. The radiologic findings were suggestive of malformation of Abernethy. The ECPSS can be classified into 2 main groups (with complete and partial shunting). The patients have different clinical presentation. Some of them are completely asymptomatic while in others the shunt can manifest even before birth as fetal growth retardation or in the early neonatal period with neonatal cholestasis and galactosemia. Common complications are hepatic encephalopathy and hepatopulmonary syndrome and there is a wide variety of concomitant abnormalities. The imaging modalities play a crucial role in the diagnosis, classification, follow-up and the proper choice of therapeutic management in patients with ECPSS.

A case of hemichorea in RNF213-related vasculopathy

Background Internal carotid artery (ICA) stenosis has been recently reported to cause hemichorea, mainly in East Asia. The East Asian-specific p.R4810K variant of RNF213, a susceptibility gene for moyamoya disease (MMD), accounts for up to 25% of sporadic ischemic stroke with ICA stenosis cases in East Asia. However, as RNF213-related vasculopathy does not meet the diagnostic criteria for MMD, the creation of a new disease category has been suggested. Here, we report the first case of hemichorea in RNF213-related vasculopathy. Case presentation An 81-year-old woman was admitted to our hospital with choreic movements in the periphery of the right extremities at rest. Though head magnetic resonance imaging showed no fresh or old cerebral infarction, 123I-iodoamphetamine-single photon emission computed tomography showed cerebral blood flow of < 80% in the anterior territory of the left middle cerebral artery (MCA) in a resting state and cerebrovascular reactivity of < 10% in the broader area supplied by the left MCA after acetazolamide challenge. Head magnetic resonance angiography and digital subtraction angiography revealed left ICA C1 portion stenosis with compromised collateral vessels. Involuntary movements resolved with haloperidol administration within 3 days, without apparent recurrence from continuation of the medication for a year. Genetic testing revealed the presence of the heterozygous RNF213 p.R4810K variant. Conclusions Chorea is thought to be caused by damage to circuitry connecting the basal ganglia with the cerebral cortex, as found in cases of MMD, which possess aberrant vessels in the basal ganglia. However, aberrant vessels and cerebral infarctions were not observed in the basal ganglia in the current case, decreasing the likelihood of a role in chorea. Alternatively, as RNF213 regulates vascular endothelial function and angiogenesis, dysregulation may impair the neurovascular unit and damage basal ganglia circuitry, contributing to the development of chorea. This case may renew interest in the concept of RNF213-related vasculopathy and the pathophysiological mechanisms behind chorea in ICA stenosis.

A Modified VATS Procedure for Treating Adult Intralobar Pulmonary Sequestration

Video-assisted thoracoscopic surgery (VATS) makes it possible to treat intralobar sequestration (ILS) more minimally invasive compared with conventional open surgery. However, this procedure is challenging to expose and isolate the aberrant arteries of ILS and the risk of bleeding is high. Herein, we developed a modified VATS procedure in which the aberrant vessels are treated in the last step of lobectomy, rather than at the beginning. In this way, we can expose the aberrant vessels easier and reduce the risk of massive blood loss, also simplifying the surgical procedure.

Ganglioneurofibroma arising within the extralobar pulmonary sequestration

Background Neurogenic tumor arising within the pulmonary sequestration (PS) is rare. Case presentation A 42-year-old asymptomatic female was referred to our hospital for work-up of extralobar PS. The independent feeding artery from the thoracic aorta was confirmed by three-dimensional computed tomography angiography (3D-CTA). Uniportal thoracoscopic resection of the sequestrated lung with mediastinal lymph node sampling was performed successfully. Ganglioneurofibroma within the PS was diagnosed as the specimen revealed positive expression of SRY-related HMG-box 10 protein, neuron-specific enolase, S-100, chromogranin A and synuclein. Tumor recurrence was not recorded 1 year after the surgery. Conclusion Preoperative 3D-CTA is useful to identify the aberrant vessels of PS. An elaborate diagnostic work-up after a timely resection is necessary for subsequent management and follow-up plan.

Ganglioneurofibroma Arising Within the Extralobar Pulmonary Sequestration

Background: Neurogenic tumor arising within the pulmonary sequestration (PS) is rare.Case Presentation: A 42-year-old asymptomatic female was referred to our hospital for work-up of extralobar PS. The independent feeding artery from the thoracic aorta was confirmed by three-dimensional computed tomography angiography (3D-CTA). Extrapulmonary lesions were excluded by whole-body radiographical examinations. Uniportal thoracoscopic resection of the sequestrated lung and mediastinal lymph node sampling was performed successfully. Finally, ganglioneurofibroma within the PS was confirmed by pathological staining. The patient was discharged from the hospital on the 3rd postoperative day without tumor recurrence a year after surgery.Conclusion: Preoperative 3D-CTA is useful to verify the aberrant vessels of PS; whereas an elaborate diagnostic work-up after a timely resection is necessary for possible subsequent management and follow-up plan.

Herniation of the tuft with outgrowth of vessels through the glomerular entrance in diabetic nephropathy damages the juxtaglomerular apparatus

As shown in our previous paper (Kriz W, Löwen J, Federico G, van den Born J, Gröne E, Gröne HJ. Am J Physiol Renal Physiol 312: F1101–F1111, 2017), mesangial matrix expansion in diabetic nephropathy (DN) results for a major part from the accumulation of worn-out undegraded glomerular basement membrane material. Here, based on the reevaluation of >900 biopsies of DN, we show that this process continues with the progression of the disease finally leading to the herniation of the matrix-overloaded tuft through the glomerular entrance to the outside. This leads to severe changes in the glomerular surroundings, including a dissociation of the juxtaglomerular apparatus with displacement of the macula densa. The herniation is associated with a prominent outgrowth of glomerular vessels from the tuft. Mostly, these aberrant vessels are an abnormal type of arteriole with frequent intramural insudations of plasma. They spread into glomerular surroundings extending in intertubular and periglomerular spaces. Their formation is associated with elevated mRNA levels of vascular endothelial growth factor-A, angiopoietins 1 and 2, and the corresponding receptors. Functionally, these processes seem to compromise tubuloglomerular feedback-related functions and may be one factor why Na+-glucose cotransporter-2 inhibitors are not effective in advanced stages of DN.