Aberrant Vein Research Articles

Biliary atresia with rare associations: a case report

BackgroundIt is not often written in medical journals that preduodenal portal vein, biliary atresia, intestinal malrotation, and situs inversus totalis are all related. This is a rare association.Case reportsA 2-month-old female infant had biliary atresia type III, situs inversus totalis, midgut malrotation, and a preduodenal portal vein. She had been operated on by the Kasai procedure (hepato-portoenterostomy).DiscussionIt is important to carefully look into the relationship between the preduodenal portal vein and biliary atresia because the patient is at risk of injury from this aberrant vein during operative intervention.ConclusionThe relationship between biliary atresia and other congenital anomalies like preduodenal portal vein, intestinal malrotation, and situs inversus must be taken into consideration to avoid other diverse effects during surgical intervention.

Role of Digital Subtraction Angiography in the Preoperative Assessment of a Patient with a Petrosquamous Sinus: a Case Report.

We report a case of a 15-year-old Indian girl who presented with tinnitus, pain and ear discharge for one month and was preliminarily diagnosed with Chronic Suppurative Otitis Media (CSOM) with mastoiditis. She underwent a routine presurgical CT scan which revealed an aberrant vein, making it essential to exercise caution during surgery for CSOM. The aberrant vessel was identified as a Petrosquamous Sinus. A Petrosquamous Sinus is a persistent fetal vein that connects the transverse sinus with the retromandibular vein and may regress in an individual by birth. Its importance lies in the risk of haemorrhage it carries during otological surgeries. A Digital Subtraction Angiography proved to be a vital step in isolating the path of the vein for better visualisation of the course, thus giving a better idea about the anatomical relations of the vessel during the surgery. The tympanoplasty was performed with care to prevent damaging the vein. The patient had no complications in the postoperative period and made a quick recovery.

Normal variations in MR venography that may cause pitfalls in the diagnosis of cerebral venous sinus thrombosis

Objectives: Magnetic resonance venography (MRV) is a widely used non-invasive imaging technique to diagnose cerebral dural venous sinus thrombosis (CVST) and intracranial venous abnormalities. Non-visualization of a dural venous sinus is diagnostic of CVST on MRV. However, there are numerous common variances and technical aberrations on MRV that mimic filling defects and might be confused with CVST, making diagnosis difficult. These include aberrant veins and missing, hypoplastic or asymmetric venous sinuses. In addition, reconstruction artifacts might take the form of flow gaps with various lengths. The purpose of this study is to examine the occurrences, geographic distribution, and imaging properties of these variations and artifacts on MRV. Materials and Methods: After obtaining clearance from the Institution’s Ethics Committee, magnetic resonance imaging (MRI) brain of 60 patients were taken for the study. MRI was performed using 1.5 Tesla Intera PHILIPS. A cross-sectional study was conducted to study the normal anatomy of the intracranial venous system and its normal variations. Results: This study showed that hypoplasia of the left transverse sinus was found to be more predominant (60%) among other sinuses of the brain followed by right transverse sinuses (31%); the left sigmoid sinus was hypoplastic in 35%, the right sigmoid sinus hypoplastic in 18% and superior sagittal sinuses in 5%. Conclusion: It is essential to know the anatomical variations of the dural venous system for the discrimination between pathological processes such as thrombosis and physiologic conditions. Furthermore, the association of these variations with each other must be kept in mind for explanation of the presence of multiple variations in the same individuals.

PS-BPR01-4: A CASE OF ALDOSTERONE-PRODUCING ADENOMA DIAGNOSED BY ADRENAL ARTERIOGRAPHY AND BRANCH SELECTIVE ADRENAL VENOUS SAMPLING

Case report: A 43-year-old man had been followed up for hypertension and hypokalemia, but self-interrupted his hospital visits for the last four years. Based on the serum potassium level of 2.7 mEq/L, plasma aldosterone concentration of 202 pg/mL, and plasma renin activity of 1.1 ng/mL/hr, he was suspected of primary aldosteronism and was admitted to the previous hospital. A saline suppression test and a rapid ACTH stimulation test were both positive, and 24-hr urinary aldosterone level was 19 μg/day under sodium loading of 170 mEq/day, leading to the diagnosis of primary aldosteronism. Since contrast-enhanced CT showed an 8 mm-sized adenoma in the lower portion of the left adrenal gland, an aldosterone-producing adenoma was suspected and a non-segmental adrenal venous sampling (AVS) was performed. However, it yielded apparent bilateral aldosterone suppression in the main trunk of the bilateral adrenal veins, where the aldosterone-to-cortisol (A/C) ratio was lower than that in the inferior vena cava even after ACTH loading. The patient was referred to our hospital for a segmental AVS. Arteriography was first performed from the left inferior adrenal artery to confirm the outflow vessels from the adenoma. Strikingly, there was an aberrant vein which drained into the proximal segment of the left adrenal vein. CT imaging at the time of arteriography revealed, in addition to the known adenoma, a 6 mm-sized micronodule in the lateral region of the left adrenal gland, and the aberrant vein appeared to be the main outflow vessel from the micronodule. A segmental AVS was performed, and blood samples were taken from the bilateral adrenal veins including the aberrant vein and the main trunk of the left adrenal vein proximal to its confluence. The A/C ratio was highest in the aberrant vein, followed by in the main trunk of the proximal left adrenal vein, while it was markedly suppressed in other branches and the main trunk of the left adrenal vein distal to the confluence of the aberrant vein. Based on these results, the presence of aldosterone-producing adenoma in the left adrenal gland was suggested. Laparoscopic partial left adrenalectomy was performed without postoperative complications. Hypertension and hypokalemia were fully resolved afterwards. Conclusions: Adrenal arteriography and segmental AVS should be considered in such cases that apparent bilateral aldosterone suppression is noted in conventional AVS in patients with clinically suspected aldosterone-producing adenoma.

Robotic-Assisted Resection of Pelvic Arteriovenous Malformation Utilising Intraoperative Video Angiography with Indocyanine Green

<h3>Study Objective</h3> Surgical resection of pelvic arteriovenous malformations (AVM) can be challenging, and pre-operative imaging may not clearly identify feeding vessels. We describe a case of pelvic AVM in a young woman and demonstrate the robotic surgical resection utilising video angiography with indocyanine green (ICG). <h3>Design</h3> Case report and surgical video. <h3>Setting</h3> Four-arm robotic assisted laparoscopy. <h3>Patients or Participants</h3> A nullipara with pelvic AVM. <h3>Interventions</h3> Robotic-assisted resection of pelvic AVM guided by intraoperative ICG angiography. <h3>Measurements and Main Results</h3> A 22-year-old woman presented with progressively worsening left pelvic pain. Pelvic ultrasound demonstrated a 7cm left adnexal vascular mass and magnetic resonance angiogram confirmed a left pelvic AVM. The patient was counselled regarding the risks of embolization versus surgery and decided on the latter. Using a robotic surgical system, the left pelvic side wall was opened at the pelvic brim and careful dissection of the retroperitoneal alveolar tissue was performed. 3mL of a 2.5mg/mL solution of ICG was injected peripherally, followed by 10mL bolus of normal saline. Using the near infra-red imaging of the robotic system, the ICG demonstrates small efferent vessels arising from the left ovarian vessels and a large vein communicating with the left external iliac vein. Vascular clips were applied to the aberrant vein prior to ligation. The AVM was carefully dissected away from the surrounding tissues, with use of bipolar diathermy for coagulation of smaller vessels. A further dose of ICG was injected after resection to ensure haemostasis and to confirm the perfusion of remaining organs. Mesosalpinx and pelvic side wall peritoneum were closed with 2-0 barbed suture. The patient was discharged the following day with complete resolution of left pelvic pain and normal pelvic ultrasound scan 6 weeks later. <h3>Conclusion</h3> Robotic surgical resection of pelvic AVM is enhanced with guidance from intraoperative ICG video angiography to ensure haemostasis and complete excision.

Porto-Sinusoidal Vascular Disease and Focal Nodular Hyperplasia-like Nodules in a Patient with Neurofibromatosis and Non-Cirrhotic Portal Hypertension

Abstract Introduction/Objective Non-cirrhotic portal hypertension (NCPH) is uncommon. The underlying pathophysiology appears to lie at the level of intrahepatic portal veins and sinusoids, hence the term “porto-sinusoidal vascular disease” (PSVD). We report a rare case of PSVD with focal nodular hyperplasia (FNH)-like nodules in a patient with neurofibromatosis type 2 (NF2). Methods/Case Report A 57-year-old male with NF2 and type 2 diabetes, presented with a large variceal bleed requiring blood transfusion and subsequent transjugular intrahepatic portosystemic shunt (TIPS). Imaging showed a nodular liver, presumed to be cirrhosis due to non-alcoholic liver disease. Liver biopsy was not done. Thereafter, he had several episodes of hepatic encephalopathy and TIPS was downsized to prevent recurrences. The patient required liver transplantation for intractable portal hypertension and severe hepatic encephalopathy; his liver synthetic function was near normal and MELD was 11. Portal vein was patent. The explanted liver was micronodular, soft and weighed 946 grams. Unencapsulated nodules, a few mm to 1 cm in size, were present. Microscopically, there was diffuse nodularity in the absence of bridging fibrosis. Thin, incomplete curvilinear fibrous septa were present. There were aberrant veins, hypervascular portal tracts, herniated portal veins and rare occluded portal veins. Trichrome and reticulin stains confirmed architectural abnormalities including nodularity, lack of bridging fibrosis and approximation of portal tracts. Immunohistochemistry for glutamine synthetase accentuated architectural distortion and revealed nodules with FNH-like geographic areas of staining. Results (if a Case Study enter NA) NA Conclusion This is a rare case of NCPH due to PSVD in a patient with NF2. Microscopy suggested incomplete septal cirrhosis (ISC), a pattern associated with both PSVD and regression of fibrosis in a cirrhotic liver. Isolated portal hypertension without loss of synthetic function favors primary PSVD over regression of fibrosis. FNH-like nodules are consistent with regenerative changes caused by localized abnormalities of blood flow.

Unusual Gastrointestinal Hemorrhaging Mimicking a Rupture of Solitary Gastric Varices Due to a Gastric Gastrointestinal Stromal Tumor with Exogenous Growth

Gastric gastrointestinal stromal tumors can lead to upper gastrointestinal hemorrhaging, which is usually caused by dimpling or ulceration on the tumor surface. While rare, pedunculated gastric gastrointestinal stromal tumors outside the stomach can present as a huge mass with delayed complaints. We herein report an unusual hemorrhaging mimicking a rupture of solitary gastric varices due to a pedunculated gastric gastrointestinal stromal tumor. In this case, contrast-enhanced computed tomography (CECT) was essential for tumor detection. An endoscopic investigation revealed dilated, aberrant veins and arteries in the submucosa of this tumor, recognized as solitary gastric varices.

Ulnar Neuropathy at the Elbow: From Ultrasound Scanning to Treatment.

Ulnar neuropathy at the elbow (UNE) is commonly encountered in clinical practice. It results from either static or dynamic compression of the ulnar nerve. While the retroepicondylar groove and its surrounding structures are quite superficial, the use of ultrasound (US) imaging is associated with the following advantages: (1) an excellent spatial resolution allows a detailed morphological assessment of the ulnar nerve and adjacent structures, (2) dynamic imaging represents the gold standard for assessing the ulnar nerve stability in the retroepicondylar groove during flexion/extension, and (3) US guidance bears the capability of increasing the accuracy and safety of injections. This review aims to illustrate the ulnar nerve's detailed anatomy at the elbow using cadaveric images to understand better both static and dynamic imaging of the ulnar nerve around the elbow. Pathologies covering ulnar nerve instability, idiopathic cubital tunnel syndrome, space-occupying lesions (e.g., ganglion, heterotopic ossification, aberrant veins, and anconeus epitrochlearis muscle) are presented. Additionally, the authors also exemplify the scientific evidence from the literature supporting the proposition that US guidance is beneficial in injection therapy of UNE. The non-surgical management description covers activity modifications, splinting, neuromobilization/gliding exercise, and physical agents. In the operative treatment description, an emphasis is put on two commonly used approaches—in situ decompression and anterior transpositions.

Robotic right upper lobectomy: Twelve steps.

Robotic right upper lobectomy: Twelve steps.

Scimitar syndrome with bicuspid aortic valve. A case report of cross-sectional non- invasive imaging allowing a complete anatomical and functional assessment

Scimitar syndrome is a variant of partial anomalous pulmonary venous return with an aberrant vein, the Scimitar vein, draining the right lung to the inferior vena cava instead of the left atrium, resulting in a left-to-right shunt. The classic frontal radiographic finding, designated as “the scimitar sign”, is of a scimitar (a Turkish sword) shaped density along the right cardiac border. The diagnosis can be made by echocardiography, and cardiac catheterisation remains the gold standard to assess the left-to-right shunt. However, the place of multimodal cardiac imaging by computed tomography and magnetic resonance imaging is increasing. We report the case of a 26 year-old man presenting with chest pain during a brief panic attack, in whom scimitar syndrome was associated with a bicuspid aortic valve, a clinical association rarely reported in the literature. CT and MRI cardiac imaging was as accurate as echocardiography and hemodynamics, particularly for shunt quantification.

An accessory aberrant testicular artery branching from the inferior suprarenal artery: embryologic and genetic consideration

During routine educational dissection of a cadaver (61-year-old, male, white descent), multiple aberrant right testicular artery and veins were discovered. Detailed analysis of the abdominal vascular pattern showed that the accessory aberrant testicular artery branched off the right inferior suprarenal artery. It crossed the anterior pararenal space, gave several branches to the soft tissues of the posterior pararenal space, and descended towards the deep inguinal ring. The accessory aberrant testicular vein accompanied the aberrant artery and was drained by the right inferior suprarenal vein into the renal one. Two other testicular veins followed a typical course and opened into the inferior vena cava. The anatomical variability reported in this paper requires attention of the related specialists, as it may significantly complicate surgical and minimally invasive procedures on the organs of the retroperitoneal space and inguinal region. Thorough preoperative investigation regarding the variability of the vascular pattern should be conducted in patients considered for intervention on the adrenal gland, kidney, or testis.

Laparoscopic nephrectomy in crossed fused kidney ectopia with severe hydronephrosis in 2 year-old child

Abstract Introduction There are no specific recommendations for the surgical management of crossed fused renal ectopia (CFRE), but a laparoscopy has been proposed in a limited number of children. We report the case of a complex CFRE with severe hydronephrosis in a child treated by laparoscopic nephrectomy. Case report A 2-year-old boy was admitted for hip and abdominal pain. A CFRE was diagnosed. Laparoscopic nephrectomy was suggested. The right crossed ectopic kidney appeared fused at its upper pole to the lower pole of the left kidney. The left kidney was in total rotation postero superiorly The left ureter crossing over the ectopic dilated right pelvis deliniated the fusion line between both kidneys. The right ureter was dissected distally. The right artery and vein were dissected and the right kidney pelvis opened making the separation of the kidneys easier and so reducing the danger of damaging the lower pole of the left kidney. During the dissection an aberrant vein arising from the left iliac vein and draining from both kidneys was identified. The right vein was dissected preserving the left vein, and the nephrectomy completed. Conclusions Laparoscopy is a recommended option to treat CFRE, offering all advantages of this approach.

CT and MRI manifestations of mediastinal cavernous hemangioma and a review of the literature

BackgroundThe cavernous hemangioma of mediastinum (CHM) is a rare benign lesion caused by congenital vascular dysplasia. However, its incidence is extremely low, and patients often lack relevant clinical symptoms. So we analyzed retrospectively some cases to investigate the imaging features of cavernous hemangioma of mediastinum (CHM) and improve the diagnostic accuracy.MethodsThe CT/MRI imaging features and clinical information of 19 patients with CHM were analyzed retrospectively.ResultsThe lesions of 18 CHM patients were single. Twelve cases in the anterior mediastinum and 8 in the posterior mediastinum. The diameter of CHM ranges from 2.0 to 7.0 cm. Thirteen cases were oval-shaped or round, 4 cases were lobulated, and 2 cases were irregular. Phleboliths or nodular calcification were identified in four cases. High signal of T2WI lipid suppression in two cases and blood vessel shadows were observed in two cases. After contrast-enhanced scan, the nodular enhancement of arteries were identified in 14 cases and contrast agent was further filled of the venous phase, where “fast in and slow out” feature was performed. One case showed inhomogeneous enhancement, one case performed “fast in and slow out” feature of multiphase-enhanced MRI. Besides, aberrant veins can be seen in or around the lesion among five cases.ConclusionsCHM is more frequently located at the anterior mediastinum than at the posterior mediastinum. The performance of phleboliths, high signal on T2WI fat suppression and DWI, the nodular enhancement of the artery, venous and delayed phase filling, enhanced “fast in and slow out,” and aberrant veins in the lesion are helpful for the diagnosis and differential diagnosis. Multiple period contrast-enhanced CT and MRI scan is helpful for the diagnosis of CHM.

Right Hepatectomy In Patient with Absent Portal Vein Bifurcation

Introduction: Understanding of hepatic vascular anatomy and its variations are crucial for performing liver resection and transplantation. Here we present a case of successful right hepatectomy in the patient with absent portal vein bifurcation. Case presentation: 34 years old female presented for elective right hepatectomy. Preoperative CT scan demonstrated absence of portal vein bifurcation. Right hepatectomy was safely achieved by parenchymal division under ultrasound guidance with preservation of intrahepatic portal branches to left hemiliver and without postoperative complications. Discussion: Normal portal vein anatomy is observed in 65-86% of all cases [1, 2]. Of all portal vein anatomy variations, absence of the portal vein bifurcation is rarest among reported with incidence as low as 0.03% [1]. Conclusion: Preoperative recognition of aberrant portal vein anatomy is important for prevention of life-threatening complications after elective hepatectomy and transplantation.

Venous identity requires BMP signalling through ALK3

Venous endothelial cells are molecularly and functionally distinct from their arterial counterparts. Although veins are often considered the default endothelial state, genetic manipulations can modulate both acquisition and loss of venous fate, suggesting that venous identity is the result of active transcriptional regulation. However, little is known about this process. Here we show that BMP signalling controls venous identity via the ALK3/BMPR1A receptor and SMAD1/SMAD5. Perturbations to TGF-β and BMP signalling in mice and zebrafish result in aberrant vein formation and loss of expression of the venous-specific gene Ephb4, with no effect on arterial identity. Analysis of a venous endothelium-specific enhancer for Ephb4 shows enriched binding of SMAD1/5 and a requirement for SMAD binding motifs. Further, our results demonstrate that BMP/SMAD-mediated Ephb4 expression requires the venous-enriched BMP type I receptor ALK3/BMPR1A. Together, our analysis demonstrates a requirement for BMP signalling in the establishment of Ephb4 expression and the venous vasculature.

Mini-Laparoscopic Management of Ureteropelvic Junction Obstruction in Adults and Children: A High-Volume Case Series.

Purpose: We retrospectively reviewed the perioperative outcomes of mini-laparoscopic procedure in the treatment of ureteropelvic junction obstruction (UPJO) in children and adults. Methods: From August 2009 to March 2017, 229 patients referred to our center to repair UPJO by mini-laparoscopic operation. In 203 cases, dismembered pyeloplasty was accomplished, while in other 26 cases, crossing aberrant vein division and crossing artery upward transposition were performed. A follow-up renal ultrasound was done on the cases 3 and 6 months after surgery. During the follow up period, if the patients had persistent hydronephrosis or sustained clinical complaints, diethylenetriamine pentaacetic acid (DPTA) scan was done to rule out the stenosis. Results: Among 229 patients, 140 patients were younger than 18 years (Range: 2 months-18 years old, mean: 3.01 ± 1.2 year) and others were scheduled as Adult (Range: 18-57 years old, mean: 35.12 ± 7.54 year). Total clinical and radiological success rates were 99.5% (228/229) and 86.5% (198/229) respectively. Mean operative times were 127.4 ± 20.3 minutes in dismembered pyeloplasty and 110.6 ± 12.7 minutes in crossing vessel transposition surgery. Mean of hemoglobin decreasing in children and adults was 0.3 ± 0.1 mg/dL; P = .26, and 0.5 ± 1.5 mg/dL; P = .13, respectively. Length of hospital stay was 3.41 ± 0.6 days in dismembered pyeloplasty and 2.1 ± 0.6 days in patients with crossing vessel transposition surgery. We did not suture the skin incision in the entrance site of 3 mm ports and the surgical scar was hardly visible after 6 months. Conclusions: The results of our study suggest that mini-laparoscopic pyeloplasty in adults and particularly in children is feasible, and it seems to be safe and effective in the treatment of UPJO. Furthermore, the patients tolerated the surgery well and they appreciated its outstanding cosmetic outcomes.

Rare localized extralobar sequestration with congenital cystic adenomatoid malformation: a case report

Extralobar sequestrations constitute a rare form of congenital pulmonary airway malformations that are difficult to diagnose. Here, we report a rare case of a localized extralobar sequestration in the right superior portion of the mediastinum accompanied by congenital cystic adenomatoid malformation.A 19-year-old man presented with a right upper mediastinal mass that was detected using chest radiography, had a history of left spontaneous pneumothorax, and had undergone a bullectomy 4 years previously.The initial diagnosis included a mature teratoma and a bronchogenic cyst in the mediastinum; however, the presence of a cystic mass in the right upper lobe of the lung prompted further examination. A preoperative diagnosis of extralobular sequestration was finally determined using contrast-enhanced computed tomography. The aberrant artery was connected to the brachiocephalic artery, and its drainage vein was connected to the right pulmonary artery, uniquely behind the pericardium. Despite the unique location, right mediastinal extralobular sequestration with a congenital cystic adenomatoid malformation in the right upper lobe was confirmed pathologically. Examination of contrast-enhanced chest computed tomography (CT) and three-dimensional computed tomography images enabled a correct diagnosis. It is very important for surgeons to correctly diagnose and identify an aberrant artery and drainage vein to prevent uncontrolled hemorrhage.

Successful subcarinal dissection using a laparoscopic transhiatal approach for esophageal cancer with an anomalous pulmonary vein

We herein reported a case of a dangerous anomaly of a pulmonary vein in subcarinal dissection. A 74-year-old man underwent esophagectomy with systematic nodal dissection using a laparoscopic transhiatal approach. Under pneumomediastinum pressure, dissection of the posterior plane of the pericardium was extended, and a vessel that independently drained into the left atrium was identified. Although this anomalous vein penetrated the subcarinal lymph nodes and crossed behind the right main bronchi, subcarinal dissection was successfully performed without its injury. Our surgical procedure enabled the intraoperative identification of this rare abnormality, an aberrant segmental vein in the right upper lobe, and safe en bloc subcarinal dissection.

Anatomical variations of liver blood supply in patients with pancreaticobiliary maljunction.

Pancreaticobiliary maljunction (PBM) arises from a developmental abnormality during the embryonic period; thus, these patients may have anatomical variations of the liver blood supply, including aberrant vessels and unusual locations. We reviewed retrospectively the vascular anatomy of 52 patients with PBM, who were seen between 1998 and 2013. There were 11 male patients and 41 female patients. Sixteen patients had bile duct type (C-P type) PBM, 35 had pancreatic duct type (P-C type) PBM, and one had complex type PBM. Thirty-three patients had biliary dilatation. Nine (17.3%) patients had an aberrant (replaced or accessory) hepatic artery and 2 (3.8%) had an aberrant portal vein. Among 39 patients evaluated by contrast-enhanced computed tomography, 8 (20.5%) had their right hepatic artery positioned on the ventral side of the common bile duct. The presence of aberrant hepatic vessels was not related to the type of confluence, biliary dilatation, type of Todani classification, or associated biliary malignancies. The incidence of the right hepatic artery being located on the ventral side of the common bile duct may be higher in patients with PBM than the naturally occurring incidence of about 10% in the general Japanese population.

A rare case: Vena cava inferior agenesis with aberrant hepatic vein in an asymptomatic patient

SummaryInferior vena cava agenesis is a rare anomaly in an asymptomatic patient. A 58-year-old female patient with complete absence of the inferior vena cava and aberrant right hepatic vein is presented with radiologic findings. To our best knowledge, such a combination of venous anomalies has not previously been documented in the literature and this is the first case in the literature due to the coincidence of total inferior vena cava agenesis with aberrant hepatic vein.