Aberrant Course Research Articles

Aberrant Course of the Intratemporal Facial Nerve in Children with Congenital Hearing Loss.

Congenital facial nerve (FN) malformations are uncommon. Our aim is to determine the clinical and radiological features of FN malformations along with the associated cochlear and vestibular malformations. We conducted a retrospective study including children with a profound sensorineural hearing loss who were candidates for cochlear implantation. We evaluated the presence of FN malformations through temporal bone computed tomography scan and magnetic resonance imaging. We recorded an aberrant FN course in five out of 165 patients in a total of 9 ears. They consisted of a bifurcation of the mastoid segment, an anterior or posterior displacement of the labyrinthine segment and a hypoplasia of the geniculate ganglion. Associated inner ear malformations included vestibular aqueduct dilation, cochlear hypoplasia and total labyrinthine aplasia. We noted a bilateral agenesis of the cochlear nerve in three patients. Facial nerve malformations should be suspected in patients presenting a congenital hearing loss especially in association with other temporal bone malformations. Their pre-operative discovery is helpful in planning the surgical procedure.

EBF1 Limits the Numbers of Cochlear Hair and Supporting Cells and Forms the Scala Tympani and Spiral Limbus during Inner Ear Development.

Early B-cell factor 1 (EBF1) is a basic helix-loop-helix transcription factor essential for the differentiation of various tissues. Our single-cell RNA sequencing data suggest that Ebf1 is expressed in the sensory epithelium of the mouse inner ear. Here, we found that the murine Ebf1 gene and its protein are expressed in the prosensory domain of the inner ear, medial region of the cochlear duct floor, otic mesenchyme, and cochleovestibular ganglion. Ebf1 deletion in mice results in incomplete formation of the spiral limbus and scala tympani, increased number of cells in the organ of Corti and Kölliker's organ, and aberrant course of the spiral ganglion axons. Ebf1 deletion in the mouse cochlear epithelia caused the proliferation of SOX2-positive cochlear cells at E13.5, indicating that EBF1 suppresses the proliferation of the prosensory domain and cells of Kölliker's organ to facilitate the development of appropriate numbers of hair and supporting cells. Furthermore, mice with deletion of cochlear epithelium-specific Ebf1 showed poor postnatal hearing function. Our results suggest that Ebf1 is essential for normal auditory function in mammals.

A rare variation of omohyoid and its embryological correlations- A case report

The omohyoid muscle is important in radical neck dissection, as it is a landmark for this operation. Because it divides the anterior and posterior cervical triangles into smaller triangles and its particular relationship to the large cervical vessels, the presence of an anatomical variation of the omohyoid muscle is important. Either belly may be absent or double; and the inferior belly may be attached directly to the clavicle and the superior is sometimes fused with sternohyoid. Variations in the omohyoid muscle might be linked to differences in embryological development, which could influence the muscle’s final form and function. Anderson (Anderson, 1881) theorized that the superior belly of the omohyoid muscle is a true infrahyoid muscle, whereas the inferior belly most likely shares a common embryology with the subclavius muscle. As omohyoid mucle is used to achieve the reconstruction of the laryngeal muscles and bowed vocal folds, the knowledge of the possible anomalies of the omohyoid muscle is important.In the present study, we have found a case with two inferior bellies & aberrant course of superior belly of omohyoid on right side of 40 years female cadaver embalmed with formalin during routine dissection at Department of Anatomy AIIMS Gorakhpur.Omohyoid muscle is closely related to large vessels of neck & brachial plexus, so variations in this muscle are clinically important.

Trajet aberrant d'une artère carotide interne anévrismale révélé par une dysphonie et une toux chronique : à propos d'un cas

Aberrant internal carotid artery is a rare birth defect. It occurs when the artery takes an abnormal course, the discovery of which is fortuitous but in the presence of dysphonia or chronic cough; this anomaly remains a diagnosis of exclusion. Cervicothoracic CT scan with injection of contrast product confirms the diagnosis. We report the case of a 64-year-old patient who presented with an aberrant course of an aneurysmal internal carotid artery revealed by dysphonia and chronic cough.

A rare case of Zinner syndrome with unilateral testicular agenesis and aberrant course of left common iliac artery

BackgroundZinner syndrome is a rare Wolffian duct anomaly presenting as a triad of unilateral renal agenesis, ipsilateral seminal vesicle cyst and ipsilateral ejaculatory duct obstruction. The condition is due to a developmental anomaly leading to malformation of the mesonephric duct. Magnetic resonance imaging is considered to be the gold standard for the diagnosis of Zinner syndrome. Due to the limited number of cases reported in the literature, the associations of Zinner syndrome are not well established. Case presentationIn this case report, we are presenting a case of a 38-year-old male with dysuria, intermittent pain in the scrotum and lower abdomen and painful ejaculation for the past six months. Imaging findings led to the diagnosis of Zinner syndrome, with unilateral testicular agenesis and an aberrant course of the left common iliac artery.ConclusionsApart from the known associations of Zinner syndrome, testicular agenesis in a patient with Zinner syndrome has not been reported in the literature and is an important addition to the limited literature available.

A case of microtia with bilateral conductive hearing loss with unusual findings

Microtia refers to a group of congenital auricle malformations that range in severity from minor partial structural abnormalities to full auricle absence (anotia). A 17-year-old female, who was also a known case of left ear microtia presented to OPD with chief complaint of reduced hearing from both ears since 4 years. Examination showed Left ear microtia with narrow canal with retracted tympanic membrane while right ear had a normal pinna and canal with grade 3 retraction present in the tympanic membrane. In pure-tone audiometry (PTA) a conductive hearing loss of 57 dB in the right ear and 52 dB in the left ear, and impedance audiometry showed a curve of "Ad" and bilaterally ipsilateral reflexes were found to be absent. During exploratory tympanotomy, intraoperatively facial nerve was found to be low lying over the promontory, showing an abnormal course. A piece of cartilage was placed over the posterosuperior region. Temporalis fascia graft was placed over it. Any such case that has a congenital anomaly like microtia should not be planned for any kind of surgical intervention as they might have abnormal findings like an aberrant course of the facial nerve, and any kind of intraoperative manipulation with the ear might further increase the complications of the patient like facial nerve paresis. These patients should be properly assessed and counselled to directly go for a hearing aid rather than undergo any kind of surgical risk or complication.

Bilateral Retropharyngeal Internal Carotid Artery ‘Kissing Carotids’: A Case Report

Aberrant course of the internal carotid arteries is a rare congenital malformation, accounts for 5% of cases. Several cases of fatal hemorrhage due to an injury to the aberrant internal carotid artery have been observed in patients undergoing pharyngeal surgery procedures. This was associated with a lack of detection during surgery. We report a case of aberrant commun and internal carotid arteries, manifested by odynophagia. This particular anatomical condition should be suspected in patients with pharyngeal mass before any invasive procedure.

Absent stapedius muscle, tendon and pyramid – An anomaly revisited

Congenital absence of stapedius is an exquisite entity, with an incidence of 0.5% and very few cases reported in live patients. We herein report a case of a middle-aged female presented with bilateral ear discharge and decreased hearing for more than a year, which, on examination, showed a large central perforation bilaterally, and an audiogram preoperatively revealed an average hearing loss of 45 dB and air-bone gap of 35dB bilaterally. Right endoscopic type I tympanoplasty was done, which unveiled absent stapedius muscle, tendon, and pyramid on the right side. The absent stapedius should circumspect the operating surgeon to look for other associated anomalies such as tympanosclerosis, fixed footplate and aberrant course of the facial nerve.

Surgical reporting for laparoscopic cholecystectomy based on phase annotation by a convolutional neural network (CNN) and the phenomenon of phase flickering: a proof of concept

PurposeSurgical documentation is an important yet time-consuming necessity in clinical routine. Beside its core function to transmit information about a surgery to other medical professionals, the surgical report has gained even more significance in terms of information extraction for scientific, administrative and judicial application. A possible basis for computer aided reporting is phase detection by convolutional neural networks (CNN). In this article we propose a workflow to generate operative notes based on the output of the TeCNO CNN.MethodsVideo recordings of 15 cholecystectomies were used for inference. The annotation of TeCNO was compared to that of an expert surgeon (HE) and the algorithm based annotation of a scientist (HA). The CNN output then was used to identify aberrance from standard course as basis for the final report. Moreover, we assessed the phenomenon of ‘phase flickering’ as clusters of incorrectly labeled frames and evaluated its usability.ResultsThe accordance of the HE and CNN was 79.7% and that of HA and CNN 87.0%. ‘Phase flickering’ indicated an aberrant course with AUCs of 0.91 and 0.89 in ROC analysis regarding number and extend of concerned frames. Finally, we created operative notes based on a standard text, deviation alerts, and manual completion by the surgeon.ConclusionComputer-aided documentation is a noteworthy use case for phase recognition in standardized surgery. The analysis of phase flickering in a CNN’s annotation has the potential of retrieving more information about the course of a particular procedure to complement an automated report.

PO-717-04 ABERRANT COURSE OF RIGHT PHRENIC NERVE ALONG RIGHT INFERIOR PULMONARY VEIN ANTRUM: IMPLICATIONS FOR ATRIAL FIBRILLATION ABLATION

The right phrenic nerve (RPN) usually descends anterior to the mid to distal right superior pulmonary vein (RSPV), and can be injured during electrical isolation of RSPV. To assess proximity of the nerve to RSPV antrum, high output bipolar pacing to check for RPN stimulation is typically utilized. Aberrant course of RPN, however, can increase risk of injury, requiring careful evaluation.

Anomalous branching of the aortic arch in a dog.

Anomalies in the subclavian and common carotid arteries can be of interest in cases of cranial mediastinal surgeries, as well as to diagnose the cause of oesophageal constrictions leading to clinical signs of dysphagia (dysphagia lusoria). The development and regression of the aortic arches are of key importance in understanding the origin of these type of vascular anomalies. This report describes the congenital anomalous aortic origin of the common carotid and the subclavian arteries in a 14-year-old dog and the plausible developmental pattern failure. Academic dissection revealed a common bicarotid trunk and bisubclavian trunk arising from the most cranial aspect of the aortic arch. Despite the abnormal origin, these vessels displayed a predominantly standard anatomical course. All the anticipated branches were identified and described. Cardiac abnormalities were also noted including right atrial dilation, coronary sinus enlargement, right and left valvular endocardiosis, a patent foramen ovale and marked concentric left ventricular hypertrophy with compensatoryleft atrial dilation. Additionally, the right recurrent laryngeal nerve demonstrated an aberrant course consistent with a 'non-recurrent laryngeal nerve' (non-RLN). Awareness of the anatomical variations of the aortic arch is important for surgical interventions of the cranial mediastinum as well as radiological interpretation. Although infrequent, the variants similar to the one described here have been reported in different species.

A distal anterior cerebral artery tripod branching to a bihemispheric pericallosal artery.

The anterior cerebral artery (ACA) branching pattern may be particularly complex and variant including bifurcations, trifurcations or quadrifurcations, thus leading to typical or atypical vessels. The bihemispheric ACA (BhACA) variant crosses the midline to supply the contralateral hemisphere. The current case highlights a left-sided ACA trifurcation into a bihemispheric pericallosal artery, and two ipsilateral atypical arteries: a callosomarginal artery of short course and a left-sided pericallosal artery of an aberrant course into the cingulate sulcus. The depicted trifurcation was characterized as a distal ACA tripod. The bihemispheric branch supplied the contralateral pericallosal area and coursed into the cingulate sulcus. Deviations from the typical ACA pattern, especially when they coexist are quite rare and may complicate neurosurgical approaches or raise diagnostic difficulties. The BhACA may be implicated in aneurysm formation and bilateral ischemia after its occlusion. Knowledge of such variants, as well as awareness of the complications they may be related to, is of great significance.

Abnormal Course of Internal Carotid Artery Encountered During Neck Dissection.

Internal carotid artery (ICA) is one of the most important structures to be preserved during neck dissection. Many anomalous courses of ICA have been explained and must be kept in mind while proceeding with surgery. The variation mentioned in this article has not been described yet. This is very important for head and neck surgeons, especially while performing neck dissection or external carotid artery ligation. A 51-year-old male with no known comorbidities underwent surgery for carcinoma right buccal mucosa. As noted in preoperative imaging, intraoperatively he had a abnormal course of ICA especially with abnormal relation to the internal jugular vein (IJV). It is imperative to study the anatomy of ICA preoperatively to prevent intraoperative surprises. A careful dissection of the level II cervical region keeping in mind regarding an aberrant course of ICA especially with relation to IJV can prevent catastrophic consequences.

Another Piece of the Puzzle of Anomalous Connectivity in Joubert's Syndrome.

We report on the conventional and diffusion tensor imaging (DTI) findings of a 2-year-old child with clinical presentation of Joubert's Syndrome (JS) and brainstem structural abnormalities as depicted by neuroimaging.Conventional magnetic resonance imaging (MRI) showed a "molar tooth" configuration of the brainstem. A band-like formation coursing in an apparent axial plane anterior to the interpeduncular fossa was noted and appeared to partially cover the interpeduncular fossa.DTI maps and three-dimensional (3D) tractography demonstrated a prominent red-encoded white matter bundle anterior to the midbrain. Probable aberrant course of the bilateral corticospinal tracts (CST) was also depicted. Absence of the decussation of the superior cerebellar peduncles and elongated thickened, horizontal superior cerebellar peduncle (SCP) reflecting the molar tooth sign were also shown.Our report and the review of the published cases suggest that DTI and tractography may be very helpful to differentiate between interpeduncular heterotopias and similarly located white matter bundles corroborating the underlying etiology of axonal guidance disorders in the complex group of ciliopathies including JS. Our case represents an important additional puzzle piece to explore the variability of these ciliopathies.

Anatomic Variations of the Hepatic Artery in 5625 Patients.

To analyze the origin and anatomic course of the hepatic arteries by using digital subtraction angiography (DSA) and multidetector CT in a large study sample. This retrospective study included 5625 patients who underwent liver CT and chemoembolization between January 2005 and December 2018 (mean age, 60 years ± 11 [range, 11-99 years]; 4464 males). The CT and DSA images were reviewed to evaluate the visceral arterial anatomy for variations in the celiac axis and hepatic arteries. Aberrant right hepatic arteries (aRHAs) and aberrant left hepatic arteries (aLHAs) were defined on the basis of their origin and anatomic course. Statistical analyses were performed to evaluate the association between aRHAs and aLHAs and the association between variations in the hepatic artery and celiac axis. Right hepatic arteries were categorized as being aRHAs (if originating from the proximal to middle common hepatic artery, gastroduodenal artery, superior mesenteric artery [SMA], celiac axis, aorta, splenic artery, or left gastric artery [LGA]) or as being aLHAs (if arising from the LGA, celiac axis, aorta, or SMA). The prevalence of aRHAs (15.63%; 879 of 5625) and the prevalence aLHAs (16.32%; 918 of 5625) were similar. Patients with an aRHA were more likely to have an aLHA than those without an aRHA (29.01% vs 13.97%; P < .001), and patients with an aLHA were more likely to have an aRHA than those without an aLHA (27.78% vs 13.26%; P < .001). There was no association between the hepatic arterial variations and celiac axis variations. A hypothetical anatomic model summarizing the observed variations was created. A comprehensive list of hepatic arterial variations and a three-dimensional hypothetical model for the observed variations were described.Keywords: CT, Angiography, Liver, Anatomy, Arteries© RSNA, 2021 Supplemental material is available for this article. See also commentary by Sutphin and Kalva in this issue.

Imaging of Aberrant Internal Carotid Arteries.

Aberrant internal carotid arteries (ICAs) may take abnormal courses in the cervical region. We present the ICA imaging features that suggest the presence of a submucosal mass in the posterior pharyngeal wall caused by such an aberration. Cervical computed tomography angiography images of 2400 patients obtained over 5 years were retrospectively reviewed. The ICA courses at the levels of the hypopharynx and oropharynx were evaluated. Aberrant ICAs running posterior to the pharynx were selected. Vessel location was classified into 3 types: lateral to the vertebral foramen (normal, type I); between the lateral foramen and the uncovertebral joint (type II); and medial to the uncovertebral joint (type III). Medical records were examined, and relationships between symptoms, the ICA variations, and imaging features evaluated. Cervically aberrant ICAs of type II were detected in 45 patients and aberrations of type III in 18. In the latter group, the mean patient age was 66 years, 12 were men and 6 were women. Six (33%) patients (4 men) had clinical symptoms associated with their aberrant ICA courses. In 2 men (11%), both ICAs took aberrant courses. Otherwise, the right ICA was affected in 9 (50%) cases and the left ICA in 7 (39%). Parapharyngeal ICA aberrations were both common and asymptomatic. Such ICAs may be damaged during pharyngeal surgery or intubation. The ICA location should be investigated before any interventional procedure.

A rare case of aberrant facial nerve course in the mastoid segment

Facial nerve is the main motor supply to the part of facial skeleton system responsible for expressions. The reported rate of iatrogenic injury to the facial nerve in primary mastoid surgeries was 0.6% to 3.7%. Temporal bone is one of the most complex anatomical parts of human body. A variety of facial nerve courses has been described in literature. Normally, horizontal segment of the facial nerve traverses from geniculate ganglion to second genu which is usually situated medial and inferior to lateral semicircular canal. From here it passes posteriorly and laterally along the medial wall of the middle ear. Mastoid or vertical segment extends from the second genu to stylomastoid foramen deep to tympano-mastoid suture line from where the nerve exits out of the temporal bone. During our endoscopic dissection we encountered a grossly anomalous course of facial nerve in which after turning at second genu, the nerve curves posteriorly and lies in the floor of mastoid cavity and traverse’s its whole length of mastoid and instead of moving out of foramen it travels towards sinus plate and then takes another (3rd) turn to travel anteriorly towards the tip of mastoid from where it finally exits.

Prenatal diagnosis of an aberrant ductus venosus draining into the coronary sinus using two- and three-dimensional echocardiography: a case report

BackgroundDuctus venosus (DV) abnormalities may be associated with intracardiac or extracardiac deformities, chromosomal anomalies, and/or congestive heart failure. Aberrant DV connecting with the coronary sinus (CS) is rare and the prenatal diagnosis presents challenges for most examiners.Case presentation A 35-year-old pregnant woman, gravida 2, para 1, was referred to our center at 27 gestational weeks for a full evaluation of fetal cardiac anomalies. Transverse scans indicated normal cardiac anatomy except for a dilated CS; we then scanned sagittal planes to clarify the reasons for the CS dilatation. High-definition flow imaging (HDFI) together with radiant flow (R-flow) imaging was used to delineate the aberrant DV returning to the CS, enabling the diagnosis. Three-dimensional (3D) technology was also used to obtain color-rendered images showing the spatial relationships of the vessels involved, thus confirming the two-dimensional (2D) diagnosis. Chromosomal analysis revealed a normal karyotype. The neonate appeared healthy and the echocardiogram showed a normal cardiac anatomy except for a dilated CS with the DV closed and imperceptible.ConclusionsThe aberrant course of the DV returning to the CS was clearly demonstrable by traditional 2D echocardiography using HDFI and the R-flow technique. We deem it helpful to trace the inflow of the dilated CS to make the differential diagnosis. The 3D modality might also provide additional spatial information on the associated vessels and thereby assist in prenatal diagnosis.

Otopathologic Abnormalities in CHARGE Syndrome.

To perform an otopathologic analysis of temporal bones (TBs) with CHARGE syndrome. Otopathologic study of human TB specimens. Otopathology laboratories. From the otopathology laboratories at the University of Minnesota and Massachusetts Eye and Ear Infirmary, we selected TBs from donors with CHARGE syndrome. These TBs were serially sectioned at a thickness of 20 µm, and every 10th section was stained with hematoxylin and eosin. We performed otopathologic analyses of the external ear, middle ear (middle ear cleft, mucosal lining, ossicles, mastoid, and facial nerve), and inner ear (cochlea, vestibule, internal auditory canal, and cochlear and vestibular nerves). The gathered data were statistically analyzed. Our study included 12 TBs from 6 donors. We found a high prevalence of abnormalities affecting the ears. The most frequent findings were stapes malformation (100%), aberrant course of the facial nerve (100%) with narrow facial recess (50%), sclerotic and hypodeveloped mastoids (50%), cochlear (100%) and vestibular (83.3%) hypoplasia with aplasia of the semicircular canals, hypoplasia and aplasia of the cochlear (66.6%) and vestibular (91.6%) nerves, and narrowing of the bony canal of the cochlear nerve (66.6%). The number of spiral ganglion and Scarpa's ganglion neurons were decreased in all specimens (versus normative data). In our study, CHARGE syndrome was associated with multiple TB abnormalities that may severely affect audiovestibular function and rehabilitation.

Aberrant origin of right vertebral artery from the arch of aorta.

We present a rare case of an aberrant right vertebral artery originating from the arch of aorta distal to the origin of the left subclavian artery. The incidence of this particular variant of aberrant origin of the right vertebral artery is extremely uncommon with only seventeen cases reported in literature to date. This case was incidentally detected on a staging positron emission tomography-computerized tomography (PET-CT) scan for lung cancer. We review the incidence, embryological mechanism, and clinical importance of this aberrant course of the right vertebral artery.