Congenital Remnant Research Articles

Giant Eustachian Valve Mimicking an Inter-atrial Septal Defect

A 68-year-old woman presented with dyspnoea attributed to acute pulmonary oedema. Due to an audible diastolic murmur, a transthoracic and transoesophageal echocardiogram (TEE) was performed revealing severe rheumatic mitral stenosis and regurgitation. Although initial assessment of the interatrial septum did not reveal any atrial septal defects, upon TEE evaluation using a 112° window, an interatrial defect was seen, with turbulent flow seen through this. However, using multiplanar imaging, a separate elongated Eustachian valve was shown to be mimicking an ‘arm’ of the interatrial septum and giving rise to the illusion of a defect. The Eustachian valve is a congenital remnant which often regresses at birth but can exist as an elongated structure originating from the inferior portion of the right atrium and may appear as an atrial septal defect. By employing multiplanar imaging on TEE, we were able to systematically discern the reason behind the apparent defect and avoid further delays to the patient’s care.

Interesting case report on Chiari network

The Chiari network is a congenital remnant that is typically found in the right atrium and is generally not associated with significant pathophysiological implications. Usually, this is detected on general routine health checkup screening or casual examination. But, occasionally in certain cases, it is possible for this condition to results in diagnostic ambiguity when assessing right atrial disorders. Additionally, it has potential to contribute to the development of thromboembolism by inducing flow blockage. The Chiari network can be accurately diagnosed by echocardiography. This network appear as a highly mobile, highly reflectant echo target at many regions in the right atrium during transthoracic and transesophageal echocardiography. In this study, we provide a case involving an 18 year old male students studying cardiac technology who was diagnosed incidentally during echocardiography practical classes with Chiari network, mild TR and mild PR. The value and precision of echocardiography are becoming more widely recognized and being used more frequently. The potential for finding normal anatomical variations and their potential incorrect interpretation as pathological states coincides with the wider implementation of this imaging method.

A rare congenital abnormality mimicking the appearance of a type A aortic dissection

Type A aortic dissection is a tear in the wall of the ascending aorta and/or arch between the tunica intima and tunica media layers. This is a surgical emergency and requires immediate surgical repair. Aortic CT angiography is essential for classification, assessing extent of the disease and planning the management. An intimal flap on CT is characteristic of the pathology. We present a case where a fibrous cord, presumed to be a congenital remnant, mimicked the appearance of an intimal flap but was not an aortic dissection.

Small bowel segment with Meckel’s diverticulum volvulus related to short mesodiverticular band: a case report

BackgroundMeckel’s diverticulum is a remnant of the omphalomesenteric duct and occurs in only about 2% of people. Mesodiverticular band is the congenital remnant of the vitelline artery and is an even less often occurring phenomenon.Presented caseWe present the case of a 56-year-old Caucasian male who was admitted to the emergency department with a very intense, sudden abdominal pain, without past abdominal surgery history. Contrast enhanced computed tomography showed a possibly ischemic closed loop of the small intestine. Urgent laparotomy was performed, during which bloody content in the peritoneal cavity and torsed loop of the small intestine with Meckel’s diverticulum were found. The bowel loop and Meckel’s diverticulum were ischemic. At the tip of Meckel’s diverticulum there was a broken fibrous band extending to mesentery with pulsating artery. We did segmental resection of small intestine including Meckel’s diverticulum and primary end-to-end anastomosis. The patient had an unremarkable postoperative hospital stay and was discharged home after 5 days.ConclusionIn our case, we describe a patient with the volvulus of a segment of small bowel and Meckel’s diverticulum, which eventually led to small bowel obstruction and ischemia. It was a very rare case that required urgent surgical treatment.

DOZ047.123: Choose typology: oral communication multiple congenital bronchogenic cysts and thyroglossal duct remnant, case report

Abstract Background Thyroglossal duct remnants, most frequently presenting as cysts, are the most common congenital anomalies of the neck. Bronchogenic cysts are rare congenital anomalies of the primitive foregut and are a subtype of foregut supplication cysts. Those cysts are the most common cystic lesions of the mediastinum. They can present in other areas such as the neck. Such cystic lesions are usually seen in pediatric population and symptomology depends on the location, size and degree of compression to the surrounding structures. The synchronous presence of both lesions in one patient is rare. To the best of our knowledge, such presentation has not been reported in the literature. We present a case of multiple lesions in the neck and mediastinum including precricoid, paraglotttic and paratracheal regions. Case Presentation A full-term male newborn presented in day 1 post normal vaginal delivery with progressive stridor and decreased feeding. Urgent airway endoscopy revealed a left supraglottic cyst and proximal non pulsatile tracheal wall compression by another cystic lesion. Aspiration of the supraglottic cyst was done and one month later, he developed stridor again for which he had endoscopic marsupialization of right supraglottic cyst and internal aspiration of paratracheal cyst. MRI showed right paratracheal cystic lesion with evidence of a tubular tract and an infected cyst inferiorly. Decision was made to explore the neck for definite removal of the cysts. Three lesions were identified: a precricoid mass, and paraglottic and paratracheal cysts. Cervical lesions were carefully dissected and excised. For the paratracheal cyst which extended to the mediastinum, incision and drainage was done and part of cyst wall was excised, and a drain inserted into the cyst. Histopathology confirmed the precrcoid lesion to be a thyroglossal duct remnant, the paraglottic cyst in keeping with bronchogenic cyst and the paratracheal cyst wall showed granulation tissue. Discussion The fact that the embryological basis for development of thyroglossal duct cyst and bronchogenic cyst is different makes diagnosis of both lesions is challenging and such presentation is unique. Definite diagnosis depends on accurate history, imaging particularly MRI and histopathology. Surgery is the treatment of choice for symptomatic cases.

Meckel's diverticulum and the eponymous legend.

Johann Friedrich Meckel (1781-1833) was a 19th century anatomist born into an eminent dynasty. He was a professor of anatomy, pathology, and zoology at the University of Halle, in Central Germany. The diverticulum, a congenital remnant of the vitellointestinal duct was named after him. Other eponyms include Meckel's cartilage, Meckel syndrome, and Meckel-Serres law of recapitulation. His concepts in comparative anatomy, embryology, and teratology anticipated Darwin. This review is a short tribute to this legend and his prolific contributions. LEVEL: Historic review, level V.

Incidental Imaging Finding of Persistent Left Superior Vena Cava – A Case Report

Persistent left superior vena cava (PLSVC) is the most common thoracic venous anomaly and is persistent congenital remnant of vena caval system from early cardiac development. In presence of PLSVC, the venous blood of upper half of the body is drained into coronary sinus or right atrium. PLSVC is uncommon but clinically very closely connected venous congenital abnormality that comes across by non-invasive cardiovascular imaging. Here, we report a case of PLSVC in 11-years-old male patient.

The incidental finding of a persistent left superior vena cava: implications for primary care providers-case and review.

Persistent left superior vena cava (PLSVC) is the most common thoracic venous anomaly and is a persistent congenital remnant of the vena caval system from early cardiac development. Patients with congenital anomalous venous return are at increased risk of developing various cardiac arrhythmias, due to derangement of embryologic conductive tissue during the early development of the heart. Previously this discovery was commonly made during the placement of pacemakers or defibrillators for the treatment of the arrhythmias, when the operator encountered difficulty with proper lead deployment. However, in today's world of various easily obtainable imaging modalities, PLSVC is being discovered more and more by primary care providers during routine testing or screening for other ailments. Given the known association between anomalous venous return and the propensity for cardiac arrhythmias, we review the embryology of PLSVC and the mechanisms by which it leads to conduction abnormalities. We also provide the practitioner with recommendations for certain baseline cardiac observations and suggestions for proper surveillance in hopes that better understanding will reduce unnecessary and potentially harmful testing, premature subspecialty referral, and unneeded patient anxiety.

Limb-Threatening Ischemia Secondary to a Congenital Acromioclavicular Remnant

Upper extremity vascular compromise from thoracic outlet syndrome is rare and is usually the result of a "cervical rib," anterior scalene muscle abnormality, or clavicular trauma. We report a case of acute axillary artery thrombosis secondary to a congenital acromioclavicular remnant in a 40-year-old woman.

Chiari Network Endocarditis: Not Just an Innocent Bystander

A Chiari's network, noted in approximately 2% of the general population, is a congenital remnant of the sinus venosum valve present in the right atrium. We report a case of a 67-year-old male who presented with acute infective endocarditis of the tricuspid valve due to coagulase-negative Staphylococci. Despite appropriate antimicrobial therapy for 2 weeks, the patient remained febrile with septic emboli to the pulmonary vasculature. Repeat transthoracic echocardiography (TTE) revealed persistent vegetations adherent to the tricuspid valve, extending onto the Chiari network, necessitating surgical intervention.

Chiari Network Entanglement and Herniation into the Left Atrium by an Atrial Septal Defect Occluder Device

The Chiari network is a fenestrated membrane consisting of threads and strands in the right atrium. First described in 1897 by anatomist Hans Chiari, 1 it is a congenital remnant of embryonic development resulting from incomplete resorption of the right valve of the sinus venosus. Found in 2% to 3% of the population, 2,3 it is generally not of clinical importance. Rarely, however, the network may be associated with serious complications such as thrombus formation, 2 embolus entrapment, 4 arrhythmia, 5 tumor development, 6 and catheter entrapment. 7 We report the entanglement of an Amplatzer septal occluder device catheter in a prominent Chiari network that was herniated into the left atrium. Transesophageal echocardiographic recognition of this before deployment and guidance during disentanglement is described below. (J Am Soc Echocardiogr 1999;12:601-3.)

Treatment and histopathology of a congenital vitreous cyst

Objective This study aimed to evaluate the treatment efficacy of a congenital vitreous cyst and to examine the cyst histopathologically to determine its cellular makeup and possible origin. Study design The study design was a case report, including a clinicopathologic correlation. Intervention A 35-year-old woman with a known vitreous cyst since childhood became increasingly troubled by its symptoms. The cyst was treated initially with argon laser photocoagulation. Vitrectomy subsequently was performed because the deflated cyst remained near the visual axis. Histopathologic studies included light and electron microscopy; immunocytochemistry for actin and glial fibrillary acidic protein (GFAP); and enzyme histochemistry for carbonic anhydrase (CA). Results The cyst was composed of a single layer of heavily pigmented cells with a thick basement membrane along the internal borders of the cells. Ultrastructurally, the cells were connected with tight junctions, had microvillous processes at their apices, and contained numerous large melanosomes in various stages of maturity, including premelanosomes. Immunochemistry showed the cells were positive for actin but negative for GFAP. Enzyme histochemical staining for CA also was strongly positive. Conclusions The confinement of this cyst to the region of Cloquet’s canal, the presence of a Mittendorf’s dot, the cyst’s existence for many years, and the finding of pigment epithelial-type cells having immature melanosomes (a feature not seen after birth in normal pigment epithelium) lead the authors to believe that this cyst was a congenital remnant of the primary hyaloidal system. Because pigmented cells are not normally present in this part of the eye, the cyst was a choristoma of the primary hyaloidal system.

Chiari's network: Normal anatomic variant or risk factor for arterial embolic events?

This study was performed to assess the prevalence of Chiari's network in patients undergoing transesophageal echocardiography and to determine whether this anomaly is associated with other cardiac lesions or is characterized by typical clinical findings. Chiari's network is a congenital remnant of the right valve of the sinus venosus. It has been found in 1.3% to 4% of autopsy studies and is believed to be of little clinical consequence. Video recordings of 1,436 consecutive adult patients evaluated by transesophageal echocardiography over a 30-month period were reviewed for the presence of Chiari's network. Echocardiographic contrast studies had been performed in all patients with Chiari's network and were compared with those of 160 consecutive patients without a Chiari net, serving as a control group. Chiari's network was present in 29 of 1,436 patients (prevalence 2%). A frequently associated finding was a patent foramen ovale in 24 (83%) of the 29 patients with Chiari's network versus 44 (28%) of 160 control patients (p < 0.001). Intense right-to-left shunting occurred significantly more often in patients with Chiari's network than in control patients (16 [55%] of 29 patients vs. 19 [12%] of 160 control patients, p < 0.001). Another frequent association was an atrial septal aneurysm in 7 (24%) of 29 patients. The indication for transesophageal echocardiography was a suspected cardiac source of arterial embolism in 24 (83%) of 29 patients with a Chiari net, 13 of whom (54%) had recurrent embolic events. Chiari's network was significantly more common in patients with unexplained arterial embolism than in patients evaluated for other indications (24 [4.6%] of 522 patients vs. 5 [0.5%] of 914 patients, p < 0.001). Potential causes for arterial embolism were present in 9 of the 24 patients with a Chiari net and embolic events (atrial septal aneurysm in 7, cerebrovascular lesion in 2). In 15 (62%) of 24 patients only a patent foramen ovale could be identified. Three patients had deep venous thrombosis and pulmonary embolism at the time of arterial embolism; none had a thrombus detected within the network. In patients undergoing transesophageal echocardiography, the prevalence of Chiari's network was 2%, which is consistent with autopsy studies. By maintaining an embryonic right atrial flow pattern into adult life and directing the blood from the inferior vena cava preferentially toward the interatrial septum, Chiari's network may favor persistence of a patent foramen ovale and formation of an atrial septal aneurysm and facilitate paradoxic embolism.

Cardiac chiari network as an etiology for embolic stroke

The Chiari network is a congenital remnant in the right atrium. It is not commonly associated wtih clinical complications. We report the first case of medically treated embolic stroke in a patient with a cardiac Chiari network and a patent foramen ovale. A 59-year-old woman without cardiac history or signs presented with multiple cerebral infarctions in both the anterior and posterior cerebral circulations. Standard evaluation for cerebral infarction was unrevealing. Transesophageal echocardiography demonstrated a Chiari network in the right atrium and a right-to-left shunt. The Chiari network appeared thickened. After anticoagulation with warfarin, the patient developed no new symptoms of stroke, and repeat transesophageal echocardiography showed resolution of the presumed thrombus on the Chiari network. In workup of patients with ischemic strokes of unknown etiology, transesophageal echocardiography should be considered as an important method for comprehensive evaluation of the right atrium.

Importance of gastric mucosa in Meckel's diverticulum.

The object of this brief communication is to reemphasize some of the complications of Meckel's diverticulum, the majority of which are the result of ectopic gastric mucosa. The course of the embryologic mishap responsible for the persistence of this congenital remnant has often been reviewed; during early fetal life bits of gastric mucosa are occasionally misplaced in the diverticulum, and in this functioning gastric mucosa peptic ulcers develop. In a group of 25 consecutive cases of symptom-producing remnants of the omphalomesenteric duct, 4 patients had persistent umbilical sinuses that required excision and were found to extend to the ileum. Two patients had rupture of an ulcer in Meckel's diverticulum with all the signs of acute abdominal disease; two had strangulation obstruction due to a persistent strand of tissue extending from the diverticulum to the abdominal wall; and one had a Meckel's diverticulum associated with intussusception. In the remaining 16 patients,

TUBERCULOSIS OF MECKEL'S DIVERTICULUM

It is well said that medical and pathologic curiosities often become of sufficient importance to be considered of practical interest. It is certain that the list of complications arising in Meckel's diverticula is daily becoming a larger and more impressive compilation. Over twenty-seven years ago, Porter1collected 184 cases of Meckel's diverticula exhibiting pathologic complications. Since that time there has been a steady and rapid rise in the reported cases, with complications ranging from ulcers of the mucosa with perforation (heterotopia of the gastric mucosa) to primary bizarre neoplasms. Previously reported by Ruysch2in 1701, it remained for Meckel,3in 1809, to describe more fully this congenital remnant and to further the theory of its origin in the remains of the omphalomesenteric duct. According to Christie,4this pouch is encountered in about 1 per cent of all persons, and is seen in the proportion of 75