In Utero Presentation of Left Ventricular Aneurysm.

Congenital left ventricular aneurysm, pseudoaneurysm, and diverticulum are rare entities. These diagnoses can be made pre- and/or postnatally. Although these entities overlap clinically and morphologically, important distinctions can allow for accurate diagnoses. Appropriate diagnosis can be imperative for risk stratification and guidance of prenatal and postnatal management. The case described in the present report highlights a challenging case of a fetal left ventricular aneurysm, management during the prenatal and postnatal periods, and important differentiating features from a ventricular diverticulum and pseudoaneurysm.

[Urethral diverticulum in a patient with two stones after combined treatment of prostate cancer with metastasis to the left cavernous body of the penis].

Urethral diverticula are sac-like dilatations of the urethra that communicate with its lumen. They may be congenital or acquired. In males, urethral diverticula are rare and classified as congenital (true) and acquired, which are associated with trauma, abscess, strictures, surgical procedures, in particular in patients with hypospadias. A clinical observation of urethral diverticulum with two stones in a man after complex treatment of prostate cancer with metastasis to the left cavernous body of the penis is presented in the article. At the first stage, the patient underwent endoscopic lithotripsy with a biopsy of the diverticulum wall and laser ablation of the stricture of urethrovesical anastomosis. At the second stage the excision of the diverticulum with urethroplasty was performed. The team of authors considers this clinical observation to be unusual. Previously, postoperative urethral diverticula with two stones in patients after complex treatment of prostate cancer with metastasis to the left cavernous body of the penis was not described in the scientific literature.

Large congenital bladder diverticula in children.

OBJECTIVE: Large congenital bladder diverticula (LCBD), congenital bladder diverticula (CBD) larger than 2 cm diameter, is a rare anomaly. The aim of this study was to report long-term surgical and clinical outcomes of children with LCBD. METHODS: Medical charts of all children who were diagnosed with LCBD at our institution between April 2005 and December 2017, with at least 2 year follow-up were retrospectively reviewed. Patients' demographics, symptoms, operative technique, diverticulum size and localization, surgical outcomes and complications were recorded. RESULTS: Fourteen patients with 18 LCBD, all male and age between 7 and 240 months (mean age: 53.5 months) were included in the study. Urinary tract infection was the main complaint in 10. Vesicoureteral reflux was detected in eight patients. Diverticula were 2-5.5 cm (mean 3.3 cm) in size. All diverticulectomies were performed transvesically and ureteroneocystostomy was added in 12 patients, 5 of whom were bilateral. No postoperative infection or recurrent reflux were observed. The median follow-up period was 4.5 years (2-12 years). CONCLUSION: Treatment of LCBD is mostly surgical and transvesical approach for diverticulectomy was found to be a safe and effective surgical procedure in long term follow-up.

Hybrid repair of aberrant right subclavian artery with aortic dissection caused by Kommerell diverticulum.

BACKGROUND: Aberrant right subclavian artery (ARSA) with associated Kommerell diverticulum (KD) is a rare congenital aortic disease. KD patients have a high risk of rupture, dissection, and compression of adjacent structures. Although several treatment options have been proposed (traditional surgery, hybrid operation, and endovascular intervention), a consensus regarding optimal surgical management has not yet been established. CASE PRESENTATION: A case of successful hybrid repair of distal aortic arch dissection aneurysm by dissecting KD and ARSA with debranching of right and left common carotid arteries, left subclavian artery, and stent grafting was presented. CONCLUSIONS: The hybrid operation is suitable for elderly patients or those with high risks. Along with intervention, the hybrid operation needs to be developed as a minimally invasive method.

A Case of Giant Right Atrium Diverticulum with Atrial Septal Defect.

Right atrium diverticulum is a rare congenital malformation. We present a previously unreported case of giant right atrium diverticulum (153 × 109 mm) in a 17-year-old female patient. Echocardiographic examination also showed an atrial septal defect (11 mm). Considering the risk of right atrium rupture, we performed femoral arteriovenous cannulation first, followed by median thoracotomy. The defect was repaired with an autologous pericardial patch. The patient recovered well after the operation, and this case is referential for surgical treatment of giant right atrium diverticulum.

Congenital right ventricular diverticulum formed by pulmonary regurgitative jet flow in a fetus with tetralogy of Fallot and absent pulmonary valve.

Congenital ventricular diverticulum is a rare anomaly with an unclear pathology. Here, we report a male fetus at 24 weeks of gestation, diagnosed with right ventricular diverticulum associated with tetralogy of Fallot and absent pulmonary valve. The diverticulum was located at the anterosuperior wall of the right ventricle and faced into the massive pulmonary regurgitation flow jet. Intrauterine fetal death from heart failure resulted at 26 weeks of gestation. An autopsy revealed significant subendocardial fibrosis in the diverticular wall without inflammatory cell infiltration. Clinical and pathologic features suggested that the regurgitative blood flow contributed to the formation of the right ventricular diverticulum.

Internal Auditory Canal Diverticula in Children: A Congenital Variant.

OBJECTIVES/HYPOTHESIS: Internal auditory diverticula in adults have been found to exist independent of otosclerosis, and in the presence of otosclerosis. We sought to determine the prevalence of internal auditory canal (IAC) diverticula in a pediatric cohort, to assess whether IAC diverticula are a risk factor for hearing loss, and the co-occurrence of otic capsule hypoattenuation. STUDY DESIGN: Retrospective review. METHODS: A single-site retrospective review of high-resolution temporal bones computed tomography (CT) scans including the presence and size of diverticula and hypoattenuation of the otic capsule. Demographic, imaging, and audiometric data were collected and descriptively analyzed. Bivariate analysis of collected variables was conducted. Comparisons between sides in unilateral cases were also performed. RESULTS: 16/600 (2.7%; 95% CI [2.0%, 3.4%]) were found to have IAC diverticula. Six were bilateral. Thirty-one patients (5.2%) were found to have hypoattenuation of the otic capsule. There were no coincident cases of IAC diverticulum and hypoattenuation of the otic capsule. There was no association between the presence of IAC diverticula and age (P = .13). In six patients with unilateral diverticula, pure tone average (P = .42), and word recognition (P = .27) scores were not significantly different when compared to the normal, contralateral side. CONCLUSIONS: The prevalence of IAC diverticula in children is lower than the prevalence in adults. IAC diverticula in children likely represent congenital variants of temporal bone anatomy. Similar to adult populations, there is evidence that IAC diverticula in children are likely not an independent risk factor for hearing loss. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E1683-E1687, 2021.

Congenital left ventricular diverticulum repair in an adult with dual patch technique.

Rupture of a congenital left ventricular diverticulum (CLVD), a rare anatomical anomaly, is a catastrophic event, with potential fatal consequences. Repair techniques documented in the literature include primary closure and single patch closure. We describe a case of a 57-year-old woman with symptomatic anterolateral CLVD. Our approach involves a linear incision through the epicardial surface of the diverticulum with exclusion of the cavity, and restoration of normal ventricular geometry via a two-patch technique.

Congenital left ventricular diverticulum: A rare cause of cardiac arrhythmia.

Congenital left ventricular diverticula are rare cardiac malformations that usually remain asymptomatic. However, life-threatening complications as ventricular arrhythmias, systemic embolism, spontaneous rupture and development of valvular regurgitation, are described. Diagnosis is based on excluding coronary artery disease, traumatic or inflammatory causes, and other underlying cardiomyopathies. Treatment is directed towards the potential complications, yielding mainly therapy of ventricular arrhythmia. Surgical resection is required for larger-sized congenital aneurysms with adverse hemodynamic effects. We present two cases of a left ventricular diverticulum causing cardiac arrhythmia which led to further surgical treatment.

Autopsy and postnatal follow-up of prenatally diagnosed ventricular outpouchings.

OBJECTIVES: Prenatal ventricular outpouchings (VOs), which include congenital ventricular aneurysms (CAs) and congenital ventricular diverticula (CD), are very rare. We describe the features and outcomes of prenatal VOs diagnosed over a 4-year period. METHODS: Retrospective cohort study of cases of prenatal diagnoses of CAs and CD at our center between June 2014 and January 2018. The prenatal and postnatal echocardiogram data were reviewed, and telephone follow-up was conducted of liveborn cases. RESULTS: A total of 25 VOs were identified. Two were lost to follow-up, 15 chose termination of pregnancy, and eight resulted in livebirths. Only two cases underwent autopsy: Histopathology showed that the CA wall was substituted by collagen fibers. At follow-up, none of the eight liveborn babies experienced adverse events, and three VOs near the tricuspid annulus almost disappeared, though one was extremely large. CONCLUSIONS: In our center, all liveborn babies with VO had good prognoses. We hypothesize that VOs located near the right ventricular annulus may be caused by prenatally unbalanced pressure, given their decrease in size after birth when the right heart pressure declines.

Natural history and management of Kommerell's diverticulum in a single tertiary referral center.

OBJECTIVE: The Kommerell diverticulum (KD) is an extremely rare developmental abnormality of the aorta related to an aberrant subclavian artery (ASCA). The objective of our study was to review the natural history of KD and ASCA using our single-center experience in diagnosing and managing KD and ASCA. METHODS: A retrospective review of the Yale radiological database from January 1999 to December 2016 was performed. Only patients with KD/ASCA and a computed tomography (CT) scan of the chest were selected for review. The primary goal was to examine the natural history of KD and ASCA and the secondary goals were to review the management and outcomes of those patients treated for KD and ASCA. RESULTS: There were 75 patients with KD/ASCA identified, with a mean age of 63 ± 19 years; 49 were female (65%). On CT scans, left- and right-sided aortas were present in 47 (63%) and 28 (37%) patients. A right ASCA or a left ASCA were present in 47 (63%) and 28 (37%) patients. Six patients were symptomatic on presentation. Symptoms included dysphagia, chest or back pain, and emboli to the fingers. The mean KD diameter was 21.8 ± 6.0 mm and the distance to the opposite aortic wall (DAW) was 48.3 ± 10.8 mm. Sixty-six patients were followed for a mean of 31.7 ± 32.5 months. One patient ruptured without repair. Nine patients underwent operative intervention, including eight open and one endovascular repair. Complications from operative intervention included ischemic stroke with hemorrhagic transformation, deep vein thrombosis and pneumonia. The mean growth rate for KD and DAW was 1.45 ± 0.39 mm/year and 2.29 ± 0.47 mm/year, respectively. On multivariable regression analysis, hypertension was a predictor of growth of DAW (P = .03). CONCLUSIONS: KD is uncommon and shows a female predominance. The diverticulum grows, albeit slowly (KD and DAW growth rates of 1.45 ± 0.39 mm/year and 2.29 ± 0.47 mm/year). Most patients are asymptomatic, but dysphagia, chest/back pain, and distal emboli may occur. Rupture is rare. Symptomatic patients should be operated. Asymptomatic patients can be followed with serial CT scans.

Outcomes of Repair of Kommerell Diverticulum.

BACKGROUND: Kommerell diverticulum (KD) is an uncommon congenital vascular anomaly. This study assessed outcomes of open and endovascular repair of this disorder. METHODS: Between 1997 and 2016, 152 adult patients presented with KD at Cleveland Clinic (Cleveland, OH), 87 had no intervention, and 65 underwent open repair (n = 55; 19 elephant trunk [ET] procedures [9 with aortic dissection], including 7 frozen ET, and completions with 5 thoracic endovascular aortic repairs, 2 open, and 5 lost to follow-up) or endovascular procedures (n = 10). Non-ET open KD repairs consisted of resection (n = 15), interposition graft (n = 16), patch (n = 4), or aortopexy (n = 1). Maximum KD diameter was 2.1 cm for nonsurgical patients and 3.2 cm for surgical patients. Among surgical patients, 51 of 65 had dysphagia or dyspnea. A total of 608 patient-years of follow-up were available. RESULTS: For patients who underwent open and endovascular procedures, after multivariable adjustment, symptoms of dysphagia and hypertension predicted the likelihood of surgery (P < .05, all). There was no operative mortality. Complications included nondisabling stroke (n = 5; 8%), tracheostomy (n = 3; 4.6%), vocal cord paralysis (n = 2; 3%), and reoperation for bleeding (n = 3; 4.6%). During follow-up, 3 of 10 patients treated with hybrid or endovascular procedures required reinterventions for endoleaks. One patient had residual symptoms after aortopexy. Among nonsurgical patients, 2 patients refused surgery, and 1 died of aortic rupture, with a 4.7-cm descending aorta and a 3.4-cm KD. Seven additional patients died of nonaortic comorbidities. The remaining patients were asymptomatic, with an aortic diameter smaller than 4.5 cm. CONCLUSIONS: Open and endovascular approaches have a high success rate and low mortality risk. Selection of the specific type of intervention should be based on patient anatomy, additional needed procedures, and comorbid conditions.

Multiple congenital ventricular diverticula causing heart failure.

This paper reports a case of multiple congenital ventricular diverticula, a rare anomaly that is associated with heart failure.

Kommerell Diverticulum: Right Aortic Arch with Anomalous Origin of Left Subclavian Artery and Duplicity of Right Vertebral Artery in a 16-Year-Old Girl.

BACKGROUND Kommerell diverticulum with a right-sided aortic arch is a rare congenital anatomical condition most often observed in adults. A possible etiology of the subclavian artery's anomalous origin would be an abnormality in regression of the fourth primitive aortic arch during embryonic development. CASE REPORT We report on the case of a 16-year-old female patient presenting with complaints of occasional tachycardia and mild non-specific dyspnea after anxiety crises. Physical examination revealed lower amplitude of the pulses in the left upper limb compared to the right upper limb, and difference in blood pressure (BP) values of 80×60 mmHg, and 100×60 mmHg, respectively. Different radiological imaging modalities were performed to elucidate a possible vascular abnormality. Multislice detector computed tomography angiography of the thoracic aorta and supra-aortic trunks showed a right-sided aortic arch and an aberrant origin of the left subclavian artery with a retroesophageal course and dilation of its emergence (Kommerell diverticulum), as well as duplicity of the right vertebral artery (RVA). Considering the actual small diameter of the diverticulum and the absence of dysphagia or severe external esophageal compression analyzed by the esophagogram, vascular surgery was not indicated. Since complications have been described in the literature, the patient must be kept under observation in the future. CONCLUSIONS Congenital vascular alterations, including Kommerell diverticulum with right-sided aortic arch and the aberrant origin of the left subclavian artery, should be suspected in otherwise asymptomatic young patients with few clinical manifestations. Investigation with different imaging methods helps to clarify the vascular abnormalities, to support a possible surgical procedure indication, and to monitor the patients in follow-up.