Right congenital pyriform sinus fistula.

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Surgical Repair of a Left Main Coronary Artery to Right Ventricle Fistula in a Neonate.

We describe the case of a newborn male with a large fistula from the left main coronary artery to the right ventricle. This case illustrates a rare congenital coronary artery fistula and its successful surgical management in the neonatal period.

An unusual cause of acute coronary syndrome: thrombosis of right coronary artery to right atrium fistula.

Coronary arterial fistulae are rare, but it is one of the most common coronary artery anomalies. Most of the cases are asymptomatic in younger patients unless it is large and of haemodynamic significance. The incidence of thromboembolic complications usually increases with age. We report a case of a young male in his early 20s presenting with central chest pain. Coronary computed tomographic angiography revealed acute coronary syndrome due to a fistula between right coronary artery and right atrium occluded by thrombus. After discussion with coronary and congenital heart multidisciplinary team, a consensus was agreed that we should manage him conservatively with anticoagulant and antiplatelet therapy and a 3-month follow-up strategy that included repeating cardiac imaging. After a year, his anticoagulation and antiplatelet medication was discontinued.

Auricular fistula: a review of its clinical manifestations, genetics, and treatments.

Auricular fistula is a common congenital auricular malformation, characterized as a small opening in the skin and a subcutaneous cyst. It can be classified in different ways according to positions of pits and directions of fistula tracts. The term preauricular fistula and variant type of preauricular fistula (postauricular fistula) are used. Auricular fistula prevalence varies in countries and populations, and its actual prevalence is presently unknown. The most accepted and widely cited theory of auricular fistula etiopathogenesis is an incorrect or incomplete fusion of six auricular hillocks that are mesenchymal proliferations. Auricular fistula can occur either sporadically or genetically. The pattern in inherited cases is thought to be incomplete autosomal dominant, with variable expressions, reduced penetrance, and inapparent gender differences. Auricular fistula has several forms and is reported as being a component of many syndromes. In the field of genetics, currently, there is no related review to comprehensively summarize the genetic basis of auricular fistula and related disorders. This article provides a comprehensive review of auricular fistula, especially congenital preauricular fistula, which accounts for the majority of auricular fistula, by summarizing the clinical manifestations, histological and embryological development, genetics, examinations, and treatments, as well as syndromes with auricular fistula.

Collaural fistula: Our experience.

Branchial cleft anomalies are congenital, arising from the first to the fourth pharyngeal clefts. The most common is a second arch anomaly. As it is congenital, it presents at birth though may become symptomatic later. The spectrum of anomalies includes sinus, cyst, or fistula formation or a combination of these. Here we present a case series based on first cleft anomalies. The principles of management include early diagnosis, excision of any fistulous tract, and prevention of injury to the facial nerve.

Endoscopic Suture With Chemocauterization: An Effective Treatment of Congenital Pyriform Sinus Fistula.

OBJECTIVE: Endoscopic cauterization is an effective method for treating pyriform sinus fistula (PSF). However, these approaches sometimes result in a higher failure rate. We present an effective technique utilizing suture combined with chemocauterization as first-line treatment in patients with PSF and evaluate the safety and efficacy of its use in 126 patients. STUDY DESIGN: Retrospective study. SETTING: Tertiary referral center. METHODS: Retrospective case review of patients treated between March 2012 and June 2021 at our institution with descriptive statistical analysis. RESULTS: A total of 126 patients with PSF were included in this study with a mean age of 14.7 years. There was no sex predilection. The majority of patients presented with a left-sided neck lesion (89.7%). Ten patients presented following prior attempts at the surgery of the PSF at another institution; 8 via open surgery and 2 following endoscopic CO2 laser cauterization; other patients only had a history of repeat incision and drainage or antibiotic treatment. The success rate of obliteration of the internal opening was 96.83% after a single treatment without complications. Following reoperation, a successful outcome was achieved in the remaining 4 patients. Length of stay ranged from 10 to 14 days. No recurrences occurred within 12 to 120 months followed-up. CONCLUSION: Endoscopic suture combined with chemocauterization is a safe and effective treatment of PSF. Surgery can be performed during the acute cervical inflammatory period without increased risk of complication or recurrence, however, patients found to have acute changes affecting the pyriform sinus should be treated with a staged surgery strategy.

Hoarseness as the first symptom in a patient with acute suppurative thyroiditis secondary to a pyriform sinus fistula: a case report.

BACKGROUND: Pyriform sinus fistulas (PSFs) are rare congenital anomalies of the third or fourth brachial pouch. Dyspnea is reportedly secondary to compression by a neck mass. However, hoarseness, as the first symptom of PSF, has not yet been reported. CASE PRESENTATION: This report describes an 11-year-old girl presenting with hoarseness as the first symptom of PSF. Hoarseness occurred 2 days prior to admission. On admission, she had fever, hoarseness, and an elastic soft mass on her left anterior neck. Contrast-enhanced computed tomography of the cervical region demonstrated an abscess partially infiltrating the thyroid gland and an air pocket near the pyriform sinus. Pharyngoscopy revealed swelling of the left arytenoid region, with purulent retention. The left vocal cord was swollen but not paralyzed. Additionally, the laboratory data indicated thyrotoxicosis. Suspecting a PSF infection, parenteral treatment with cefotaxime and dexamethasone was initiated. On the following day, the hoarseness disappeared, and the fever resolved. Four weeks after onset, the thyroid hormone levels returned to the normal range, and a barium esophagogram revealed residual contrast in the left pyriform sinus, leading to a diagnosis of PSF. CONCLUSION: PSF presenting with hoarseness as the first symptom in patients should be considered.

[Clinical application of modified fistulectomy in the treatment of congenital pyriform sinus fistula based on segmental anatomy of fistula].

Objective:To discuss the clinical application and significance of the modified piriform fossa fistulectomy based on segmental anatomy of fistula. Methods:The clinical data of 84 patients with CPSF treated by modified pyriform sinus fistulectomy were analyzed retrospectively. The modified piriform fossa fistula resection adopts the fistula anterograde anatomy method to fine dissect the fistula. The operation procedure can be summarized into four parts: retrograde anatomy of recurrent laryngeal nerve, anatomy of external branch of superior laryngeal nerve, anterograde anatomy of fistula and partial thyroidectomy. Results:All 84 patients successfully completed the operation and discharged from the hospital. The operation time was（64.6±20.0） min, the intraoperative bleeding was（19.6±13.0） mL, and the average hospital stay was（6.8±1.1） d. Postoperative infection occurred in 1 case（1.19%）, temporary vocal cord paralysis in 1 case（1.19%）, no bleeding, pharyngeal fistula, dysphagia, permanent vocal cord paralysis and choking cough. The incidence of complications was 2.3%（2/84）. No complications such as permanent vocal cord paralysis and hypothyroidism occurred. Follow up for 57-106（Median 74） months showed no recurrence. Conclusion:A modified procedure based on segmental dissection of the fistula not only simplifies the traditional procedure, but also procedures the specific steps to provide a targeted and precise resection, which provides a proven surgical solution for complete eradication of the lesion and significantly reduces complications and recurrence.

Congenital Common Canalicular Lacrimal Fistula in an Asymptomatic Patient.

This case report discusses a diagnosis of congenital common canalicular lacrimal fistula in a 24-year-old asymptomatic patient.

Surgical management of symptomatic coronary artery fistulae in the adult.

We describe the surgical management of adult symptomatic coronary artery fistulae. The technique is a fundamental approach entailing cardiopulmonary bypass and cardiac arrest with the goal of fully identifying the epicardial course of the coronary fistulae as well as that of the intrapulmonary artery ostial shunt. The more accurate the localization of these primary components of the fistulous tract, the more precise and successful is the ligation of the aberrant coronary connections. This result subsequently enhances the successful surgical obliteration of the symptomatic left-to-right shunt inherent in these congenital coronary fistulae that may not manifest symptoms until adulthood. With conventional cardiopulmonary bypass, myocardial protection and arrest, the main pulmonary artery is opened between the pulmonary valve and its bifurcation. Additional antegrade cardioplegia is administered, and the ostial connection of the coronary fistulae can be identified in the wall of the main pulmonary artery and internally ligated. After this, the epicardial course of the coronary fistulae can be identified and doubly ligated as close as possible to the native coronary from which they originate as well as their approximate external connection to the main pulmonary artery.

Prenatal diagnosis of pyriform sinus fistula using ultrasonography by detecting the communication to the pharynx.

OBJECTIVE: Pyriform sinus fistula (PSF) is a congenital anomaly which originates from the pharyngeal pouch. PSF is initially recognized as a cyst around the fetal neck, but accurate prenatal diagnosis of the disease is challenging. We aimed to report the key findings and tips in accurately distinguishing PSF from other differential diagnosis by which enables detection of the communication of the nuchal cyst and the pharynx. CASE REPORT: We report a case in which we were able to diagnose PSF as early as 18 weeks of gestation with ultrasonography. We used epiglottis as a landmark, and detected an unilobular cyst arising from the pharynx. CONCLUSION: Ultrasonography is a powerful tool in prenatal diagnosis of PSF especially at early stage of pregnancy. By detecting the epiglottis, it can locate the communication of the nuchal cyst and the pharynx, and thereby enables an accurate diagnosis of PSF.

Misdiagnosis of Congenital Posterior Urethroperineal Fistula and Comparison With Urethral Duplications and Rectourethral Fistula.

Congenital posterior urethroperineal fistula (CUPF) is a urothelium-lined tract between the posterior urethra and perineum. This condition is rare and has been proposed to be a urethral duplication variant. A case of CUPF that was misdiagnosed and surgically treated as a rectourethral fistula is presented. The clinical presentation, diagnosis, and treatment of CUPF are discussed and compared with those of Y-type urethral duplications and H-type rectourethral fistulas.

Spontaneous Congenital Perilabyrinthine Cerebrospinal Fluid Fistulas.

OBJECTIVES: To report a recalcitrant spontaneous cerebrospinal fluid (CSF) fistula arising from multiple, anatomically-linked lateral skull base defects, and to review the available literature to determine optimal techniques for operative repair of congenital CSF fistulae. METHODS: A patient with recurrent episodes of otologic meningitis was found to have a patent tympanomeningeal fissure, also known as a Hyrtl's fissure, and internal auditory canal (IAC) diverticulum that communicated with the jugular bulb. A systematic review of the literature characterized all reports of spontaneous congenital perilabyrinthine CSF leaks, and all cases of Hyrtl's fissures. RESULTS: An 11-year-old female was referred for recurrent meningitis. Imaging demonstrated a fistulous connection between the middle ear and IAC diverticulum via the jugular foramen. Specifically, a Hyrtl's fissure was identified, as well as demineralized bone around the jugular bulb. Obliteration of the fissure was initially performed, and a fistula reformed 4 months later. Multifocal CSF egress in the hypotympanum was identified on re-exploration, and middle ear obliteration with external auditory canal (EAC) overclosure was performed. A systematic review of the literature demonstrated 19 cases of spontaneous congenital perilabyrinthine CSF leaks. In total, 6 cases had multiple sources of CSF leak and 2 had history suggestive of intracranial hypertension. All of these noted cases demonstrated leak recurrence. Middle ear obliteration with EAC overclosure was successful in 4 recalcitrant cases. CONCLUSIONS: Repair of spontaneous congenital perilabyrinthine CSF leaks in cases demonstrating multiple sources of egress or signs of intracranial hypertension should be approached with caution. Middle ear obliteration with EAC overclosure may provide the most definitive management option for these patients, particularly if initial attempt at primary repair is unsuccessful.

Spontaneous Cervicovaginal Fistula in Obstructed Hemivagina and Ipsilateral Renal Anomaly Syndrome: A Case Report.

BACKGROUND: Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare diagnosis; patients classically present with dysmenorrhea and increasing pelvic pain. Unusual manifestations of OHVIRA syndrome might occur as a result of patient anatomy and the rupture or relief of the obstructed hemivagina. CASE: We present a 15-year-old patient with OHVIRA syndrome who developed a spontaneous cervicovaginal fistula and subsequently underwent hemihysterectomy.

Right coronary artery-coronary sinus fistula diagnosed by three-dimensional echocardiography.

Right coronary artery-coronary sinus fistula is a very rare congenital anomaly in which a right coronary artery fistula drains into the right atrium, right ventricle, or pulmonary artery. A right coronary artery-coronary sinus fistula was diagnosed in a 44-year-old man by three-dimensional echocardiography and confirmed by computed tomography angiography and surgery. Relevant published experience in diagnosing this kind of anomaly is summarized.

Absceso retrofaríngeo por fístula congénita del seno piriforme en una adolescente / Retropharyngeal abscess due to a congenital pyriform sinus fistula in a teenager

Arch Argent Pediatr 2020;118(1):e81-e84 / e81Presentación de casos clínicosRESUMENLas fístulas del seno piriforme son anomalías poco frecuentes de los arcos branquiales. La forma de presentación más común en los niños y los adolescentes es la tiroiditis aguda supurada y/o los abscesos cervicales laterales recurrentes. Sin embargo, las fístulas se pueden manifestar de forma atípica. La rareza de esta patología y la presentación clínica atípica pueden demorar el diagnóstico, lo que aumenta el riesgo de infecciones recurrentes y complicaciones.Se presenta el caso inusual de una adolescente de 13 años con absceso retrofaríngeo debido a una fístula congénita del seno piriforme, tratada de forma exitosa mediante electrocauterización endoscópica.

Pyriform sinus fistulas are rare anomalies of the branchial arches. The most common form of presentation in children and adolescents is acute suppurative thyroiditis and/or recurrent lateral cervical abscesses. However, fistulas can manifest atypically. The rarity of this pathology and the atypical clinical presentation can delay the diagnosis increasing the risk of recurrent infections and complications.We present the unusual case of a 13-year-old teenager with retropharyngeal abscess due to a congenital pyriform sinus fistula successfully treated by endoscopic electrocautery

[Retropharyngeal abscess due to a congenital pyriform sinus fistula in a teenager]. / Absceso retrofaríngeo por fístula congénita del seno piriforme en una adolescente.

Pyriform sinus fistulas are rare anomalies of the branchial arches. The most common form of presentation in children and adolescents is acute suppurative thyroiditis and/or recurrent lateral cervical abscesses. However, fistulas can manifest atypically. The rarity of this pathology and the atypical clinical presentation can delay the diagnosis increasing the risk of recurrent infections and complications. We present the unusual case of a 13-year-old teenager with retropharyngeal abscess due to a congenital pyriform sinus fistula successfully treated by endoscopic electrocautery.

Las fístulas del seno piriforme son anomalías poco frecuentes de los arcos branquiales. La forma de presentación más común en los niños y los adolescentes es la tiroiditis aguda supurada y/o los abscesos cervicales laterales recurrentes. Sin embargo, las fístulas se pueden manifestar de forma atípica. La rareza de esta patología y la presentación clínica atípica pueden demorar el diagnóstico, lo que aumenta el riesgo de infecciones recurrentes y complicaciones. Se presenta el caso inusual de una adolescente de 13 años con absceso retrofaríngeo debido a una fístula congénita del seno piriforme, tratada de forma exitosa mediante electrocauterización endoscópica.