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.

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.

[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.

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.

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

Congenital pyriform sinus fistula presenting as a neck abscess in a newborn: A case report.

RATIONALE: Congenital pyriform sinus fistula (CPSF) is a branchial abnormality originating from the third or fourth branchial pouch and is an important cause of anterior cervical abscess in children. Here we present a case of neck abscess in a newborn that was diagnosed as CPSF. PATIENT CONCERNS: A male infant with a birth weight of 3660 g was admitted to hospital 25 minutes after birth after discovery of a cystic mass with extensive skin swelling in the left side of the neck. B-mode ultrasonography of the left neck showed an anterior cervical cystic mass of indeterminate nature. DIAGNOSIS: Congenital pyriform sinus fistula. INTERVENTIONS: The neck abscess was incised and drained under general anesthesia. Examination under suspension laryngoscopy revealed a pyriform sinus fistula. Laser cauterization was performed simultaneously. The wound was dressed and anti-inflammatory treatment was provided. OUTCOMES: The neck wound healed uneventfully. After 3 months, the fistula was confirmed to be closed by laryngoscopy under general anesthesia. No recurrence was detected during 9 months of follow-up. LESSONS: CPSF should be strongly suspected in a patient with an unexplained neck abscess or recurrent acute suppurative thyroiditis, especially on the left side.

Congenital aneurysmal right coronary artery with a fistula to the left atrium in an adult.

BACKGROUND: Congenital coronary artery fistula in association with aneurysm of the involved coronary artery in adults is rare. Moreover, the right coronary artery- left atrial fistula is also uncommon. Most of the cases are asymptomatic. However, symptomatic patients need therapeutic interventions. The potential complications associated with this anomaly are life-threatening, therefore, there is a need to explore more on differential diagnosis, investigations, management strategies and prevention of complications. CASE PRESENTATION: We present herewith a 26-year-old male patient with symptoms of chest pain and dyspnea. He was diagnosed with aneurysmal dilatation of the right coronary artery in its entire course which terminated as a fistulous communication into the left atrium. The closure of the fistula was done using autologous pericardial patch under cardiopulmonary bypass. Currently, the patient is being followed up after surgery and receiving anticoagulants. CONCLUSION: The advancement in the diagnostic imaging modalities have made it possible to find similar abnormalities more frequently. Due to rare nature of this anomaly, there is a need to explore and discuss management strategies that include medical management, surgical intervention or percutaneous interventions for a successful outcome.

Anomalías congénitas de arterias coronarias, estudio de aquellas con Importancia hemodinámica / Evaluation of the hemodynamically significant congenital anomalies of coronary arteries

Resumen: Las anomalías de las arterias coronarias son poco frecuentes, con una prevalencia de 0,21- 5,79%. Su presentación clínica es amplia, pudiendo ser asintomáticas o presentarse como isquemia miocárdica y muerte súbita, la que puede estar ligada o no al ejercicio. Existen varias clasificaciones, siendo las más usadas las que las agrupan desde un punto de vista anatómico en relación con el segmento afectado (origen, curso o terminación) y desde el punto de vista funcional (hemodinámicamente significativa y no significativa). Actualmente la Tomografía Computada Cardiaca se considera el estándar de referencia, siendo de suma importancia su adecuada caracterización ya que, de requerir tratamiento, este generalmente es quirúrgico. El objetivo de este trabajo es identificar, caracterizar y clasificar las anomalías congénitas de las arterias coronarias por su importancia hemodinámica mediante la presentación de casos clínicos y revisión de la literatura.

Abstract: Coronary artery anomalies are rare, with a prevalence ranging from 0.21 to 5.79%. Their clinical presentation is variable; being either asymptomatic or presenting as myocardial ischemia and sudden death, which may or may not be linked to exercise. There are several classifications, the most commonly used being those that sort them from an anatomical point of view in relation to the affected segment (origin, course or termination) and from a functional point of view (hemodynamically significant and not significant). Currently, Cardiac Computed Tomography is considered the reference standard allowing an adequate characterization of the anomaly, which is highly relevant since, if treatment is needed, it usually involves surgery. The purpose of this review is to identify, characterize and classify congenital anomalies of the coronary arteries according to their hemodynamic significance through the presentation of clinical cases and review of the literature.

[Clinical anatomic study on the segment and adjacent of tract of congenital pyriform sinus fistula].

Objective: To investigate the anatomic tract of congenital pyriform sinus fistula (CPSF). Methods: A total of 90 patients with CPSF undergoing open surgery between August, 2007 and March, 2017 at the Department of Guangdong General Hospital were retrospectively analyzed. Results: The tracts of all the fistulas actually walked far different from those of theoretical ones. A whole fistula may be divided into 4 segments according to adjacent anatomy of CPSF. The posterior inner segment to the thyroid cartilage was initial part of the fistula. It originated from the apex of pyriform sinus, then piercing out of the inferior constrictor of pharynx inferiorly near the inferior cornu of the thyroid cartilage (ICTC), and descended between the lateral branch of the superior laryngeal nerve and the recurrent laryngeal nerve. The ICTC segment was the second part of the fistula, firstly piercing out of the inferior constrictor of pharynx and/or cricothyroid muscle, and then entering into the upper pole of thyroid. The relationship between fistula and ICTC could be divided into three types: type A (medial inferior to ICTC) accounting for 42.2% (38/90); type B (penetrate ICTC) for 3.3% (3/90); and type C (lateral inferior to ICTC) for 54.5% (49/90). The internal segment in thyroid gland was the third part of fistula, walking into the thyroid gland and terminating at its upper pole (92.2%, 83/90) or deep cervical fascia near the upper pole of thyroid (7.8%, 7/90). The lateral inferior segment to thyroid gland was the last part of the fisula, most of which are iatrogenic pseudo fistula, and started from the lateral margin of thyroid gland. Conclusions: CPSF has a complicated pathway. Recognition of the tract and adjacent anatomy of CPSF will facilitate the dissection and resection of CPSF in open surgery.

[The anatomic characteristics and surgical treatment of congenital pyriform sinus fistula].

Objective:To discuss the clinical anatomic characteristics of congenital pyriform sinus fistula and summarize the experiences of open neck surgery. Method：The clinical data of 12 patients with congenital pyriform sinus fistula were retrospectively analyzed.Result:During the operation, all lesions were located below of superior laryngeal nerve. All fistulas were closely related with the supperior part of thyroid gland and laterally passed over the left recurrent laryngeal nerve just before merging into the apex of piriform sinus or beginning of aesophagus. Follow-up periods were ranging between 8 to 65 months. Two months after surgery,one patient showed lateral neck abscess again. After open drainage procedures and antibiotics treatment, there was no recurrence signs during 3 years of follow-up.Conclusion:We found that the congenital pyriform sinus fistula was closely related to the superior thyroid gland and recurrent laryngeal nerve,which was different from the popular theory.We had not encountered types descending into mediastinum and thoracic cavity or ascending along common carotid artery sheath. All fistulas passed over the left recurrent laryngeal nerve，and then merged into the apex of pyriform sinus or beginning of aesophagus.The key point for successful resection of fistulas in block was to remove the superior involvement part of the thyroid gland and exposure the recurrent laryngeal nerve. Based on our practical findings, the neck dissection procedure should not be taken in some patients that the anatomical structure is not clear for repeated infections.

[Experience of diagnosis and treatment for congenital pyriform sinus fistula].

Objective: To evaluate the imaging features, clinical presentation, operative methods complication and the surgical outcomes of the congenital pyriform sinus fistula(CPSF). Methods: The clinical data of 185 patients with CPSF treated from January 2013 to October 2017 at the First Affiliated Hospital of Zhengzhou University were analyzed retrospectively. Results: The lesions were predominantly on the left side(170/185). Among 185 cases, 146 cases received endoscopic coblation cauterization, 27 cases had traditional open surgery, 8 cases underwent endoscopic coblation cauterization plus traditional open surgery, 2 cases for endoscopic chemocauterization, and 2 cases for endoscopic electrosection. Twenty-eight cases recurred, with a recurrence rate of 15.1%. Postoperative hoarseness occurred in 22 cases, disappeared within 0.5-6.0 months later. Conclusions: Patients with a history of recurrent cervical abscess should be highly suspect the existence of CPSF. Endoscopic coblation cauterization is a kind of surgical method with minimal injury, with low recurrence rate and low operative risk.

[The analysis of the curative effect of low-temperature plasma cauterization on the treatment of 146 cases of congenital pyriform sinus fistula].

Objective:To investigate the therapeutic effect of low-temperature plasma cauterization on the treatment of congenital pyriform sinus fistula(CPSF).Method:All the 146 patients with CPSF received imaging examination, and the low-temperature plasma cauterization under laryngoscope was performed in the stage of inflammation control.Result:After 6-59 months of follow-up, all patients did not have pharynx fistula, and no massive hemorrhage occurred during and after operation. Nine cases of hoarseness after operation 2 d-1 months to restore normal. Thirty cases were suspected of recurrence, of which 8 cases were cured after incision and drainage, and 4 cases were cured after two cauterization.Conclusion:Low-temperature plasma cauterization is the preferred treatment for the microinvasive, simple, beautiful and repeatable operation of PSF, which can be sugessted as the first-line choice for the treatment of sinus and fistula.

[Misdiagnosic analysis and treatment of pyriform sinus fistula in children].

Objective: To discuss the misdiagnosis of pyriform sinus fistula and to better understand this kind of illness. Methods: The analysis was based on twenty-eight patients with congenital pyriform sinus fistula aged from 11 months to 14 years, with the median age of 5 years, and who were surgically treated from January 2013 to January 2017 in Kunming Children's Hospital.Twenty patients were misdiagnosed in other hospital.After the routine examination of neck ultrasound and enhanced CT, internal fistula was found by self-retaining laryngoscope, traced by methylene blue, and excised by high ligation. Results: Twenty patients were misdiagnosed.The misdiagnosis time ranged from 6 months to 3 years.Under self-retaining laryngoscope, piriform fossa fistula were found in all patients.Nineteen fistula were found in the left and 1 in the right.The fistula in patients was unilateral.Seven cases were misdiagnosed as suppurative lymphadensitis, undergone abscess incisional drainage many times.Three cases were misdiagnosed as thyroglossal duct cyst and performed excision of thyroglossal duct cyst.One case was treated by extended Sistruck operation again because the doctor considered that excision of middle segment of hyoid bone was not enough and the fistula was not ligated completely.One case was misdiagnosed as second branchial cleft fistula on the right side of the neck.Nine cases were misdiagnosed as hyroid-associated diseases including 2 cases suppurative thyroiditis, 2 cases subacute thyroiditis and 5 cases thyroid neoplasms.Among them, 2 cases underwent partial thyroidectomy.All the patients were treated with high ligation of fistula under general anesthesia.The operation was smooth, and no hoarseness, bucking and pharyngeal fistula occurred after the operation.Postoperative follow-up time ranged from 12 months to 4 years and the median follow-up was 18 months without recurrence.The diagnosis was confirmed pathologically. Conclusions: Pyriform sinus fistula in children was uncommon and easily misdiagnosed in clinic.The majority of physician including some otolaryngologists were lack of understanding of the disease.It should be regarded as one of the important differential diagnosis of neck mass in children.Children with recurrent left neck infection and/or abscess should be highly suspected.Self-retaining laryngoscopic examination can make a definite diagnosis and high ligation of the fistula through the external neck approach can achieve good therapeutic effect.