The treatment of congenital pyriform sinus fistula: a single-center experience.

PURPOSE: Congenital pyriform sinus fistula (CPSF) often presents diagnosis and treatment challenges. This study aimed to explore the treatment principles and to evaluate the effectiveness of the hypothermia plasma cauterization with suspension laryngoscopy for CPSF. METHODS: The medical records of 56 patients with CPSF from January 2000 to December 2019 were retrospectively reviewed. RESULTS: Of the 56 cases, the lesions were predominantly located on the left side (95%), and the accuracy of the first diagnosis was 30%. Ultrasound showed an abnormal rate of 86%, while CT or MRI displayed an abnormal anatomic lesion of 92%. The 3D visual reconstruction enabled the analysis of morphological characteristics of CPSF. The positive predictive value of barium esophagography was 89%, whereas the positive rate of the internal opening in CPSF under local anesthesia laryngoscopy was 33%. Nine cases of sinus type underwent open resection, and the recurrence rate was 33%. Interestingly, ten patients with sinus type underwent hypothermia plasma cauterization with suspension laryngoscopy, leading to a success rate of 100% without apparent complications. CONCLUSIONS: Hypothermia plasma cauterization with suspension laryngoscopy alongside 3D imaging is both minimally invasive and repeatable with neglectable complications, which has the potential to serve as the first-line treatment for CPSF in the future.

Congenital Pyriform Sinus Fistula: Management of an Extra-Delayed and Atypical Case.

Clinical presentations of pyriform sinus fistulas vary, and this sometimes leads to a delay in diagnosis and treatment. Recently, we experienced a case of recurrent cervical abscesses occurring after thyroidectomy in an adult affected by a bifidus pyriform sinus fistula. The diagnostic dilemma was related to the timing of events, with a single episode of acute suppurative thyroiditis having occurred 16 years before the onset of the more recent clinical scenario. An endoscopic approach allowed effective management of this clinical case.

Recurrent neck lesions secondary to pyriform sinus fistula.

Recurrent neck lesions associated with third or fourth branchial arch fistula are much less common than those of second arch and usually present with acute suppurative thyroiditis or neck abscess. Our aim is to describe clinical features, management and treatment outcomes of 64 cases of congenital pyriform sinus fistula (PSF). Medical record of these 64 patients (33 males, 31 females) treated at the First Affiliated Hospital of Zhengzhou University from 2011 to 2014 were reviewed. The patients comprised 33 males and 31 females, and their ages ranged from 18 months to 47 years (median 10 years, mean 12.7 years). Neck abscess and recurrent infection was the mode of presentation in 37 cases (57.8 %), 4 patients (6.3 %) presented with acute suppurative thyroiditis, neck mass was the mode of presentation in 17 cases (26.6 %), 2 patients (3.1 %) presented with neck mass with respiratory distress, and cutaneous discharging fistula was the mode of presentation in 1 cases (1.6 %). The remaining 3 patients (4.7 %) presented with cutaneous discharging fistula with neck infection. Investigations performed include barium swallow, CT scan, and ultrasound which were useful in delineating PSF tract preoperatively. Barium swallow was taken as the gold standard for diagnosis. Our patients were treated by fistulectomy with hemithyroidectomy, fistulectomy, fistulectomy with endoscopic electric cauterization, endoscopic electric cauterization or endoscopic coblation cauterization, respectively. Histopathologic examination of the surgical specimens revealed that they were lined with ciliated epithelium, stratified cuboid epithelium with chronic inflammatory cell infiltration and fibrosis. Voice hoarseness occurred after operation in seven patients, but disappeared 1 week later. PSF recurred in 6 patients, 4 of them were cured by a successful re-excision. One patient was cured by successful endoscopic electric cauterization. The other 1 has remained asymptomatic for 5 months. In our series, mean follow-up period was 13.3 months and median follow-up period was 12.5 months (range 2-40 months). Presence of congenital PSF should be suspected when intra-thyroidal abscess formation occurs as the gland is resistant to infection. Strong clinical suspicion, barium swallow study, CT scan and ultrasound are the key to diagnosis. Both fistulectomy with hemithyroidectomy and endoscopic treatment have comparable success rate. Endoscopic coblation cauterization may prove a useful and equally effective method of treatment for PSF in future.

[Lung perfusion scintigraphy in pediatric patients with congenital malformations]. / La scintigrafia polmonare in età neonatale e pediatrica per patologie congenite.

Congenital malformations are an heterogeneous group of disorders that can lead changes in pulmonary perfusion and then can be evaluated with lung perfusion scintigraphy. We selected five patients in which the role of lung perfusion scintigraphy emerges as a reliable and non-invasive imaging technique. Lung perfusion scintigraphy is a useful tool in pediatric patients with congenital malformations allowing an accurate evaluation of the best therapeutic strategy and its results.

Surgical correction of midline nasal dermoid sinus cysts.

Nasal dermoid sinus cysts (NDSCs) are rare congenital anomalies affecting approximately 1 in 30,000 live births. Nasal dermoid sinus cysts are unsightly, prone to infection, and, importantly, may communicate with the central nervous system. Treatment is complete surgical excision. This study retrospectively evaluated management of a large single-center cohort of intracranial NDSCs.Nineteen patients with NDSC were identified from all patients presenting to the Leeds craniofacial service between June 2000 and August 2008. Patient demographics, clinical presentation, preoperative investigations, and surgical procedures undertaken were analyzed.Mean age at presentation and surgery were 6.3 and 7.6 years, respectively. Fifty-three percent were males. Computed tomography (CT) and magnetic resonance imaging (MRI) were performed in 15 and 17 patients, respectively. One patient (5.3%) required local excision only. Eighteen (96.7%) underwent a bicoronal approach, and 13 (68.4%) of these required a craniotomy. The dura was opened in 7 (36.8%) patients. Neither CT nor MRI predicted the presence or absence of intracranial extension in all patients. Positive and negative predictive values for intracranial extension were 85.7% and 50% for CT and were 100% and 50.0% for MRI. Mean follow-up of 4.1 years shows no deep recurrences and 5 (26.3%) were superficial nasal recurrences only.A multidisciplinary approach can achieve good results with infrequent intracranial recurrence. We used a bicoronal approach to facilitate craniotomy when required intraoperatively because imaging is unable to diagnose intracranial extension with sufficient accuracy.

Developmental oronasal fistula of the incisive papilla.

Various terms (including patent nasopalatine fistula and patent nasopalatine duct) have been used to describe the presence of a developmental fistulous tract that connects the oral and nasal cavities through an oral opening located at the incisive papilla. Reportedly, this condition is a rare developmental variant; approximately 40 cases have been reported in the literature. Because awareness of this entity is important to avoid misdiagnosis, this article presents the clinical and conebeam computed tomography findings of two cases. Based on a review of the development of the nasopalatine structures in man, the authors propose that this entity be classified as a developmental oronasal fistula of the incisive papilla.

[The patent nasopalatine duct. A rare anomaly and diagnostic pitfall]. / Der offene Ductus nosopalatinus. Eine seltene Missbildung und diagnostische Falle.

The patent nasopalatine duct is a rare anomaly in the anterior maxilla. During the early fetal period, a bilateral and epithelium-lined duct is formed within the primary palatal process as an oro-nasal communication. However, the duct obliterates and degenerates before birth. A persisting patent or through-and-through nasoplatine duct is therefore considered a developmental anomaly. A patent nasopalatine duct normally presents as one (or two) tiny openings lateral or posterior to the incisive papilla. In such a case, the ducts can be partially or completely probed with gutta-percha points with subsequent radiographic imaging. The patients report strange sensations such as squeaking noise, palatal drainage, nasal regurgitation, or airway communication between nasal and oral cavities; however, patients rarely complain about pain. About 40 cases have been documented in the literature. We describe two patients who have been referred to our department for evaluation of "sinus tracts" in the anterior palate. Since a patent nasopalatine duct can become a diagnostic pitfall, a thorough inspection of the mucosa around the incisive papilla is essential to avoid unnecessary endodontic or surgical interventions in the area of the central maxillary incisors.

CO2 laser cauterization approach to congenital pyriform sinus fistula.

OBJECTIVE: To evaluate the efficacy of CO2 laser cauterization with suspension microlaryngoscopy as a definitive surgical treatment for pediatric Congenital Pyriform Sinus Fistula (CPSF). MATERIAL AND METHODS: This is a cohort retrospective study. Thyroid function and cervical ultrasonography examinations were performed before operation. Enhanced magnetic resonance imaging (MRI) was performed on patients with a repeated infection (≥2 times) and/or if they had a prior open surgery. Patients were divided into two groups: the <8-year-old group and the ≥8-year-old group. The differences in the number of cauterization procedures between the two age groups and between the initial treatment and the retreatment groups were analyzed. RESULTS: CO2 laser cauterizations with suspension microlaryngoscopy were performed for 104 CPSF patients. No complications occurred. Three patients had a recurrence in the follow-up. The number of surgical cauterization operations was fewer than 3 in 85.1% of the patients. There was no significant difference in the number of cauterizations among the different age groups or between the initial treatment and retreatment groups (P>0.05). CONCLUSION: CO2 laser cauterization with suspension microlaryngoscopy is a safe, effective, and minimally invasive approach to CPSF with optimal patient outcomes. TYPE OF STUDY: Treatment Study. LEVEL OF EVIDENCE: Level III.

How to make an accurate diagnosis of fetal pyriform sinus fistula in utero: experience at a single medical center in mainland China.

OBJECTIVE: The aim of this study was to make an accurate diagnosis of pyriform sinus fistula (PSF) for prenatal diagnosis. STUDY DESIGN: Medical records were reviewed for all 35 pyriform sinus fistula patients presenting between 2011 and 2017. Ultrasonography (US), fetal magnetic resonance imaging (MRI) and karyotyping were offered during gestation, while computer tomography (CT) and barium esophagography were performed after birth. RESULTS: Patients included 21 males (60%) and 14 females (40%) with a sex ratio of 1.5:1. The lesion was located on the left side in 32 (91.4%) cases, the right side in 2 (5.7%), and was bilateral (2.8%) in only one case. The sensitivity of CT, MRI, ultrasonography and barium esophagography were 100% (35/35), 69.2% (9/13), 22.9% (8/35), and 80% (20/25), respectively. If the diagnosis was correct, there was almost no recurrence after treatment. Karyotype analysis of all fetuses was normal. CONCLUSIONS: Pyriform sinus fistula is more commonly seen in the left side. Compared with ultrasonography, MRI has more advantages in prenatal diagnosis, and it is more accurate in postpartum CT examination. The outcome of children with pyriform sinus fistula may be guarded when it correct diagnosis.

[Advances in endoscopic treatment of congenital pyriform sinus fistula in children].

Pyriform sinus fistula is a rare clinical disease, often with recurrent acute suppurative thyroiditis or neck infection in children.The traditional method treatment is complete resection of the fistula with or without hemithyroidectomy in external cervical surgical approach, but in recent years, minimally invasive endoscopic occlusion of the internal opening for the treatment of pyriform sinus fistula is performed in many hospitals, including electrocautery, chemocautery, laser cauterization, biocauterization, and suture closure. Literatures about endoscopic management of pyriform sinus fistula in children are reviewed and various surgical methods, complications and success rate are evaluated.

KTP laser assisted endoscopic tissue fibrin glue biocauterization for congenital pyriform sinus fistula in children.

OBJECTIVE: This study aims to assess the efficacy of a novel endoscopic management for congenital pyriform sinus fistula (CPSF) using potassium titanyl phosphate (KTP) laser assisted endoscopic tissue fibrin glue biocauterization in children. METHOD: From 2010 to 2014, a total of 5 children with recurrent or acute suppurative thyroiditis or neck abscess secondary to CPSF were enrolled retrospectively in this study. RESULTS: Mean age at the first time of endoscopic biocauterization was 6.2 ± 0.7 (5-7) years. The barium swallow study detected a fistula in four cases. Endoscopy identified an internal opening at the pyriform sinus in all cases with four on the left side and one on the right side. All patients underwent KTP laser assisted endoscopic tissue fibrin glue biocauterization as treatment for CPSF. Only one case required the second endoscopic procedure due to fluctuation of symptoms. Post-endoscopic follow-up duration of these patients was 24.6 ± 11.6 (7-36) months. Neither complications nor recurrences were noted during follow-up in all patients. CONCLUSIONS: For children presenting with repeated acute suppurative thyroiditis or neck infections, clinicians should highly suspect the possibility of CPSF. Endoscopy should be performed not only to confirm the diagnosis but also could be served as an initial treatment modality of biocauterization by KTP laser and tissue fibrin glue, which was demonstrated as a less invasive, safe, and effective method in children.

Congenital tracheobiliary fistula in an adolescent patient.

Congenital tracheobiliary fistula is a rare malformation that allows communication between the respiratory system and hepatobiliary tract. We describe a male adolescent patient who was admitted with a destroyed lung caused by repetitive bile pneumonitis with a congenital tracheobiliary fistula. Left pneumonectomy was performed, and the fistula tract was successfully divided.

Communicating bronchopulmonary pancreatic foregut malformation.

Bronchopulmonary foregut malformations include intralobar and extralobar pulmonary sequestrations, bronchogenic cysts, and communicating bronchopulmonary foregut malformations (CBPFM). These malformations, formes frustes, originate as developmental abnormalities of ventral foregut budding of the tracheobronchial tree or the gastrointestinal tract. The communication's patency with the parent viscus determines if a contained malformation occurs, or if an abnormal communication persists as a CBPFM. This case demonstrates a unique example of a CBPFM in which the main pancreatic duct communicated with pulmonary parenchyma through a retroperitoneal fistula.

Congenital tracheocutaneous fistulas.

OBJECTIVE: To outline two cases of congenital tracheocutaneous fistula and discuss the potential pathogenesis of this previously unreported developmental abnormality. METHODS: Two cases of tracheocutaneous fistula evaluated at John Peter Smith Hospital (Fort Worth, TX) from May to October 2001 were reviewed. The surgical treatment of one infant is described. RESULTS: Two infants were evaluated with a congenital fistula extending from the suprasternal region of the neck dorsally to the trachea in the midline. The infants were otherwise developmentally normal with unremarkable prenatal histories. Primary surgical closure of a fistula was accomplished without complication. CONCLUSIONS: Congenital tracheocutaneous fistula appears to be an isolated developmental abnormality not associated with the same degree of morbidity as acquired tracheocutaneous fistula. The development of a congenital tracheocutaneous fistula may be the result of abnormal epidermal migration secondary to a localized midline mesodermal defect. Congenital tracheocutaneous fistula may be successfully treated with primary closure. Observation and close follow-up of asymptomatic fistulas may be reasonable.

Endoscopic-assisted surgery for pyriform sinus fistula in children: experience of 165 cases from a single institution.

BACKGROUND: Congenital pyriform sinus fistula (PSF) is relatively rare, but often presents diagnostic and therapeutic challenges. Herein, we report our experience of endoscopic-assisted surgery of PSF in children. METHODS: Since 1999, 165 children (100 males, 65 females) with PSF had been enrolled. Their clinical manifestations were recurrent lateral neck infection, cervical mass and respiratory distress. Preoperative investigations included barium swallow, ultrasound, computed tomography, and thyroid scan. After resolution of the infection, the fistulas were identified by the endoscopic-assisted technique at operation. The fistula tract was completely excised just at the apex of the pyriform sinus. More recently, the anatomic point where the fistula tract penetrated into the pharynx was specified and recorded. RESULTS: Of the 165 cases, the male to female ratio was 1.54:1, the median age of onset was 3.2 years (range, 1 day to 13.8 years), and median age at operation was 5.0 years (range, 17 days to 15.0 years). One hundred fifty-six (94.6%) fistulas located on the left side, 7 right, and 2 bilateral. Twelve neonates and young infants (younger than 3 months) presented with a large cervical cyst. The fistulas were completely excised in all but 2 (98.8%) with intraoperative gastroscopy successfully conducted in 160 cases (97.0%). In 77 cases the points where fistulas penetrated into the pharynx were specified intraoperatively, which were classified into 3 types according to their anatomic relationship with the inferior cornu of the thyroid cartilage (ICTC): type I (anterior to ICTC), 22 cases (28.5%); type II (inferior to ICTC), 18 cases (23.4%); and type III (posterior to ICTC), 37 cases (48.1%). Postoperatively, 160 cases recovered well without complications. PSF recurred in 5 cases, 2 of whom were cured by fistula re-excision and 3 remained asymptomatic. Esophageal perforation was found and repaired uneventfully in 1 neonate and 1 young infant. Transient postoperative hoarseness happened in 1 neonate. All the latter 3 cases had cervical cysts. CONCLUSIONS: To our knowledge, this series is the largest report of PSF in children. Our results suggested that PSF is more common in males. With the help of endoscopy and a better understanding of the anatomic relationship between ICTC and the points where the fistulas penetrated into the pharynx, PSF excision can be done successfully with minimized complications. For neonates and young infants with a cervical cyst, however, the management of PSF continues to be a challenge.

Congenital tracheobiliary fistula.

Congenital tracheobiliary fistula is a rare malformation that can present with a variety of respiratory symptoms. We present a case of a newborn patient with a tracheobiliary fistula and severe respiratory insufficiency needing extracorporal membrane oxygenation to recover.