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.

Prenatal diagnosis and early postnatal management of pyriform sinus cyst: experience at a single medical center in mainland China.

OBJECTIVE: The aim of this study was to determine whether prenatal diagnosis of pyriform sinus cyst can improve the prognosis of this disorder. METHODS: A retrospective review was performed in 15 neonates with a pyriform sinus cyst seen at a single center between 2010 and 2014. Among the 15 cases, the diagnosis was made prenatally in eight cases (PreD), while the diagnosis was made postnatally in seven cases (PostD). Neonatal outcome was compared in the two subgroups. RESULTS: The mean gestational age at diagnosis of PreD was 27 ± 6.8 weeks, while the mean age at admission of PostD was 10.1 ± 8.8 days. Cervical mass, fever, respiratory distress, and hoarseness were common symptoms. The mean duration of postoperative mechanical ventilation was 11.5 ± 13.9 and 100.71 ± 80.0 h, respectively, in PreD and PostD (p < 0.01). The average postoperative length of stay and the length of hospital stay were 11.3 ± 3.34 and 19.6 ± 4.41 days in PreD, and 15.14 ± 8.28 and 24.14 ± 8.51 days in PostD, respectively. CONCLUSION: Prenatal diagnosis and timely postnatal sequential intervention were associated with less complications and shortened duration of postoperative mechanical ventilation. © 2016 John Wiley & Sons, Ltd.

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.

[Piriform sinus fistula resulting in acute dyspnea in a newborn]. / Sinus-piriformis-Fistel als seltene Ursache von Dyspnoe bei einem Neugeborenen.

Piriform sinus fistulas are rare congenital deformities that may become symptomatic through cyst enlargement and inflammation. The fistula usually manifests as bacterial thyroiditis and is very uncommonly seen as a cause of acute dyspnea in newborns. We report the case of a newborn in whom a piriform sinus fistula led to acute breathing impairment. If the piriform sinus fistula and adherent cyst cannot be totally removed initially, the treatment must be followed later by a complete resection to prevent infection.

[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.

[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.

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.

[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.

Endoscopic Cauterization with Pneumatic Distension for Piriform Fossa Sinus Tracts.

INTRODUCTION: Piriform fossa sinus tracts (PFSTs) are a cause of recurrent neck infections in the pediatric population. Conventional management required open resection, but over the last years minimally invasive approaches have been reported in an attempt to endoscopically obliterate the PFST, using different methods such as electrocautery, laser, trichloroacetic acid, or silver nitrate. MATERIALS AND METHODS: We undertook a retrospective review of the medical records of 12 children (aged 4 months to 14 years) with PFSTs treated with endoscopic sclerosis with diathermy (ESD) between 2010 and 2016 at a tertiary care children's hospital. We also present a technical modification of ESD, using continuous infusion of airflow through the gastroscopy, to distend the piriform sinus and facilitate its recognition. PFST obliteration was performed using diathermy through a guide wire. RESULTS: Clinical presentation of the 12 affected children included neck tumor (7 [58%]), neck abscesses (4 [33%]), and thyroiditis (5 [41%]). All lesions occurred on the left side. All patients underwent both ultrasonography and barium esophagography (the latter being positive only in 50%). Two patients were treated with ESD after the open approach had failed. There was no procedure-related morbidity. One patient had a recurrence (positive barium swallow without symptoms). The success rate of this procedure in our series was 91% with one attempt and 100% with two attempts. CONCLUSION: In our experience, treatment of PFST with ESD is a reproducible, noninvasive, and an effective option. ESD could be considered a primary approach and also for revision after open surgery has failed in these patients.

Abnormal sensorimotor integrative function of the larynx in congenital laryngomalacia: a new theory of etiology.

OBJECTIVES/HYPOTHESIS: Laryngomalacia is an enigmatic disease in which laryngeal tone is weak, resulting in dynamic prolapse of tissue into the larynx. Sensorimotor integrative function of the brainstem and peripheral reflexes are responsible for laryngeal tone and airway patency. The goal of this study was to elucidate the etiology of decreased laryngeal tone through evaluating the sensorimotor integrative function of the larynx. The secondary goal was to evaluate factors and medical comorbidities that contribute to the wide spectrum of symptoms and outcomes. STUDY DESIGN: Prospective and retrospective collection of evaluative data on infants with congenital laryngomalacia at two tertiary care pediatric referral centers. METHODS: Two hundred one infants with laryngomalacia were divided into three groups on the basis of disease severity (mild, moderate, severe). Patients were followed prospectively every 8 to 12 weeks until symptom resolution or loss to follow-up. Sensorimotor integrative function of the larynx was evaluated in 134 infants by laryngopharyngeal sensory testing (LPST) of the laryngeal adductor reflex (LAR) by delivering a duration- (50 ms) and intensity- (2.5-10 mm Hg) controlled air pulse to the aryepiglottic fold to induce the LAR. Medical records were retrospectively reviewed for medical comorbidities. RESULTS: The initial LPST was higher (P < .001) in infants with moderate (6.8 mm Hg) and severe disease (7.4 mm Hg) compared with those with mild disease (4.1 mm Hg). At 1, 3, and 6 months, infants with moderate and severe disease continued to have a higher LPST compared with those with mild disease. At 9 months, the LPST decreased in all subjects (3.1-3.5 mm Hg, P = .14), which also correlated with symptom resolution. Neurologic, genetic, and cardiac diseases were more common in infants with severe disease (P < .001). Gastroesophageal reflux disease (GERD) and feeding problems more common in those with moderate and severe disease (P < .001). Apgar scores were lower in those with severe disease (P < .001). Most symptoms resolved within 12 months of presentation. Those with GERD benefited from treatment. Supraglottoplasty resulted in few complications. Multiple comorbidities (>3) influenced the need for tracheotomy. CONCLUSIONS: Laryngeal tone and sensorimotor integrative function of the larynx is altered. The degree of alteration correlated with disease severity, indicating that factors that alter the peripheral and central reflexes of the LAR have a role in the etiology of signs and symptoms of laryngomalacia. GERD, neurologic disease, and low Apgar scores influenced disease severity and clinical course, explaining the spectrum of disease symptoms and outcomes. Sensorimotor integrative function improved as symptoms resolved.

[Value of modified Killian's method in diagnosis of congenital pyriform sinus fistula].

Objective: To investigate the feasibility and significance of modified Killian(MK) method in the clinical diagnosis of congenital pyriform sinus fistula(CPSF) by electronic laryngoscopy. Methods: The following examinations were performed for 30 suspected cases of CPSF, including the traditional electronic laryngoscopy, MK examination(modified Killian position+ head rotation+ the Valsalva maneuver), barium swallow X-ray(BSX) and CT , and a prospective comparison among them were done. Patients were divided into two groups according to their age: young age group(≤14 years old) and older age group (>14 years old). The results of MK examination from the patients were analyzed and the positive diagnostic rates (PDR) between groups were compared by using χ(2) tests. Results: Sinuses in 20 of 30 patients were depicted from pyriform sinus in BSX, and the PDR was 66.7%(20/30). The PRD of CT was 83.3%(25/30). The presence of air bubbles around the upper lobe of the thyroid gland or at the inferomedial edge of cricothyroid joints, morphological changes of thyroid grand as well as pseudo-fistula formation on lower neck were detected clearly on CT. Comparing to the traditional electronic laryngoscopy, the effect of exposing piriform fossa fistula by MK examination is significant(χ(2)=17.05, P<0.05), with the PDR of 13.3%(4/30) and 76.7%(23/30) respectively. Nevertheless, comparing to BSX and CT, there were no statistically significant differences in the effect to diagnose CPSF (χ(2)=0.31, χ(2)=0.10 respectively, P>0.05). The PDR of MK in older group is significantly higher than younger group(χ(2)=6.68, P<0.05). Conclusions: MK examination can clearly reveal the hypopharyngeal anatomical structure and detect the sinus of CPSF .It could be a safe, feasible, convenient and economical method as an application in preoperative diagnosis and follow-up examination of clinical suspected CPSF.

[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.

Congenital hairy polyp of the oropharynx presenting as an esophageal mass in a neonate, a case report and literature review.

PURPOSE: To review the literature of congenital hairy polyps and describe the clinical presentation, operative management, and histologic findings of a congenital hairy polyp arising from the palatopharyngeus muscle in a neonate with recurrent choking episodes. METHODS: Chart review of a 2-month-old male referred to a tertiary care pediatric hospital. RESULTS: We present a case of a 2-month-old male who presented to the emergency room with recurrent episodes of choking and vomiting. The patient was previously healthy with no prior medical or neonatal history. The parents noted a small fleshy mass in the patient's oropharynx that he would chew on and swallow after several minutes. However, on physical exam, there was no evidence of oropharyngeal mass. The patient did not have respiratory distress. Imaging revealed a 22×7×11mm oblong, fatty mass in the lower cervical and upper thoracic esophagus with a thin stalk extending proximally to the upper collapsed esophagus. Intraoperative recorded laryngoscopy revealed a pedunculated soft palate mass attached to the right superior palatopharyngeus muscle. Histopathology revealed ectodermal and mesodermal elements in a polypoid structure lined by keratinizing squamous epithelium with adnexal structures and central mature adipose tissue, consistent with congenital hairy polyp resembling an accessory tragus of the ear and branchial anomaly. At 6-week follow up, the patient was doing well and gaining weight appropriately with no further choking episodes. There was no evidence of velopharyngeal dysfunction on follow up exam. The surgical site was completely healed and there was no evidence of recurrence. DISCUSSION: Congenital hairy polyps of the naso- and oropharynx are rare but may present as airway or esophageal masses, causing respiratory distress or choking episodes in a pediatric patient. The pathologic findings of keratinizing squamous epithelium, adnexal structures, adipose and cartilage tissues resemble congenital accessory tragus and may be considered a branchial arch anomaly.

Systematic Review of Endoscopic Obliteration Techniques for Managing Congenital Piriform Fossa Sinus Tracts in Children.

BACKGROUND: Piriform fossa sinus tracts (PFSTs) are a recognized cause of recurrent deep neck infections in the pediatric population. Conventional management has historically required open resection, but over recent years minimally invasive endoscopic approaches to obliterate the pharyngeal opening of the sinus have been performed in many centers. However, there is a lack of clear evidence regarding the success rate and safety of these approaches. OBJECTIVE: To determine the success rate of endoscopic management of PFST through a systematic review of the existing literature. DATA SOURCES: MEDLINE (1964-2014) and bibliographies of identified papers. REVIEW METHODS: Two authors independently reviewed 170 abstracts and identified relevant studies for full-text review. Data were independently extracted from those studies, and the Oxford Centre for Evidence-Based Medicine guidelines were used to classify the level of evidence. RESULTS: Thirteen studies met the inclusion criteria, comprising a total of 84 patients. All included studies were evidence level 4 (case series). Various methods of obliterating the PFST were described: electrocautery (n = 39), laser (n = 19), trichloroacetic acid (n = 19), silver nitrate (n = 4), combination of silver nitrate and laser (n = 2), and fibrin glue (n = 1). The success rate for endoscopic management of PFST was 89.3% overall (90.5% in primary cases and 85.7% in revision cases). The only adverse event reported was temporary vocal cord immobility in 2.4% (n = 2) of cases. CONCLUSION: Endoscopic management of pediatric PFST appears to be safe and effective, as a primary option and for revision after open surgery.

Hairy Polyp of the Nasopharynx Arising from the Eustachian Tube.

Hairy polyps of the nasopharynx display characteristic radiological imaging findings, including the presence of fat in the polypoid mass. Furthermore, diagnostic imaging is useful for delineating the site of origin of these lesions, which can facilitate surgical planning. For instance hairy polyps that arise from the right Eustachian tube can be amputated via a trans-nasal approach with endoscopy, but may necessitate a two stage approach in order to avoid injury to critical structures, such as the internal carotid artery. On histology, hairy polyps comprise an outer keratinizing squamous epithelium with adnexal tissue, including hair follicles, and central fibroadipose and cartilaginous tissue. These features are exemplified in this sine qua non radiology-pathology correlation article.

[Fourth branchial cleft deformity with skin orifice: a series of 10 cases].

Objective: To report rare cases of congenital neck cutaneous sinus with an orifice near the sternoclavicular joint and to investigate their origins and managements. Methods: A total of ten patients with congenital neck cutaneous sinus having an orifice near the sternoclavicular joint treated in the Guangdong General Hospital from January 2010 to June 2015 were retrospectively analyzed. Results: There four boys and six girls, aging from 11 months to 96 months with an average of 33.4 months, and they had a common feature showing a congenital cutaneous sinus with an orifice near sternoclavicular joint. Discharge of pus from the orifice or abscess formation was commonly seen soon after infection. With bacteriological study, staphylococcus aureus was positive in five cases and klebsiella pneumonia in a case. Another orifice of fistula/sinus was not depicted in pyriform with barium swallow X-ray in five cases Ultrasound studies of three cases demonstrated anechoic (i.e., nearly black) and solid-cystic lesion near sternoclavicular joint with posterior acoustic enhancement. Magnetic resonance imaging (MRI) showed isointensity of the lesion on T1 and T2 weighted images with heterogeneous enhancement and a close relationship with sternoclavicular joint. All patients underwent laryngoscopic examination, which showed no orifice of sinus in pyriform at same side. Surgical resection of fistula/sinus was performed in all cases. The lengths of the fistula varied from 5 mm to 22 mm with an average of 11 mm. Postoperative pathological examination showed all specimens were accordance with fistula. No complications were noticed. Recurrence was not observed in the cases by following-up of 6 months to 70 months (median: 33 months). Conclusion: Congenital neck cutaneous sinus with orifice near the sternoclavicular joint maybe a special clinical phenotype of the fourth branchial cleft sinus with skin orifice in cervicothoracic junction. Differential diagnoses between low cervical diseases are required. The curative treatment is a complete excision during inflammatory quiescent period.