Journal of Pediatric Surgery 49 (2014) 455–459
Contents lists available at ScienceDirect
Journal of Pediatric Surgery journal homepage: www.elsevier.com/locate/jpedsurg
Diagnosis and management of pyriform sinus fistula: Experience in 48 cases☆ Qingfeng Sheng a, Zhibao Lv a,⁎, Xianmin Xiao b, Shan Zheng b, Yimin Huang a, Xiong Huang a, Hui Li a, Yibo Wu a, Kuiran Dong b, Jiangbin Liu b a b
Department of General Surgery, Children's Hospital of Shanghai, Shanghai Jiao Tong University, Shanghai 200040, P.R. China Department of Surgery, Children's Hospital of Fudan University, Shanghai 201102, P.R. China
a r t i c l e
i n f o
Article history: Received 31 May 2013 Received in revised form 1 July 2013 Accepted 20 July 2013 Key words: Pyriform sinus fistula Endoscopy Barium esophagography
a b s t r a c t Purpose: Pyriform sinus fistula (PSF) is often overlooked, and presents diagnostic and management challenge. The aim of this study was to highlight the value of intraoperative endoscopy-assisted intubation or methylene blue injection through the internal opening as a guide in searching for the fistula. Methods: The charts of 48 patients diagnosed with PSF during January 1990 until January 2013 were retrospectively reviewed. The records were analyzed for sex, side of lesion, age at onset/diagnosis, initial presentations, diagnostic methods, microbiologic cultures, pathologic findings, treatments and outcomes. Results: There were 22 males and 26 females, with a median age at onset and diagnosis of 2 years (range, 8 months to 9 years) and 4 years (range, 12 months to 13 years), respectively. The lesions were predominantly left sided (93.7%). The most common presentation was neck abscess (62.5%). Other presentations were acute suppurative thyroiditis/thyroid abscess (7), neck mass with or without dyspnea (9), and thyroid nodule (2). Barium esophagography showed the sinus tract in 100% cases. The positive predictive value of other modalities was oral-contrast CT 88.9%, intravenous contrast-enhanced CT 53.8%, noncontrast CT 33.3%, and sonography 7.9%. Thyroid function were reported normal in most tested cases (14/ 15, 93.3%). The fistula tract was lined with pseudostratified squamous epithelium or ciliated columnar epithelium, often associated with inflammatory changes. Bacteria cultured from the discharge were found to be oral flora. Partial thyroidectomy was operated on 11 cases. Two patients (2/8, 25%) who underwent open surgery without endoscopic assistance exhibited recurrence, while no recurrence was noted in children with the help of intraoperative endoscopy. Postoperative results were good in majority (93.7%). Conclusion: PSF should be considered in any children with repeated neck or thyroid infection/mass. The combination of barium esophagography, CT scan and ultrasound is useful to establish the diagnosis. Intraoperative endoscopy-assisted intubation or methylene blue injection through the internal opening as a guide can facilitate identification of the tract during dissection. © 2014 Elsevier Inc. All rights reserved.
Pyriform sinus fistula (PSF), which originates from the failure obliteration of the third or fourth pharyngeal pouches, is rare and often overlooked [1–3]. Misdiagnosis exposes the patients to the risk of repeated infection and inadequate management. During the past two decades, we have cared for 48 patients with PSF who underwent surgical management, to our knowledge, the largest series of its kind reported to date. The aim of this study was to highlight the value of intraoperative endoscopy-assisted intubation or methylene blue injection through the internal opening as a guide in searching for the fistula, especially in recurrent cases.
☆ Conflict of interest: The authors declare no conflict of interest. ⁎ Corresponding author. Department of General Surgery, Children's Hospital of Shanghai, Shanghai Jiao Tong University, Shanghai 200040, P.R. China. Tel./fax: +86 21 62790494. E-mail addresses: [email protected], [email protected] (Z. Lv). 0022-3468/$ – see front matter © 2014 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpedsurg.2013.07.008
1. Materials and methods We retrospectively reviewed the charts of 48 cases with PSF from Children's Hospital of Shanghai Jiao Tong University and Children's Hospital of Fudan University during January 1990 until January 2013. Other six patients diagnosed with PSF in the same period were excluded because the parents refused definitive surgery. The files were analyzed for sex, side of lesion, age at onset/diagnosis, initial presentations, diagnostic methods, thyroid functions, microbiologic cultures, pathologic findings, treatments and outcomes. Ethical approval was obtained from the Ethics Boards of the Children's Hospital of Shanghai Jiao Tong University and Children's Hospital of Fudan University. Eight cases who were admitted from January 1990 to December 1998 have been reported in a previous publication in Chinese [4]. We performed intraoperative endoscopy-assisted surgical management since January 1999. The patients and their families were contacted by letter, phone, or interview in our clinics by an experienced surgeon (Y. Wu).
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Table 1 Features of 48 cases with pyriform sinus fistula. Features
Values
Male, n (%) Age at onset, median (range) Age at diagnosis, median (range) Time from onset to diagnosis, median (range) Side of lesion, n (%) Left Right Bilateral Initial presentations, n (%) Neck abscess AST/thyroid abscess Neck mass Neck mass with dyspnea Thyroid nodule Pharyngeal anomaly, n (%) Third Fourth Follow-up, median (range)
22 (45.8) 2 years (8 months–9 years) 4 years (12 months–13 years) 1 years (0–9 years) 45 (93.7) 1 (2.1) 2 (4.2) 30 (62.5) 7 (14.6) 7 (14.6) 2 (4.2) 2 (4.2) 10 (20.8) 38 (79.2) 4 years (4 months–14 years)
The criteria we used to identify third from fourth pharyngeal arch anomalies are the location in the pyriform sinus (base, apex), the course of the sinus tract (pass through the thyrohyoid membrane and superiorly toward the hypoglossal nerve, cross the cricothyroid membrane and inferiorly toward the aorta on the left/ subclavian artery on the right), and the relation to the superior laryngeal nerve (cranial, caudal) [1,2,5]. The presence of thymic tissue, thyroid or parathyroid tissue was not specific, so it was not used as a selection criterion. Data were recorded as numeric or nominative variables. Statistical analysis was performed using the SPSS 17.0 software package. The recurrent rate after definitive surgery was compared using the Fisher's exact test, with α set at 0.05, two-tailed.
2 years later because no evidence of a sinus tract was found during surgery. intraoperative endoscopy-assisted ligation and excision of the fistula was performed on these two patients after an attempt of incision and drainage. Other 12 patients who had open surgery as initial or secondary procedure were referred from outside hospitals. There were 22 males and 26 females. The median age at onset and diagnosis was 2 years (range, 8 months to 9 years) and 4 years (range, 12 months to 13 years), respectively. The great majority of cases occurred on the left side (45/48, 93.7%). One patient (1/48, 2.1%) presented with a right-sided neck abscess. Two patients (2/48, 4.2%) were admitted to our hospital because of a left-sided lesion (thyroid abscess and neck abscess) but later identified as bilateral PSF by barium esophagography. The most common presentation was neck abscess (30/48, 62.5%). Other presentations such as acute suppurative thyroiditis/thyroid abscess, neck mass with or without dyspnea, and thyroid nodule were reported in 7, 9 and 2 cases, respectively. Based on the criteria listed above, there were 38 cases of PSF originated from fourth pharyngeal anomaly, and 10 from third pharyngeal anomaly (Table 1).
2.2. Diagnostic methods All patients underwent barium esophagography after resolution of the acute infection with a perfect positive predictive value (100%, Fig. 1). Other methods were used and positive predictive values (ppv) were as follows: computed tomography (CT) scan with oral contrast in 18.8% of cases (ppv, 88.9%, Fig. 2), intravenous contrast-enhanced CT scan in 54.2% (ppv, 53.8%), CT scan without contrast in 12.5% (ppv, 33.3%), and ultrasound examination in 79.2% (ppv, 7.9%). Thyroid scan, x-ray film of the neck, and fine-needle aspiration had no contribution to the diagnosis (Table 2).
2.3. Findings 2. Results 2.1. Clinical features The summary of patients was depicted in Table 1. Eight patients underwent open surgery without endoscopic assistance from January 1990 to December 1998. Two of them developed recurrent infection
Thyroid function tests were reported in 15 cases. Fourteen (14/15, 93.3%) children displayed normal thyroid functions. Only one patient showed slight hyperthyroidism and did not require medical treatment. Histopathology showed evidence of a tract with pseudostratified squamous epithelium in 37 cases (77.1%) and ciliated column epithelium in 11 cases (22.9%), always associated with inflammatory
Fig. 1. Barium esophagography shows pyriform sinus fistula (arrow) on the left side (A) and on both sides (B). Anteroposterior view.
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Fig. 2. (A) CT scan of the neck shows an air density (arrow) within the sinus tract. CT images after ingestion of dilute barium reveal the contrast entering the fistulous tract on the left side (arrow). (B) Axial scan. (C) Sagittal scan. (D) Coronal scan.
and fibrotic changes (Fig. 3). Thyroid tissues were demonstrated in 11 cases (22.9%). Organisms cultured from the specimens were reported in 12 cases. They were found to be oral flora such as Streptococcus sp (two cases), Staphylococcus sp (three cases), Bacteroides sp (two cases), Escherichia Table 2 Summary of diagnostic methods, treatments and outcomes. Features Diagnostic methods, n (%), positive predictive value Barium esophagography CT scan (oral contrast) CT scan (intravenous contrast-enhanced) CT scan (noncontrast) Ultrasound Thyroid scan X-ray film of the neck Fine-needle aspiration Treatments Recurrent surgery, n (%) I&D as initial procedure, n (%) I&D as secondary procedure, n (%) No. of attempts, median (range) Open surgery as initial procedure, n (%) Open surgery as secondary procedure, n (%) No. of attempts, median (range) Partial thyroid surgery, n (%) Outcomes Complications, n (%) Recurrence after open surgery, n (%) Recurrence after endoscopy-assisted open surgery, n (%) Comparison of recurrent rate
Values 48 (100), 100% 9 (18.8), 88.9% 26 (54.2), 53.8% 6 (12.5), 33.3% 38 (79.2), 7.9% 9 (18.8), 0% 2 (4.2), 0% 1 (2.1), 0%
coli (one case), and mixed (three cases). Negative culture was described in one case. 2.4. Treatments and outcomes Primary treatments performed were incision and drainage (I&D) in 37 cases (77.1%), open surgery in 2 cases (4.2%) and conservative therapy in 3 cases (6.3%). Repeated I&D, after initially failed operations (I&D or open) was performed in 30 cases. The number of I&D attempts ranged from 1 to 8 (median, 2). Twelve patients received open surgery after the failure of initial procedure but neck
39 (81.3) 37 (77.1) 2 (4.2) 2 (1–8) 2 (4.2) 12 (25) 1 (1–2) 11 (22.9) 3 (6.3)a 2 (25)b 0 (0) P = 0.023
a Three patients developed postoperative complications: wound infection, temporary vocal fold motion impairment, and left-sided Horner syndrome, respectively. b These two patients underwent open surgery without endoscopic assistance in our hospital and recurred within 2 years.
Fig. 3. Histopathological examination demonstrates the tract wall formed by pseudostratified squamous epithelium (arrow), with chronic inflammatory cells in the surrounding tissue. Thyroid tissue is also reported (arrowheads; H&E, original magnification 100×).
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infection still recurred. Partial thyroidectomy was performed on 11 cases because the ipsilateral thyroid gland was involved (Table 2). Between 1990 and 1998, we performed open neck surgery without endoscopic assistance in eight cases with a median followup of 10.5 years (range, 4–14 years). Two (25%) of them exhibited recurrence within 2 years (Table 2). However, among the 42 cases who underwent definite ligation and resection of the fistula with the help of intraoperative endoscopy-assisted intubation or methylene blue injection, no recurrence was noted during a median follow-up of 3 years (range, 4 months–14 years). A difference of recurrent rate between these two groups became statistically significant (P = 0.023). Postoperative course was uneventful in most cases (45/48, 93.7%). However, three patients had complications after definitive open surgery. Of these, one girl developed wound infection and received dressing change and oral antibiotics therapy, one boy had temporary vocal fold motion impairment, and one girl exhibited left ptosis and miosis (Horner syndrome) which did not recover at 4-month followup (Table 2).
3. Discussion The pyriform sinus is a true space of the larynx bordered by the laryngeal cartilage and the hypopharynx. Third and fourth pharyngeal arch anomalies are reported to be accounting for 3%–10% of all pharyngeal lesions [3]. In certain cases, the embryologic origin of the PSF is difficult to appreciate because of postinfectious fibrosis. These lesions can present, in theory, as a cleft sinus, a pouch sinus, a cyst, or a fistula. However, a complete congenital third or fourth pharyngeal fistula tract has never been demonstrated. These pharyngeal arch anomalies often present with recurrent anterior neck infection, mainly on the left side, often following episodes of upper airway infection. The sinus tract acts as the pathway for the infection spreading. The predominance of left-sided lesions was also noted in previous reports [6–9]. James et al. [9] and Thomas et al. [10] suggested that one possible explanation for this discrepancy was that these sinuses arise from the persistence of a patent thymopharyngeal duct. However, the exact cause of this tendency is still unclear. The frequent delay in accurate diagnosis was reported in the literature [6,7,9]. The median time from the onset to diagnosis in this study is 1 year, with a range of 0 to 9 years. All suspected cases of neck/thyroid infection or mass in infants and children, especially on the left side, should undergo barium esophagography, which may illustrate pharyngeal anomaly.
The diagnosis of PSF is usually established by a barium esophagography [1,2,10–12]. We performed the barium swallow study often in the noninfected state to reduce the false-negative results. Thin contrast can improve the accuracy. Multiple diagnostic investigations should be used to ensure the proper diagnosis. Ultrasound is useful in showing the relationship of the mass with thyroid and the presence of gas within the lesion [10–12], but with a low positive predictive value (7.9%). We found that CT scan with oral contrast was also a convenient tool to delineate the tract and its surrounding structures [13]. An intravenous contrast-enhanced CT study has been considered a valuable modality in demonstrating the site and extent of the lesion. Other authors advocated that magnetic resonance imaging (MRI) and pharyngoscopy/(micro)laryngoscopy are essential investigations of choice [8–11]. Technetium scan of the thyroid showed a decreased uptake in the upper portion, but had no contribution to the diagnosis. Other modalities, such as x-ray film of the neck and fine-needle aspiration, cannot establish an unequivocal diagnosis. Incision and drainage was frequently performed, but with a high failure rate. Clear identification and exact resection of the fistula, after resolution of the acute infection, are vital to the success of surgical management. Identification of the fistula tract during surgery can be quite difficult in children with repeated infections and surgical drainages. Recurrence was reported in two patients who underwent open surgery without endoscopic assistance in our hospital because no definite fistula tract had been dissected. In order to facilitate localization, we used intraoperative endoscopy-assisted intubation or methylene blue injection through the internal orifice as a guide since 1999. To begin with, meticulous exploration of the neck was performed after mobilizing the ipsilateral thyroid. The sinus tract could penetrate the thyroid cartilage near the inferior cornu, the inferior pharyngeal constrictor muscle, or the cricothyroid membrane when it emerged from the larynx. Then, a 3F catheter was inserted into the tract from the internal opening and left it in the loci. In some cases, the catheter was found could easily slip out of the position and then a volume of about 0.5 ml methylene blue was injected into the sinus instead as an indicator. Incision of the inferior pharyngeal constrictor muscle and exposure of the inferior cornu of thyroid cartilage might simplify the dissection of the proximal part of the fistula. The tract was clearly demonstrated in the operative field in all 42 cases (Fig. 4). The cannulation of the tract with a guide wire or light was also reported useful by other researchers [14,15]. It should be reminded injury to the recurrent or superior laryngeal nerves and neighboring great vessels should be minimized [16], and accidental removal of important immune organ thymus must be avoided especially in children younger than 5 years. The fistula should be
Fig. 4. Operative view. Clear identification of the fistulous tract (arrow) is shown after intraoperative endoscopy-assisted methylene blue injection through the internal opening as a guide during ligation (A) and resection (B) of the distal tract.
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ligated and excised as high as possible. If the ipsilateral thyroid is involved, partial thyroidectomy is indicated. The fistula, surrounding fibrotic tissues, and a portion of thyroid gland were excised en bloc. However, some cases have been reported in which the visualization of the fistula opening failed with assistance of endoscopy [7,10]. Then the verification of the tract should be confirmed by histopathology. Recently, endoscopic obliteration of the sinus alone using laser, chemo (trichloroacetic acid, silver nitrate, or fibrin) or electrocautery represents a minimally invasive technique [17–22]. Although vocal fold immobility and recurrence have been noted in patients after chemocauterizaton [23,24], this new approach is a viable alternative to open excision. The postoperative results were good in majority of cases. However, there were still three patients (6.3%) who developed unexpected complications. The vocal fold motion impairment is always temporary and the function can restore within weeks or months [8,24,25]. However, left-sided Horner syndrome, with the symptoms of ptosis and miosis, was noted in one child 2 days after the operation. Also, the symptoms did not improve after 4-month follow-up. Hence, it should be recognized that there is a risk of iatrogenic sympathetic nervous system injury during neck exploration. Other complications such as facial nerve paralysis and salivary fistula were also reported in the literature [1,2,26]. In summary, pyriform sinus fistula is not as rare as previously reported, and the diagnosis should be considered in any children with repeated neck or thyroid infection/mass. Imaging studies, such as barium esophagography, CT scan (with oral contrast or intravenous contrast), and pharyngoscopy/laryngoscopy advocated by other investigators, are essential to establish the diagnosis. Intraoperative endoscopy-assisted intubation or methylene blue injection through the internal opening as a guide can facilitate the exhibition of the tract during dissection. The exact ligation and resection of fistula is important for preventing recurrence. References [1] Nicoucar K, Giger R, Pope Jr HG, et al. Management of congenital fourth branchial arch anomalies: a review and analysis of published cases. J Pediatr Surg 2009;44: 1432–9. [2] Nicoucar K, Giger R, Jaecklin T, et al. Management of congenital third branchial arch anomalies: a systematic review. Otolaryngol Head Neck Surg 2010;142: 21–28e2. [3] Goff CJ, Allred C, Glade RS. Current management of congenital branchial cleft cysts, sinuses, and fistulae. Curr Opin Otolaryngol Head Neck Surg 2012;20:533–9.
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[4] Lv Z, Xiao X, Zheng S, et al. Gastroscopic assistant operation for children with pyriform sinus fistula (in Chinese). Chin J Pediatr Surg 2005;26:17–9. [5] Franciosi JP, Sell LL, Conley SF, et al. Pyriform sinus malformations: a cadaveric representation. J Pediatr Surg 2002;37:533–8. [6] Makino S, Tsuchida Y, Yoshioka H, et al. The endoscopic and surgical management of pyriform sinus fistulae in infants and children. J Pediatr Surg 1986;21:398–401. [7] Liberman M, Kay S, Emil S, et al. Ten years of experience with third and fourth branchial remnants. J Pediatr Surg 2002;37:685–90. [8] Garrel R, Jouzdani E, Gardiner Q, et al. Fourth branchial pouch sinus: from diagnosis to treatment. Otolaryngol Head Neck Surg 2006;134:157–63. [9] James A, Stewart C, Warrick P, et al. Branchial sinus of the piriform fossa: reappraisal of third and fourth branchial anomalies. Laryngoscope 2007;117: 1920–4. [10] Thomas B, Shroff M, Forte V, et al. Revisiting imaging features and the embryologic basis of third and fourth branchial anomalies. AJNR Am J Neuroradiol 2010;31: 755–60. [11] Wang HK, Tiu CM, Chou YH, et al. Imaging studies of pyriform sinus fistula. Pediatr Radiol 2003;33:328–33. [12] Gan YU, Lam SL. Imaging findings in acute neck infection due to pyriform sinus fistula. Ann Acad Med Singapore 2004;33:636–40. [13] Stone ME, Link DT, Egelhoff JC, et al. A new role for computed tomography in the diagnosis and treatment of pyriform sinus fistula. Am J Otolaryngol 2000;21:323–5. [14] Kubota M, Suita S, Kamimura T, et al. Surgical strategy for the treatment of pyriform sinus fistula. J Pediatr Surg 1997;32:34–7. [15] Ukiyama E, Endo M, Yoshida F, et al. Light guided procedure for congenital pyriform sinus fistula; new and simple procedure for impalpable fistula. Pediatr Surg Int 2007;23:1241–3. [16] Mantle BA, Otteson TD, Chi DH. Fourth branchial cleft sinus: relationship to superior and recurrent laryngeal nerves. Am J Otolaryngol 2008;29:198–200. [17] Cigliano B, Cipolletta L, Baltogiannis N, et al. Endoscopic fibrin sealing of congenital pyriform sinus fistula. Surg Endosc 2004;18:554–6. [18] Pereira KD, Smith SL. Endoscopic chemical cautery of piriform sinus tracts: a safe new technique. Int J Pediatr Otorhinolaryngol 2008;72:185–8. [19] Chen EY, Inglis AF, Ou H, et al. Endoscopic electrocauterization of pyriform fossa sinus tracts as definitive treatment. Int J Pediatr Otorhinolaryngol 2009;73: 1151–6. [20] Leboulanger N, Ruellan K, Nevoux J, et al. Neonatal vs delayed-onset fourth branchial pouch anomalies: therapeutic implications. Arch Otolaryngol Head Neck Surg 2010;136:885–90. [21] Watson GJ, Nichani JR, Rothera MP, et al. Case series: endoscopic management of fourth branchial arch anomalies. Int J Pediatr Otorhinolaryngol 2013;77: 766–9. [22] Cha W, Cho SW, Hah JH, et al. Chemocauterization of the internal opening with trichloroacetic acid as first-line treatment for pyriform sinus fistula. Head Neck 2013;35:431–5. [23] Zhang J, Huang S, Li H, et al. Relapsing suppurative neck abscess after chemocauterization of pyriform sinus fistula. Clin Imaging 2012;36:826–8. [24] Park JH, Jung YH, Sung MW, et al. Temporary vocal fold immobility after chemocauterization of the pyriform sinus fistula opening with trichloroacetic acid. Laryngoscope 2013;123:410–3. [25] Ford GR, Balakrishnan A, Evans JN, et al. Branchial cleft and pouch anomalies. J Laryngol Otol 1992;106:137–43. [26] Lin JN, Wang KL. Persistent third branchial apparatus. J Pediatr Surg 1991;26: 663–5.
Contents lists available at ScienceDirect
Journal of Pediatric Surgery journal homepage: www.elsevier.com/locate/jpedsurg
Diagnosis and management of pyriform sinus fistula: Experience in 48 cases☆ Qingfeng Sheng a, Zhibao Lv a,⁎, Xianmin Xiao b, Shan Zheng b, Yimin Huang a, Xiong Huang a, Hui Li a, Yibo Wu a, Kuiran Dong b, Jiangbin Liu b a b
Department of General Surgery, Children's Hospital of Shanghai, Shanghai Jiao Tong University, Shanghai 200040, P.R. China Department of Surgery, Children's Hospital of Fudan University, Shanghai 201102, P.R. China
a r t i c l e
i n f o
Article history: Received 31 May 2013 Received in revised form 1 July 2013 Accepted 20 July 2013 Key words: Pyriform sinus fistula Endoscopy Barium esophagography
a b s t r a c t Purpose: Pyriform sinus fistula (PSF) is often overlooked, and presents diagnostic and management challenge. The aim of this study was to highlight the value of intraoperative endoscopy-assisted intubation or methylene blue injection through the internal opening as a guide in searching for the fistula. Methods: The charts of 48 patients diagnosed with PSF during January 1990 until January 2013 were retrospectively reviewed. The records were analyzed for sex, side of lesion, age at onset/diagnosis, initial presentations, diagnostic methods, microbiologic cultures, pathologic findings, treatments and outcomes. Results: There were 22 males and 26 females, with a median age at onset and diagnosis of 2 years (range, 8 months to 9 years) and 4 years (range, 12 months to 13 years), respectively. The lesions were predominantly left sided (93.7%). The most common presentation was neck abscess (62.5%). Other presentations were acute suppurative thyroiditis/thyroid abscess (7), neck mass with or without dyspnea (9), and thyroid nodule (2). Barium esophagography showed the sinus tract in 100% cases. The positive predictive value of other modalities was oral-contrast CT 88.9%, intravenous contrast-enhanced CT 53.8%, noncontrast CT 33.3%, and sonography 7.9%. Thyroid function were reported normal in most tested cases (14/ 15, 93.3%). The fistula tract was lined with pseudostratified squamous epithelium or ciliated columnar epithelium, often associated with inflammatory changes. Bacteria cultured from the discharge were found to be oral flora. Partial thyroidectomy was operated on 11 cases. Two patients (2/8, 25%) who underwent open surgery without endoscopic assistance exhibited recurrence, while no recurrence was noted in children with the help of intraoperative endoscopy. Postoperative results were good in majority (93.7%). Conclusion: PSF should be considered in any children with repeated neck or thyroid infection/mass. The combination of barium esophagography, CT scan and ultrasound is useful to establish the diagnosis. Intraoperative endoscopy-assisted intubation or methylene blue injection through the internal opening as a guide can facilitate identification of the tract during dissection. © 2014 Elsevier Inc. All rights reserved.
Pyriform sinus fistula (PSF), which originates from the failure obliteration of the third or fourth pharyngeal pouches, is rare and often overlooked [1–3]. Misdiagnosis exposes the patients to the risk of repeated infection and inadequate management. During the past two decades, we have cared for 48 patients with PSF who underwent surgical management, to our knowledge, the largest series of its kind reported to date. The aim of this study was to highlight the value of intraoperative endoscopy-assisted intubation or methylene blue injection through the internal opening as a guide in searching for the fistula, especially in recurrent cases.
☆ Conflict of interest: The authors declare no conflict of interest. ⁎ Corresponding author. Department of General Surgery, Children's Hospital of Shanghai, Shanghai Jiao Tong University, Shanghai 200040, P.R. China. Tel./fax: +86 21 62790494. E-mail addresses: [email protected], [email protected] (Z. Lv). 0022-3468/$ – see front matter © 2014 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpedsurg.2013.07.008
1. Materials and methods We retrospectively reviewed the charts of 48 cases with PSF from Children's Hospital of Shanghai Jiao Tong University and Children's Hospital of Fudan University during January 1990 until January 2013. Other six patients diagnosed with PSF in the same period were excluded because the parents refused definitive surgery. The files were analyzed for sex, side of lesion, age at onset/diagnosis, initial presentations, diagnostic methods, thyroid functions, microbiologic cultures, pathologic findings, treatments and outcomes. Ethical approval was obtained from the Ethics Boards of the Children's Hospital of Shanghai Jiao Tong University and Children's Hospital of Fudan University. Eight cases who were admitted from January 1990 to December 1998 have been reported in a previous publication in Chinese [4]. We performed intraoperative endoscopy-assisted surgical management since January 1999. The patients and their families were contacted by letter, phone, or interview in our clinics by an experienced surgeon (Y. Wu).
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Table 1 Features of 48 cases with pyriform sinus fistula. Features
Values
Male, n (%) Age at onset, median (range) Age at diagnosis, median (range) Time from onset to diagnosis, median (range) Side of lesion, n (%) Left Right Bilateral Initial presentations, n (%) Neck abscess AST/thyroid abscess Neck mass Neck mass with dyspnea Thyroid nodule Pharyngeal anomaly, n (%) Third Fourth Follow-up, median (range)
22 (45.8) 2 years (8 months–9 years) 4 years (12 months–13 years) 1 years (0–9 years) 45 (93.7) 1 (2.1) 2 (4.2) 30 (62.5) 7 (14.6) 7 (14.6) 2 (4.2) 2 (4.2) 10 (20.8) 38 (79.2) 4 years (4 months–14 years)
The criteria we used to identify third from fourth pharyngeal arch anomalies are the location in the pyriform sinus (base, apex), the course of the sinus tract (pass through the thyrohyoid membrane and superiorly toward the hypoglossal nerve, cross the cricothyroid membrane and inferiorly toward the aorta on the left/ subclavian artery on the right), and the relation to the superior laryngeal nerve (cranial, caudal) [1,2,5]. The presence of thymic tissue, thyroid or parathyroid tissue was not specific, so it was not used as a selection criterion. Data were recorded as numeric or nominative variables. Statistical analysis was performed using the SPSS 17.0 software package. The recurrent rate after definitive surgery was compared using the Fisher's exact test, with α set at 0.05, two-tailed.
2 years later because no evidence of a sinus tract was found during surgery. intraoperative endoscopy-assisted ligation and excision of the fistula was performed on these two patients after an attempt of incision and drainage. Other 12 patients who had open surgery as initial or secondary procedure were referred from outside hospitals. There were 22 males and 26 females. The median age at onset and diagnosis was 2 years (range, 8 months to 9 years) and 4 years (range, 12 months to 13 years), respectively. The great majority of cases occurred on the left side (45/48, 93.7%). One patient (1/48, 2.1%) presented with a right-sided neck abscess. Two patients (2/48, 4.2%) were admitted to our hospital because of a left-sided lesion (thyroid abscess and neck abscess) but later identified as bilateral PSF by barium esophagography. The most common presentation was neck abscess (30/48, 62.5%). Other presentations such as acute suppurative thyroiditis/thyroid abscess, neck mass with or without dyspnea, and thyroid nodule were reported in 7, 9 and 2 cases, respectively. Based on the criteria listed above, there were 38 cases of PSF originated from fourth pharyngeal anomaly, and 10 from third pharyngeal anomaly (Table 1).
2.2. Diagnostic methods All patients underwent barium esophagography after resolution of the acute infection with a perfect positive predictive value (100%, Fig. 1). Other methods were used and positive predictive values (ppv) were as follows: computed tomography (CT) scan with oral contrast in 18.8% of cases (ppv, 88.9%, Fig. 2), intravenous contrast-enhanced CT scan in 54.2% (ppv, 53.8%), CT scan without contrast in 12.5% (ppv, 33.3%), and ultrasound examination in 79.2% (ppv, 7.9%). Thyroid scan, x-ray film of the neck, and fine-needle aspiration had no contribution to the diagnosis (Table 2).
2.3. Findings 2. Results 2.1. Clinical features The summary of patients was depicted in Table 1. Eight patients underwent open surgery without endoscopic assistance from January 1990 to December 1998. Two of them developed recurrent infection
Thyroid function tests were reported in 15 cases. Fourteen (14/15, 93.3%) children displayed normal thyroid functions. Only one patient showed slight hyperthyroidism and did not require medical treatment. Histopathology showed evidence of a tract with pseudostratified squamous epithelium in 37 cases (77.1%) and ciliated column epithelium in 11 cases (22.9%), always associated with inflammatory
Fig. 1. Barium esophagography shows pyriform sinus fistula (arrow) on the left side (A) and on both sides (B). Anteroposterior view.
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Fig. 2. (A) CT scan of the neck shows an air density (arrow) within the sinus tract. CT images after ingestion of dilute barium reveal the contrast entering the fistulous tract on the left side (arrow). (B) Axial scan. (C) Sagittal scan. (D) Coronal scan.
and fibrotic changes (Fig. 3). Thyroid tissues were demonstrated in 11 cases (22.9%). Organisms cultured from the specimens were reported in 12 cases. They were found to be oral flora such as Streptococcus sp (two cases), Staphylococcus sp (three cases), Bacteroides sp (two cases), Escherichia Table 2 Summary of diagnostic methods, treatments and outcomes. Features Diagnostic methods, n (%), positive predictive value Barium esophagography CT scan (oral contrast) CT scan (intravenous contrast-enhanced) CT scan (noncontrast) Ultrasound Thyroid scan X-ray film of the neck Fine-needle aspiration Treatments Recurrent surgery, n (%) I&D as initial procedure, n (%) I&D as secondary procedure, n (%) No. of attempts, median (range) Open surgery as initial procedure, n (%) Open surgery as secondary procedure, n (%) No. of attempts, median (range) Partial thyroid surgery, n (%) Outcomes Complications, n (%) Recurrence after open surgery, n (%) Recurrence after endoscopy-assisted open surgery, n (%) Comparison of recurrent rate
Values 48 (100), 100% 9 (18.8), 88.9% 26 (54.2), 53.8% 6 (12.5), 33.3% 38 (79.2), 7.9% 9 (18.8), 0% 2 (4.2), 0% 1 (2.1), 0%
coli (one case), and mixed (three cases). Negative culture was described in one case. 2.4. Treatments and outcomes Primary treatments performed were incision and drainage (I&D) in 37 cases (77.1%), open surgery in 2 cases (4.2%) and conservative therapy in 3 cases (6.3%). Repeated I&D, after initially failed operations (I&D or open) was performed in 30 cases. The number of I&D attempts ranged from 1 to 8 (median, 2). Twelve patients received open surgery after the failure of initial procedure but neck
39 (81.3) 37 (77.1) 2 (4.2) 2 (1–8) 2 (4.2) 12 (25) 1 (1–2) 11 (22.9) 3 (6.3)a 2 (25)b 0 (0) P = 0.023
a Three patients developed postoperative complications: wound infection, temporary vocal fold motion impairment, and left-sided Horner syndrome, respectively. b These two patients underwent open surgery without endoscopic assistance in our hospital and recurred within 2 years.
Fig. 3. Histopathological examination demonstrates the tract wall formed by pseudostratified squamous epithelium (arrow), with chronic inflammatory cells in the surrounding tissue. Thyroid tissue is also reported (arrowheads; H&E, original magnification 100×).
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infection still recurred. Partial thyroidectomy was performed on 11 cases because the ipsilateral thyroid gland was involved (Table 2). Between 1990 and 1998, we performed open neck surgery without endoscopic assistance in eight cases with a median followup of 10.5 years (range, 4–14 years). Two (25%) of them exhibited recurrence within 2 years (Table 2). However, among the 42 cases who underwent definite ligation and resection of the fistula with the help of intraoperative endoscopy-assisted intubation or methylene blue injection, no recurrence was noted during a median follow-up of 3 years (range, 4 months–14 years). A difference of recurrent rate between these two groups became statistically significant (P = 0.023). Postoperative course was uneventful in most cases (45/48, 93.7%). However, three patients had complications after definitive open surgery. Of these, one girl developed wound infection and received dressing change and oral antibiotics therapy, one boy had temporary vocal fold motion impairment, and one girl exhibited left ptosis and miosis (Horner syndrome) which did not recover at 4-month followup (Table 2).
3. Discussion The pyriform sinus is a true space of the larynx bordered by the laryngeal cartilage and the hypopharynx. Third and fourth pharyngeal arch anomalies are reported to be accounting for 3%–10% of all pharyngeal lesions [3]. In certain cases, the embryologic origin of the PSF is difficult to appreciate because of postinfectious fibrosis. These lesions can present, in theory, as a cleft sinus, a pouch sinus, a cyst, or a fistula. However, a complete congenital third or fourth pharyngeal fistula tract has never been demonstrated. These pharyngeal arch anomalies often present with recurrent anterior neck infection, mainly on the left side, often following episodes of upper airway infection. The sinus tract acts as the pathway for the infection spreading. The predominance of left-sided lesions was also noted in previous reports [6–9]. James et al. [9] and Thomas et al. [10] suggested that one possible explanation for this discrepancy was that these sinuses arise from the persistence of a patent thymopharyngeal duct. However, the exact cause of this tendency is still unclear. The frequent delay in accurate diagnosis was reported in the literature [6,7,9]. The median time from the onset to diagnosis in this study is 1 year, with a range of 0 to 9 years. All suspected cases of neck/thyroid infection or mass in infants and children, especially on the left side, should undergo barium esophagography, which may illustrate pharyngeal anomaly.
The diagnosis of PSF is usually established by a barium esophagography [1,2,10–12]. We performed the barium swallow study often in the noninfected state to reduce the false-negative results. Thin contrast can improve the accuracy. Multiple diagnostic investigations should be used to ensure the proper diagnosis. Ultrasound is useful in showing the relationship of the mass with thyroid and the presence of gas within the lesion [10–12], but with a low positive predictive value (7.9%). We found that CT scan with oral contrast was also a convenient tool to delineate the tract and its surrounding structures [13]. An intravenous contrast-enhanced CT study has been considered a valuable modality in demonstrating the site and extent of the lesion. Other authors advocated that magnetic resonance imaging (MRI) and pharyngoscopy/(micro)laryngoscopy are essential investigations of choice [8–11]. Technetium scan of the thyroid showed a decreased uptake in the upper portion, but had no contribution to the diagnosis. Other modalities, such as x-ray film of the neck and fine-needle aspiration, cannot establish an unequivocal diagnosis. Incision and drainage was frequently performed, but with a high failure rate. Clear identification and exact resection of the fistula, after resolution of the acute infection, are vital to the success of surgical management. Identification of the fistula tract during surgery can be quite difficult in children with repeated infections and surgical drainages. Recurrence was reported in two patients who underwent open surgery without endoscopic assistance in our hospital because no definite fistula tract had been dissected. In order to facilitate localization, we used intraoperative endoscopy-assisted intubation or methylene blue injection through the internal orifice as a guide since 1999. To begin with, meticulous exploration of the neck was performed after mobilizing the ipsilateral thyroid. The sinus tract could penetrate the thyroid cartilage near the inferior cornu, the inferior pharyngeal constrictor muscle, or the cricothyroid membrane when it emerged from the larynx. Then, a 3F catheter was inserted into the tract from the internal opening and left it in the loci. In some cases, the catheter was found could easily slip out of the position and then a volume of about 0.5 ml methylene blue was injected into the sinus instead as an indicator. Incision of the inferior pharyngeal constrictor muscle and exposure of the inferior cornu of thyroid cartilage might simplify the dissection of the proximal part of the fistula. The tract was clearly demonstrated in the operative field in all 42 cases (Fig. 4). The cannulation of the tract with a guide wire or light was also reported useful by other researchers [14,15]. It should be reminded injury to the recurrent or superior laryngeal nerves and neighboring great vessels should be minimized [16], and accidental removal of important immune organ thymus must be avoided especially in children younger than 5 years. The fistula should be
Fig. 4. Operative view. Clear identification of the fistulous tract (arrow) is shown after intraoperative endoscopy-assisted methylene blue injection through the internal opening as a guide during ligation (A) and resection (B) of the distal tract.
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ligated and excised as high as possible. If the ipsilateral thyroid is involved, partial thyroidectomy is indicated. The fistula, surrounding fibrotic tissues, and a portion of thyroid gland were excised en bloc. However, some cases have been reported in which the visualization of the fistula opening failed with assistance of endoscopy [7,10]. Then the verification of the tract should be confirmed by histopathology. Recently, endoscopic obliteration of the sinus alone using laser, chemo (trichloroacetic acid, silver nitrate, or fibrin) or electrocautery represents a minimally invasive technique [17–22]. Although vocal fold immobility and recurrence have been noted in patients after chemocauterizaton [23,24], this new approach is a viable alternative to open excision. The postoperative results were good in majority of cases. However, there were still three patients (6.3%) who developed unexpected complications. The vocal fold motion impairment is always temporary and the function can restore within weeks or months [8,24,25]. However, left-sided Horner syndrome, with the symptoms of ptosis and miosis, was noted in one child 2 days after the operation. Also, the symptoms did not improve after 4-month follow-up. Hence, it should be recognized that there is a risk of iatrogenic sympathetic nervous system injury during neck exploration. Other complications such as facial nerve paralysis and salivary fistula were also reported in the literature [1,2,26]. In summary, pyriform sinus fistula is not as rare as previously reported, and the diagnosis should be considered in any children with repeated neck or thyroid infection/mass. Imaging studies, such as barium esophagography, CT scan (with oral contrast or intravenous contrast), and pharyngoscopy/laryngoscopy advocated by other investigators, are essential to establish the diagnosis. Intraoperative endoscopy-assisted intubation or methylene blue injection through the internal opening as a guide can facilitate the exhibition of the tract during dissection. The exact ligation and resection of fistula is important for preventing recurrence. References [1] Nicoucar K, Giger R, Pope Jr HG, et al. Management of congenital fourth branchial arch anomalies: a review and analysis of published cases. J Pediatr Surg 2009;44: 1432–9. [2] Nicoucar K, Giger R, Jaecklin T, et al. Management of congenital third branchial arch anomalies: a systematic review. Otolaryngol Head Neck Surg 2010;142: 21–28e2. [3] Goff CJ, Allred C, Glade RS. Current management of congenital branchial cleft cysts, sinuses, and fistulae. Curr Opin Otolaryngol Head Neck Surg 2012;20:533–9.
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