Journal of Pediatric Surgery 49 (2014) 618–621
Contents lists available at ScienceDirect
Journal of Pediatric Surgery journal homepage: www.elsevier.com/locate/jpedsurg
Endoscopic-assisted surgery for pyriform sinus fistula in children: Experience of 165 cases from a single institution☆ Xianmin Xiao ⁎, Shan Zheng, Jicui Zheng, Linlin Zhu, Kuiran Dong, Chun Shen, Kai Li Department of Surgery of Children’s Hospital, Fudan University, Shanghai, People's Republic of China
a r t i c l e
i n f o
Article history: Received 17 September 2013 Received in revised form 10 October 2013 Accepted 3 November 2013 Key words: Pyriform sinus fistula Endoscopic-assisted surgery Children Branchial remnant
a b s t r a c t 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. © 2014 Elsevier Inc. All rights reserved.
Pyriform sinus fistula (PSF) is a rare congenital anomaly owing to failure of complete obliteration of the third or fourth branchial pouch [1], 80% of cases have their onset in infancy or childhood [2]. Complete removal of the fistula with or without affected thyroid tissue, is the most commonly used and most thorough treatment. However, it can be challenging to identify the fistula intraoperatively and therefore surrounding tissues may be damaged. Selecting an appropriate support technology to completely resect the fistula is very important. In 1983, Miller [3] firstly speculated on endoscopic cannulation of the tract in order to facilitate localization during dissection. Since then, an
☆ Statement of financial support: Key Clinical Discipline of the Chinese Ministry of Health in 2010–2012. ⁎ Corresponding author at: Department of Surgery, Children’s Hospital, Fudan University, 399 Wanyuan Road, Shanghai 201102, People's Republic of China. E-mail address: [email protected] (X. Xiao). 0022-3468/$ – see front matter © 2014 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpedsurg.2013.11.004
increasing number of case series have reported lower relapsing and few complications with endoscopic-assisted surgical resection of PSF. This report reviews a single institution experience of the clinical manifestations, treatment, and prognosis in children with PSF. 1. Materials and methods One hundred sixty-five children were diagnosed with PSF and underwent endoscopic-assisted surgery at the Department of Pediatric Surgery, Children’s Hospital, Fudan University from 1999 to 2013. This study was conducted with the approval of our institutional review board. We examined demographics, clinical manifestations, diagnostic methods, operative findings, histological findings, postoperative course and complications. In most cases, the operation was performed 2–3 months after the acute inflammation was controlled. Those with frequently recurring
X. Xiao et al. / Journal of Pediatric Surgery 49 (2014) 618–621
infection occasionally had to undertake the operation as early as 1 month or so. The surgical procedure was similar to that described by Makoto Honzumi [4,5]. Briefly, the patient was given general nasotracheal anesthesia with the neck hyperextended, and a standard thyroid surgery incision or a collar incision along the old infection and incision scar was made [6,7]. The linea alba cervicalis was incised and the anterior cervical muscle group was retracted laterally. After the superior thyroid artery and vein were ligated and cut, the upper pole of the thyroid lobe was retracted laterally to show the surfaces of cricothyroid muscle. The inferior cornu of the thyroid cartilage (ICTC) was then identified by palpation as the landmark of the inferior edge of the thyroid cartilage. At this stage, a shallow suture placed on the thyroid cartilage to provide anterior traction is helpful. The scar tissue anterior and inferior to ICTC was dissected, and the fistula tract was carefully searched. In order to avoid injury to the recurrent laryngeal nerve, the dissection did not cross the posterior edge of the thyroid cartilage. Endoscopy was then performed using a pediatric gastroscope with the tip sliding down on the lateral pharyngeal wall of the affected side. The transillumination in the operative field indicated that the tip was in the correct position. When the internal orifice of the fistula was visualized, a catheter with a round top was loosely inserted into the orifice about 3–5 mm deep. Methylene blue (1:10 dilution, 1–2 ml) was injected through the catheter into the fistula. Although some tracts could be found without the help of the endoscopy, they were further confirmed by the dye. More commonly in our series, the tracts were indentified after the dye injection. The tract was then pursued upward until reaching its expansion part, namely the apex of the pyriform sinus. As the tract might closely adhere to the perichondrium of thyroid cartilage or the capsule of cricothyroid joint, sharp dissection was often needed. A double ligation and transection were made just at the level of the expansion. The distal portion of the tract with or without a fibrous cyst was then resected.
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2. Results 2.1. Clinical features This study was composed of 165 cases with PSF (Table 1). 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), with a predominant distribution in the first 5 years (74.15%). The median age of diagnosis was 32.0 months (range, 6 days to 178 months), and the median age at operation was 5.0 years (range, 17 days to 15.0 years). The overwhelming majority of cases (94.6%) occurred on the left side. The clinical presentations included recurrent cervical inflammatory swelling, neck abscess, repeated incision and drainage, and acute suppurative thyroiditis. Twenty-nine cases presented with a distinct cervical cyst. Initial surgical treatments at other hospitals included incision and drainage (mean number, 2.1; range, 1–15) in 89 cases, and failed open surgical procedure in 15 cases. 2.2. Diagnostic investigations In our series, barium swallow delineated the fistula tract originating from the apex of the pyriform sinus in 152 cases (97.4%), but did not reveal in 4 cases. In 81 cases CT demonstrated the shallower or disappearing pyriform sinus, soft tissue cellulitis, abscess, large cystic lesion with air and fluid. Ultrasound revealed hypoechoic appearance of the parathyroid soft tissue, involving the adjacent thyroid lobe. Isotope thyroid scan showed reduced uptake of the upper lobe of thyroid gland, or “cold nodule” in quiescent period (Table 1). 2.3. Treatment and outcome The fistulas were completely excised in all but 2 (98.8%) with intraoperative gastroscopy successfully conducted in 160 cases
Table 1 Summary of the data of 165 children with PSF.
Clinical features
Male Age (y)
Side of presentation
Clinical presentation Treatment before admission
Imaging findings
Barium swallow
CT Ultrasound
Operation and findings
Complications
a b c d
Thyroid Scan Endoscopy Fistula resection Penetrating pointa
Wound infection Esophageal injury Voice hoarseness Recurrence
The point where fistula penetrates into the pharynx. Fistula anterior to the inferior cornu of the thyroid cartilage (ICTC). Fistula inferior to ICTC. Fistula posterior to ICTC.
0–5 5–10 N10 Left Right Bilateral Recurrent infection and cutaneous discharging fistula Cervical cyst Only antibiotics Incision and drainage Resection Clear fistula Not clear Normal or doubt Normal Inflammatory tissue, gas pocket, and fistula Normal Neck mass or inflammatory tissue Reduced uptake of the upper lobe of thyroid or cold nodule Successful Successful Type Ib Type IIc Type IIId
No.
(%)
100 117 43 5 156 7 2 136 29 61 89 15 152 2 2 4 81 2 81 16 160 163 22 18 37 0 2 1 5
60.6 70.9 26.1 3.0 94.6 4.2 1.2 82.4 17.6 37.0 53.9 9.1 97.4 1.3 1.3 4.7 95.3 2.4 97.6 100 97.0 98.8 28.5 23.4 48.1 0 1.2 0.6 3.0
620
X. Xiao et al. / Journal of Pediatric Surgery 49 (2014) 618–621
3. Discussion PSF is a rare congenital anomaly, which is supposed to arise from an incomplete obliteration of the third or fourth branchial pouch. The fistula consists of a thin, fragile membranous tract that is closely associated with the important structures such as the recurrent laryngeal nerve, and always leads to acute inflammation and surrounding scar tissue. Although other options such as endoscopic cauterization or sealing of the internal opening can be considered, an open neck surgery to completely excise the fistula is the most reliable and commonly used for cure. However, the surgical procedure can be quite difficult because of the anatomical and histological characteristics of the fistula. 3.1. Clinical features
Fig. 1. Schematic drawing of the points where fistulas penetrated into the pharynx, and the fistula anatomic relationship with I (T, thyroid cartilage; I, inferior cornu of the thyroid cartilage; C, cricothyroid muscle; I, anterior to I; II, inferior to I; III, posterior to I).
(97.0%). In the remaining 5 cases that gastroscopy failed, the fistula tracts could be eventually excised in 3 cases, but not found in 2 neonates with large cervical cystic mass. In 77 cases since 2002, the points where fistulas penetrated into the pharynx were specified intraoperatively, which were classified into 3 types according to their anatomic relationship with ICTC (Table 1, Fig. 1): type I (anterior to ICTC, Fig. 2A), 22 cases (28.5%); type II (inferior to ICTC, Fig. 2B), 18 cases (23.4%); and type III (posterior to ICTC, Fig. 2C), 37 cases (48.1%). While dissecting the cervical cysts, esophageal perforations happened in a neonate and a young infant and were repaired. Postoperatively, both patients had a little bit longer fasting duration, but recovered well. Voice hoarseness occurred after operation in one neonate with a cervical cyst, but disappeared 3 months later. PSF recurred in 5 cases, 2 of them were cured by a successful re-excision. The other 3 have remained asymptomatic for 13–105 months. In our series, median follow-up period was 2.4 years (range, 2 months to 13.7 years). Pathological examination of the surgical specimens revealed that they were lined with squamous cell epithelium, stratified columnar epithelium, with or without chronic inflammatory cell infiltration, thereby confirming the diagnosis of a bronchial anomaly.
Although there have been many published reports of PSF, most of them are case reports or have a limited number of patients. To provide more comprehensive information on this disease, Nicoucar et al [8] reviewed 177 published reports (from 1968 to 2006) of 526 patients including both adults and children. In his review, demographic data revealed no gender predominance. Only 41.4% cases were under 8 years old and the mean age of onset was 9 years. In contrast, to our knowledge, our series is the largest reports of PSF in children, especially for those who underwent a surgical procedure. Among our cases, the male to female ratio was 1.54:1. The median age of onset and at operation was 3.2 and 5.0 years, respectively. The results showed a male predominance and a recent trend of early diagnosis and treatment, and therefore, have the potential to facilitate our understanding of the disease. 3.2. Diagnosis Various investigations were used in our series, including barium swallow, ultrasound, computed tomography, and thyroid scan. However, barium swallow was taken as the gold standard for diagnosis and performed in nearly all (156 of 165 cases) with a very high rate (97.4%) of success. For the neonates and young infants (younger than 3 months), CT scan could be used to reveal gas pocket within the cyst or abscess, which strongly supported the diagnosis of PSF. No preoperative endoscopy was performed in our cases. 3.3. Endoscopic-assisted technique In order to facilitate the localization of the fistula, direct pharyngoscope [9,10] and the pediatric gastrofiberscope [9] were
Fig. 2. Operative findings proved the points where the fistulas penetrated into the pharynx. A, The fistula anterior to the inferior cornu of the thyroid cartilage. B, The fistula inferior to the inferior cornu of the thyroid cartilage. C, The fistula posterior to the inferior cornu of the thyroid cartilage (T, the tip of the inferior cornu of the thyroid cartilage; U, upper pole of the left thyroid lobe; arrows, the points where the fistulas penetrated into the pharynx; arrowheads, fistulas).
X. Xiao et al. / Journal of Pediatric Surgery 49 (2014) 618–621
utilized during the definitive operation. When the opening in the ipsilateral pyriform sinus is visualized, a catheter is placed into the opening as a guide, or followed by dye injection [11]. In our series, a pediatric gastroscope was used with a high success rate of 97.0%. In our experience, the pediatric gastroscopy would offer some advantages, namely, popular use in pediatric general surgical practice, reaching pyriform sinus easily, and a good stretching effect on the wrinkly mucosa [9]. The solution of methylene blue that we used for injection was properly diluted, so that the color could be washed out if the dye extravasation happened. Our gastroscopy failed in 5 cases, small body size together with a large cervical cyst might be the reason. 3.4. Treatment and results The theoretic courses of the third and fourth branchial fistulas have been widely quoted in the literature, but case reports matching these descriptions are rare [2,12]. Clinically, various courses of PSF, especially the anatomic point where the fistula tract penetrates into the pharynx, have been described. In early reports, Miyauchi et al noted a PSF course that penetrated the cricothyroid muscle at the lower edge of the thyroid cartilage [2,13]. In addition, Honzumi et al [4] reported another course that was in contact with the lowest edge of ICTC and penetrated the inferior pharyngeal constrictor in 4 of 6 children. More recently, James et al reviewed their 15 patients and found all of them having a third course that passed directly from the posteromedial surface of the thyroid into the inferior pharyngeal constrictor, traveling intimately associated with the recurrent laryngeal nerve [10]. Interestingly, all the three courses mentioned above were encountered in our series, and the points where the fistulas penetrated into the pharynx seemed to be confined to a small area around ICTC. In order to summarize our findings, the anatomic points were arbitrarily classified into 3 types according to their relationship with ICTC. In our series, more than one fifth of the cases (23.4%) had a fistula of type II, which was at the most caudal level and in close contact with the tip of ICTC, and therefore differed from the other two types. Sharp dissection was always needed to partially free the tip of ICTC so as to gain a high level ligation and excision of the fistula. The type III was more common in our series (48.1%). To avoid the recurrent laryngeal nerve injury, the dissection in searching for a fistula of this type should be restricted to the area anterior and inferior to ICTC, and be performed only with the help of endoscopy and dye injection. These fistulas could be found a few millimeters posterior to the tip of ICTC and then traced upward into the inferior pharyngeal constrictor without any difficulty. In the cases of type I, the fistulas located anterior to ICTC and penetrated into the pharynx at the level of the lower margin of the thyroid cartilage, the dissection was relatively easy and safe. It is not clear to us why the 5 cases had postoperative recurrence. Nevertheless, failure to excise the fistula at a high level, especially in the cases of type II, and inadvertent damage to the wall of pyriform sinus during endoscopic cannulation probably contributed to the complication. In our series, neonates and young
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infants with a large cervical cyst represented a special subset. Although the cysts were dissected with great caution, esophageal perforation and postoperative hoarseness occurred in 3 cases. With more experience, it is anticipated that the operative complications would be further reduced. 4. Conclusions Based on the literature and our experience, PSF would not be as rare as expected, and a higher index of suspicion of it should be raised in the management of neck lesions in children. To our knowledge, this series is the largest report of PSF in children, which could provide new insights into the clinical features and management of the disease. The clinical features in this series 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 in nearly all cases with minimized complications. For neonates and young infants with a large cervical cyst, however, the management of PSF continues to be a challenge. Acknowledgments Thanks are due to Falcone RA. of Cincinnati Children’s Hospital Medical Center for the help in editing our manuscript. This work was supported by the Key Clinical Discipline of the Chinese Ministry of Health in 2010–2012. References [1] Chaudhary N, Gupta A, Motwani G, et al. Fistula of the fourth branchial pouch. Am J Otolaryngol 2003;24:250–2. [2] Miyauchi A, Matsuzuka F, Takai S, et al. Piriform sinus fistula. A route of infection in acute suppurative thyroiditis. Arch Surg 1981;116:66–9. [3] Miller D, Hill JL, Sun CC, et al. The diagnosis and management of pyriform sinus fistulae in infants and young children. J Pediatr Surg 1983;18:377–81. [4] Honzumi Makoto, Suzuki Hiroshi, Tsukamoto Yoshihide. Surgical resection for pyriform sinus fistula. J Pediatr Surg 1993;28:877–9. [5] Kubota Masayuki, Suita Sachiyo, Kamimura Tetsuro, et al. Surgical strategy for the treatment of pyriform sinus fistula. J Pediatr Surg 1997;32:34–7. [6] Xianmin Xiao, Jihua Zheng, Baixiang Jin. Diagnosis and management of piriform sinus fistulae in children. Chin J Surg 1997;35:354–6. [7] Lv Zhibao, Xiao Xianmin, Zheng Shan, et al. Gastroscopic assistant operation for children with pyriform sinus fistula. Chin J Pediatr Surg 2005;26:17–9. [8] 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. [9] Nonomura N, Ikarashi F, Fujisaki T, et al. Surgical approach to pyriform sinus fistula. Am J Otolaryngol 1993;14:111–5. [10] 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. [11] Kubota M, Suita S, Kamimura T, et al. Surgical strategy for the treatment of pyriform sinus fistula. J Otolaryngol 1997;26:57–63. [12] Takai S-I, Miyauchi A, Matsuzaka F, et al. Internal fistula as a route of infection in acute suppurative thyroiditis. Lancet 1979;1:751–2. [13] Miyauchi A, Matsuzuka F, Kuma K. Pyriform sinus fistula: an underlying abnormality common in patients with acute suppurative thyroiditis. World J Surg 1990;14:400–5.
Contents lists available at ScienceDirect
Journal of Pediatric Surgery journal homepage: www.elsevier.com/locate/jpedsurg
Endoscopic-assisted surgery for pyriform sinus fistula in children: Experience of 165 cases from a single institution☆ Xianmin Xiao ⁎, Shan Zheng, Jicui Zheng, Linlin Zhu, Kuiran Dong, Chun Shen, Kai Li Department of Surgery of Children’s Hospital, Fudan University, Shanghai, People's Republic of China
a r t i c l e
i n f o
Article history: Received 17 September 2013 Received in revised form 10 October 2013 Accepted 3 November 2013 Key words: Pyriform sinus fistula Endoscopic-assisted surgery Children Branchial remnant
a b s t r a c t 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. © 2014 Elsevier Inc. All rights reserved.
Pyriform sinus fistula (PSF) is a rare congenital anomaly owing to failure of complete obliteration of the third or fourth branchial pouch [1], 80% of cases have their onset in infancy or childhood [2]. Complete removal of the fistula with or without affected thyroid tissue, is the most commonly used and most thorough treatment. However, it can be challenging to identify the fistula intraoperatively and therefore surrounding tissues may be damaged. Selecting an appropriate support technology to completely resect the fistula is very important. In 1983, Miller [3] firstly speculated on endoscopic cannulation of the tract in order to facilitate localization during dissection. Since then, an
☆ Statement of financial support: Key Clinical Discipline of the Chinese Ministry of Health in 2010–2012. ⁎ Corresponding author at: Department of Surgery, Children’s Hospital, Fudan University, 399 Wanyuan Road, Shanghai 201102, People's Republic of China. E-mail address: [email protected] (X. Xiao). 0022-3468/$ – see front matter © 2014 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpedsurg.2013.11.004
increasing number of case series have reported lower relapsing and few complications with endoscopic-assisted surgical resection of PSF. This report reviews a single institution experience of the clinical manifestations, treatment, and prognosis in children with PSF. 1. Materials and methods One hundred sixty-five children were diagnosed with PSF and underwent endoscopic-assisted surgery at the Department of Pediatric Surgery, Children’s Hospital, Fudan University from 1999 to 2013. This study was conducted with the approval of our institutional review board. We examined demographics, clinical manifestations, diagnostic methods, operative findings, histological findings, postoperative course and complications. In most cases, the operation was performed 2–3 months after the acute inflammation was controlled. Those with frequently recurring
X. Xiao et al. / Journal of Pediatric Surgery 49 (2014) 618–621
infection occasionally had to undertake the operation as early as 1 month or so. The surgical procedure was similar to that described by Makoto Honzumi [4,5]. Briefly, the patient was given general nasotracheal anesthesia with the neck hyperextended, and a standard thyroid surgery incision or a collar incision along the old infection and incision scar was made [6,7]. The linea alba cervicalis was incised and the anterior cervical muscle group was retracted laterally. After the superior thyroid artery and vein were ligated and cut, the upper pole of the thyroid lobe was retracted laterally to show the surfaces of cricothyroid muscle. The inferior cornu of the thyroid cartilage (ICTC) was then identified by palpation as the landmark of the inferior edge of the thyroid cartilage. At this stage, a shallow suture placed on the thyroid cartilage to provide anterior traction is helpful. The scar tissue anterior and inferior to ICTC was dissected, and the fistula tract was carefully searched. In order to avoid injury to the recurrent laryngeal nerve, the dissection did not cross the posterior edge of the thyroid cartilage. Endoscopy was then performed using a pediatric gastroscope with the tip sliding down on the lateral pharyngeal wall of the affected side. The transillumination in the operative field indicated that the tip was in the correct position. When the internal orifice of the fistula was visualized, a catheter with a round top was loosely inserted into the orifice about 3–5 mm deep. Methylene blue (1:10 dilution, 1–2 ml) was injected through the catheter into the fistula. Although some tracts could be found without the help of the endoscopy, they were further confirmed by the dye. More commonly in our series, the tracts were indentified after the dye injection. The tract was then pursued upward until reaching its expansion part, namely the apex of the pyriform sinus. As the tract might closely adhere to the perichondrium of thyroid cartilage or the capsule of cricothyroid joint, sharp dissection was often needed. A double ligation and transection were made just at the level of the expansion. The distal portion of the tract with or without a fibrous cyst was then resected.
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2. Results 2.1. Clinical features This study was composed of 165 cases with PSF (Table 1). 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), with a predominant distribution in the first 5 years (74.15%). The median age of diagnosis was 32.0 months (range, 6 days to 178 months), and the median age at operation was 5.0 years (range, 17 days to 15.0 years). The overwhelming majority of cases (94.6%) occurred on the left side. The clinical presentations included recurrent cervical inflammatory swelling, neck abscess, repeated incision and drainage, and acute suppurative thyroiditis. Twenty-nine cases presented with a distinct cervical cyst. Initial surgical treatments at other hospitals included incision and drainage (mean number, 2.1; range, 1–15) in 89 cases, and failed open surgical procedure in 15 cases. 2.2. Diagnostic investigations In our series, barium swallow delineated the fistula tract originating from the apex of the pyriform sinus in 152 cases (97.4%), but did not reveal in 4 cases. In 81 cases CT demonstrated the shallower or disappearing pyriform sinus, soft tissue cellulitis, abscess, large cystic lesion with air and fluid. Ultrasound revealed hypoechoic appearance of the parathyroid soft tissue, involving the adjacent thyroid lobe. Isotope thyroid scan showed reduced uptake of the upper lobe of thyroid gland, or “cold nodule” in quiescent period (Table 1). 2.3. Treatment and outcome The fistulas were completely excised in all but 2 (98.8%) with intraoperative gastroscopy successfully conducted in 160 cases
Table 1 Summary of the data of 165 children with PSF.
Clinical features
Male Age (y)
Side of presentation
Clinical presentation Treatment before admission
Imaging findings
Barium swallow
CT Ultrasound
Operation and findings
Complications
a b c d
Thyroid Scan Endoscopy Fistula resection Penetrating pointa
Wound infection Esophageal injury Voice hoarseness Recurrence
The point where fistula penetrates into the pharynx. Fistula anterior to the inferior cornu of the thyroid cartilage (ICTC). Fistula inferior to ICTC. Fistula posterior to ICTC.
0–5 5–10 N10 Left Right Bilateral Recurrent infection and cutaneous discharging fistula Cervical cyst Only antibiotics Incision and drainage Resection Clear fistula Not clear Normal or doubt Normal Inflammatory tissue, gas pocket, and fistula Normal Neck mass or inflammatory tissue Reduced uptake of the upper lobe of thyroid or cold nodule Successful Successful Type Ib Type IIc Type IIId
No.
(%)
100 117 43 5 156 7 2 136 29 61 89 15 152 2 2 4 81 2 81 16 160 163 22 18 37 0 2 1 5
60.6 70.9 26.1 3.0 94.6 4.2 1.2 82.4 17.6 37.0 53.9 9.1 97.4 1.3 1.3 4.7 95.3 2.4 97.6 100 97.0 98.8 28.5 23.4 48.1 0 1.2 0.6 3.0
620
X. Xiao et al. / Journal of Pediatric Surgery 49 (2014) 618–621
3. Discussion PSF is a rare congenital anomaly, which is supposed to arise from an incomplete obliteration of the third or fourth branchial pouch. The fistula consists of a thin, fragile membranous tract that is closely associated with the important structures such as the recurrent laryngeal nerve, and always leads to acute inflammation and surrounding scar tissue. Although other options such as endoscopic cauterization or sealing of the internal opening can be considered, an open neck surgery to completely excise the fistula is the most reliable and commonly used for cure. However, the surgical procedure can be quite difficult because of the anatomical and histological characteristics of the fistula. 3.1. Clinical features
Fig. 1. Schematic drawing of the points where fistulas penetrated into the pharynx, and the fistula anatomic relationship with I (T, thyroid cartilage; I, inferior cornu of the thyroid cartilage; C, cricothyroid muscle; I, anterior to I; II, inferior to I; III, posterior to I).
(97.0%). In the remaining 5 cases that gastroscopy failed, the fistula tracts could be eventually excised in 3 cases, but not found in 2 neonates with large cervical cystic mass. In 77 cases since 2002, the points where fistulas penetrated into the pharynx were specified intraoperatively, which were classified into 3 types according to their anatomic relationship with ICTC (Table 1, Fig. 1): type I (anterior to ICTC, Fig. 2A), 22 cases (28.5%); type II (inferior to ICTC, Fig. 2B), 18 cases (23.4%); and type III (posterior to ICTC, Fig. 2C), 37 cases (48.1%). While dissecting the cervical cysts, esophageal perforations happened in a neonate and a young infant and were repaired. Postoperatively, both patients had a little bit longer fasting duration, but recovered well. Voice hoarseness occurred after operation in one neonate with a cervical cyst, but disappeared 3 months later. PSF recurred in 5 cases, 2 of them were cured by a successful re-excision. The other 3 have remained asymptomatic for 13–105 months. In our series, median follow-up period was 2.4 years (range, 2 months to 13.7 years). Pathological examination of the surgical specimens revealed that they were lined with squamous cell epithelium, stratified columnar epithelium, with or without chronic inflammatory cell infiltration, thereby confirming the diagnosis of a bronchial anomaly.
Although there have been many published reports of PSF, most of them are case reports or have a limited number of patients. To provide more comprehensive information on this disease, Nicoucar et al [8] reviewed 177 published reports (from 1968 to 2006) of 526 patients including both adults and children. In his review, demographic data revealed no gender predominance. Only 41.4% cases were under 8 years old and the mean age of onset was 9 years. In contrast, to our knowledge, our series is the largest reports of PSF in children, especially for those who underwent a surgical procedure. Among our cases, the male to female ratio was 1.54:1. The median age of onset and at operation was 3.2 and 5.0 years, respectively. The results showed a male predominance and a recent trend of early diagnosis and treatment, and therefore, have the potential to facilitate our understanding of the disease. 3.2. Diagnosis Various investigations were used in our series, including barium swallow, ultrasound, computed tomography, and thyroid scan. However, barium swallow was taken as the gold standard for diagnosis and performed in nearly all (156 of 165 cases) with a very high rate (97.4%) of success. For the neonates and young infants (younger than 3 months), CT scan could be used to reveal gas pocket within the cyst or abscess, which strongly supported the diagnosis of PSF. No preoperative endoscopy was performed in our cases. 3.3. Endoscopic-assisted technique In order to facilitate the localization of the fistula, direct pharyngoscope [9,10] and the pediatric gastrofiberscope [9] were
Fig. 2. Operative findings proved the points where the fistulas penetrated into the pharynx. A, The fistula anterior to the inferior cornu of the thyroid cartilage. B, The fistula inferior to the inferior cornu of the thyroid cartilage. C, The fistula posterior to the inferior cornu of the thyroid cartilage (T, the tip of the inferior cornu of the thyroid cartilage; U, upper pole of the left thyroid lobe; arrows, the points where the fistulas penetrated into the pharynx; arrowheads, fistulas).
X. Xiao et al. / Journal of Pediatric Surgery 49 (2014) 618–621
utilized during the definitive operation. When the opening in the ipsilateral pyriform sinus is visualized, a catheter is placed into the opening as a guide, or followed by dye injection [11]. In our series, a pediatric gastroscope was used with a high success rate of 97.0%. In our experience, the pediatric gastroscopy would offer some advantages, namely, popular use in pediatric general surgical practice, reaching pyriform sinus easily, and a good stretching effect on the wrinkly mucosa [9]. The solution of methylene blue that we used for injection was properly diluted, so that the color could be washed out if the dye extravasation happened. Our gastroscopy failed in 5 cases, small body size together with a large cervical cyst might be the reason. 3.4. Treatment and results The theoretic courses of the third and fourth branchial fistulas have been widely quoted in the literature, but case reports matching these descriptions are rare [2,12]. Clinically, various courses of PSF, especially the anatomic point where the fistula tract penetrates into the pharynx, have been described. In early reports, Miyauchi et al noted a PSF course that penetrated the cricothyroid muscle at the lower edge of the thyroid cartilage [2,13]. In addition, Honzumi et al [4] reported another course that was in contact with the lowest edge of ICTC and penetrated the inferior pharyngeal constrictor in 4 of 6 children. More recently, James et al reviewed their 15 patients and found all of them having a third course that passed directly from the posteromedial surface of the thyroid into the inferior pharyngeal constrictor, traveling intimately associated with the recurrent laryngeal nerve [10]. Interestingly, all the three courses mentioned above were encountered in our series, and the points where the fistulas penetrated into the pharynx seemed to be confined to a small area around ICTC. In order to summarize our findings, the anatomic points were arbitrarily classified into 3 types according to their relationship with ICTC. In our series, more than one fifth of the cases (23.4%) had a fistula of type II, which was at the most caudal level and in close contact with the tip of ICTC, and therefore differed from the other two types. Sharp dissection was always needed to partially free the tip of ICTC so as to gain a high level ligation and excision of the fistula. The type III was more common in our series (48.1%). To avoid the recurrent laryngeal nerve injury, the dissection in searching for a fistula of this type should be restricted to the area anterior and inferior to ICTC, and be performed only with the help of endoscopy and dye injection. These fistulas could be found a few millimeters posterior to the tip of ICTC and then traced upward into the inferior pharyngeal constrictor without any difficulty. In the cases of type I, the fistulas located anterior to ICTC and penetrated into the pharynx at the level of the lower margin of the thyroid cartilage, the dissection was relatively easy and safe. It is not clear to us why the 5 cases had postoperative recurrence. Nevertheless, failure to excise the fistula at a high level, especially in the cases of type II, and inadvertent damage to the wall of pyriform sinus during endoscopic cannulation probably contributed to the complication. In our series, neonates and young
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infants with a large cervical cyst represented a special subset. Although the cysts were dissected with great caution, esophageal perforation and postoperative hoarseness occurred in 3 cases. With more experience, it is anticipated that the operative complications would be further reduced. 4. Conclusions Based on the literature and our experience, PSF would not be as rare as expected, and a higher index of suspicion of it should be raised in the management of neck lesions in children. To our knowledge, this series is the largest report of PSF in children, which could provide new insights into the clinical features and management of the disease. The clinical features in this series 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 in nearly all cases with minimized complications. For neonates and young infants with a large cervical cyst, however, the management of PSF continues to be a challenge. Acknowledgments Thanks are due to Falcone RA. of Cincinnati Children’s Hospital Medical Center for the help in editing our manuscript. This work was supported by the Key Clinical Discipline of the Chinese Ministry of Health in 2010–2012. References [1] Chaudhary N, Gupta A, Motwani G, et al. Fistula of the fourth branchial pouch. Am J Otolaryngol 2003;24:250–2. [2] Miyauchi A, Matsuzuka F, Takai S, et al. Piriform sinus fistula. A route of infection in acute suppurative thyroiditis. Arch Surg 1981;116:66–9. [3] Miller D, Hill JL, Sun CC, et al. The diagnosis and management of pyriform sinus fistulae in infants and young children. J Pediatr Surg 1983;18:377–81. [4] Honzumi Makoto, Suzuki Hiroshi, Tsukamoto Yoshihide. Surgical resection for pyriform sinus fistula. J Pediatr Surg 1993;28:877–9. [5] Kubota Masayuki, Suita Sachiyo, Kamimura Tetsuro, et al. Surgical strategy for the treatment of pyriform sinus fistula. J Pediatr Surg 1997;32:34–7. [6] Xianmin Xiao, Jihua Zheng, Baixiang Jin. Diagnosis and management of piriform sinus fistulae in children. Chin J Surg 1997;35:354–6. [7] Lv Zhibao, Xiao Xianmin, Zheng Shan, et al. Gastroscopic assistant operation for children with pyriform sinus fistula. Chin J Pediatr Surg 2005;26:17–9. [8] 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. [9] Nonomura N, Ikarashi F, Fujisaki T, et al. Surgical approach to pyriform sinus fistula. Am J Otolaryngol 1993;14:111–5. [10] 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. [11] Kubota M, Suita S, Kamimura T, et al. Surgical strategy for the treatment of pyriform sinus fistula. J Otolaryngol 1997;26:57–63. [12] Takai S-I, Miyauchi A, Matsuzaka F, et al. Internal fistula as a route of infection in acute suppurative thyroiditis. Lancet 1979;1:751–2. [13] Miyauchi A, Matsuzuka F, Kuma K. Pyriform sinus fistula: an underlying abnormality common in patients with acute suppurative thyroiditis. World J Surg 1990;14:400–5.
