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Cricopharyngeal achalasia treated with myectomy and post-operative high-resolution manometry
G Model PEDOT 7104 No. of Pages 4
International Journal of Pediatric Otorhinolaryngology xxx (2014) xxx–xxx
Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Case Report
Cricopharyngeal achalasia treated with myectomy and post-operative high-resolution manometry Toshihiko Watanabe a, * , Takahiro Shimizu a , Masataka Takahashi a , Kaori Sato a , Michinobu Ohno a , Yasushi Fuchimoto a , Takanobu Maekawa b , Katsuhiro Arai c , Kunio Mizutari d , Noriko Morimoto d , Yutaka Kanamori a a
Division of Surgery, Department of Surgical Specialties, National Center for Child Health and Development, Tokyo, Japan Division of Pediatrics, Department of General Pediatrics and Interdisciplinary Medicine, National Center for Child Health and Development, Tokyo, Japan Division of Gastroenterology, Department of Medical Subspecialties, National Center for Child Health and Development, Tokyo, Japan d Division of Otolaryngology, Department of Surgical Specialties, National Center for Child Health and Development, Tokyo, Japan b c
A R T I C L E I N F O
A B S T R A C T
Article history: Received 6 March 2014 Received in revised form 16 April 2014 Accepted 20 April 2014 Available online xxx
Cricopharyngeal achalasia is an uncommon cause of dysphagia in neonates or children. A nine-year-old female patient was referred to us with a long history of dysphagia, recurrent pulmonary infection and growth stunting. A gastrostomy was introduced to improve her nutritional condition and to minimize potential inflammation in the pharynx. Subsequently, cervical cricopharyngeal myectomy was conducted. The surgical intervention allowed prompt resolution of symptoms without complications. High-resolution manometry post myectomy demonstrated a significant reduction in upper esophageal pressure together with proper relaxation at deglutition. The patient was able to consume solid food and liquid normally, and remained asymptomatic without medications six months after the surgery. ã 2014 Elsevier Ireland Ltd. All rights reserved.
Keywords: Cricopharyngeal achalasia Cricopharyngeal myectomy Dysphagia Pharyngocele Motility High-resolution manometry
1. Introduction
2. Case report
Cricopharyngeal achalasia (CPA) is a rare clinical entity with little information available concerning etiology, pathogenesis and the best management [1]. The inappropriate contraction of the cricopharyngeal muscle results in an inability to relax the upper esophageal sphincter (UES) during deglutition, leading to dysphagia, nasopharyngeal reflux during feeding, salivation, choking and failure to thrive [2–4]. Treatment options include botulinum toxin injection, mechanical dilation and cricopharyngeal myotomy [2,5– 7]. Here, we describe the clinical case of a pediatric CPA patient with a history of dysphagia, recurrent pulmonary infection and growth stunting, who was successfully treated by cervical cricopharyngeal myectomy.
Our patient is a nine-year-old female with a long history of dysphagia and recurrent pneumonia. She was born at full-term gestation after an uncomplicated pregnancy. She had no congenital abnormalities and her family history was unremarkable. No feeding problem was observed throughout infancy. At two years of age, she suffered from sudden onset of dysphagia after varicella infection, which resulted in intermittent fever and weight loss. Tumorous obstruction was initially suspected by magnetic resonance imaging. Nasogastric tube feeding was introduced to maintain her nutritional condition. Rehabilitation program for dysphagia resulted in some subjective improvement in her swallowing function, and daily tube feeding was withdrawn in six months. She was followed-up at her regional hospital for a period of time. At seven years of age, swallowing difficulty occurred which led to insufficient consumption of food. She displayed peculiar eating behavior; tilting the head to one side in order to bring food from the pharynx down to the esophagus. Oftentimes, residues of a meal would be regurgitated, with only partially chewed food ingested. Due to frequent choking or laryngopharyngeal gurgling with excessive salivation, expectorant and bronchodilating agents were
* Corresponding author at: 2-10-1 Okura, Setagaya-ku, Tokyo 157-8535, Japan. Tel.: +81 3 3416 0181; fax: +81 3 3416 2222. E-mail addresses: [email protected], [email protected] (T. Watanabe). http://dx.doi.org/10.1016/j.ijporl.2014.04.036 0165-5876/ ã 2014 Elsevier Ireland Ltd. All rights reserved.
Please cite this article in press as: T. Watanabe, et al., Cricopharyngeal achalasia treated with myectomy and post-operative high-resolution manometry, Int. J. Pediatr. Otorhinolaryngol. (2014), http://dx.doi.org/10.1016/j.ijporl.2014.04.036
G Model PEDOT 7104 No. of Pages 4
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Fig. 1. Preoperative videofluoroscopic swallow study. (A) Persistent narrowing at the level of cricopharyngeal muscle (arrow) was present during deglutition. (B) A marked delay in pharyngeal transit results in stasis of the contrast medium in the pharynx. Note the multiple pharyngocele formation (asterisk) and pouch-shaped piriform sinus (P). Trace silent aspiration is seen (T: trachea; E: esophagus).
required, suggesting chronic microaspiration. Bedside laryngoscopy performed by a pediatric otolaryngologist revealed the stasis of massive saliva in the pharynx, and a tentative diagnosis of pharyngocele was made. Subsequently, the patient was referred to a surgical department due to refractory dysphagia, recurrent episodes of pneumonia and failure to thrive at nine years of age. Her height (121 cm, 1.6 SD) and weight (19 kg, 1.7 SD) revealed growth stunting. Fluoroscopic image showed persistent narrowing at the level of cricopharynx with a pouched pharynx and multiple pharyngoceles; a likely cause of poor clearance and aspiration of oral contents (Fig. 1). An attempt to insert a probe into her esophagus for 24-h pH monitoring and manometric study failed. Endoscopic examination under general anesthesia revealed a strikingly narrowed lumen in the region of the cricopharyngeous muscle, and a small-diameter endoscope (5.0 mm) was unable to pass through (Fig. 2B). However, under endoscopic view, a pressure probe was successfully inserted into the esophagus. The pressure of the upper esophageal sphincter (UES) was extremely hypertensive (715.7 mmHg), whereas that of the lower esophageal sphincter (LES) was normal (13.2 mmHg). A diagnosis of CPA was made at that stage, and a percutaneous gastrostomy tube was introduced to provide a means of feeding. In order to improve the affected lesion of pouched pharynx and to stabilize potential inflammation of the affected lesion, only minimum liquid meals were allowed. Cricopharyngeal myectomy was performed after the pharynx was stabilized. Successful weight gain was achieved in three months. With the assistance of endoscopy, a balloon catheter was introduced, and a notch formation was evident (Fig. 2A). Access to the pharynx and upper esophagus was achieved through a right cervical incision centered on the radiological sign of the balloon notch. Exposure of the cricopharyngeal muscle was facilitated by a left-lateral retraction of the carotid sheath and sternohyoid muscle, together with a dissection of the superior belly of the omohyoid muscle. Anterior retraction of the thyroid tissue provided wide operative field. The thickened transverse fibers of the cricopharyngeal muscle covered the upper esophagus circumferentially in the cricoid region (Fig. 3A). The exact position of the sphincteral stricture was confirmed by palpating the balloon notch at the bottom of the muscle. A strip-shaped longitudinal excision on the lateral aspect of the cricopharyngeal muscle was done, followed by myotomy down to the upper esophagus. Pathological assessment of the excised specimen demonstrated fibrous change with degenerated muscular tissue. Upon completion of myectomy,
the underlying mucosa was protruded, which allowed an endoscope to pass through the esophagus. The balloon notch was no longer observed (Figs. 2C, 2D and 3B). The postoperative course was uneventful and the patient had a quick recovery. She was orally fed on the second day after surgery and was discharged on the ninth day, swallowing solids and liquids without difficulty. Two months post-myectomy, gastrostomy button was removed as no dysphagia was reported. As well, preferable weight gain was recorded. Esophagram revealed an open lumen at the cricopharyngeal region with no delayed transit (Fig. 4B), while high-resolution manometry (Star Medical Inc., Tokyo, Japan) showed a UES pressure at the normal level of 19.0 mmHg with proper relaxation at deglutition (Fig. 4A). Thereafter, the patient continued to consume solid food and liquid orally with no difficulty, and experienced a weight gain of 5.0 kg. She remained asymptomatic without medication four months after cricopharyngeal myectomy. 3. Discussion To date, fewer than 50 pediatric CPA cases have been reported, with the first cases reported in 1969 by Utian and Thomas [8]. While the Nelson Textbook of Pediatrics has included descriptions of this condition, the amount of information was limited [9]. The lack of awareness of this entity leaves patients untreated [10], and surgical interventions, such as gastrostomy or tracheostomy misdirected [4,11]. Our case involved one of the oldest pediatric CPA patients ever reported to have undergone surgery. The etiology of CPA remains largely unknown and multifactorial, involving central nervous system disorders, neuromuscular disorders, or infections [10]. The esophagus is a unique alimentary tract lined by the squamous epithelium. Herpes simplex virus-1 or varicella-zoster virus are neurotropic viruses with a predilection of squamous epithelium, and have been proposed to be potential antigens that cause achalasia [12–14]. Given the association of varicella infection and achalasia, the onset of symptoms emerging right after varicella infection in our case cannot be overlooked. Differential diagnoses of CPA include congenital esophageal stenosis, esophageal web or ring, dysphagia lusoria (retroesophageal subclavian artery), tracheoesophageal fistula and laryngo-tracheo-esophageal cleft [3]. Upper gastrointestinal endoscopy and videofluoroscopic swallow studies may be employed for diagnosis. While endoscopic finding is non-specific, tight spasm at the level of UES, which failed to relax under general anesthesia,
Please cite this article in press as: T. Watanabe, et al., Cricopharyngeal achalasia treated with myectomy and post-operative high-resolution manometry, Int. J. Pediatr. Otorhinolaryngol. (2014), http://dx.doi.org/10.1016/j.ijporl.2014.04.036
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Fig. 2. Balloon catheter installation and endoscopic findings. (A) Balloon catheter was installed under direct vision of endoscopy, which demonstrated an evident notch formation. (B) Endoscopy revealed strikingly narrowed lumen in the region of the cricopharyngeous muscle, which prevented access through the esophagus. (C) Balloon notch disappeared post myectomy. (D) Post-myectomy endoscopy demonstrated a widely patent lumen.
may be characteristic [3,10]. Typical findings at videofluoroscopic swallow study include dilated pharynx with holdup of the contrast bolus accompanied by visible cricopharyngeal prominence – (termed posterior bar) [9,10]. Only a small amount of contrast enters the esophagus in the presence of nasal reflux or aspiration, as seen in our patient. Our case is unique as multiple diverticula in the pharynx with the pouch-shaped piriform sinus was present, suggesting end-stage configuration of CPA due to long-term exposure by high-pressure pharynx. A cricopharyngeal spasm may sometimes be severe enough to produce posterior pharyngeal diverticulum (Zenker’s diverticulum) above the obstructive sphincter. However, such entity is extremely rare in children [9,15], and it was not observed in our patient either. Hence, we performed the surgery based on this perspective together with prior careful observation at endoscopy.
The Chicago Classification of esophageal motility was developed to facilitate the interpretation of clinical highresolution manometry, concurrent with the widespread adoption of this technology into clinical practice [16]. As far as the UES is concerned, manometry is still complementary to radiology for the diagnosis of disorders. Previous literature on CPA has pointed out the importance of manometric study [2]. In our case, the patient had experienced throat discomfort with a number of diverticula in the pharynx for many years without an actual diagnosis of CPA. Post-operative highresolution manometry demonstrated improved resting pressure and proper relaxation of cricopharyngeal muscle with normal pharyngeal contraction pressure and esophageal function, although the patient was predisposed to double swallowing in most wet deglutition.
Fig. 3. Operative view of cricopharyngeal myectomy. (A) The thickened transverse fibers of the cricopharyngeal muscle covered the upper esophagus circumferentially in the cricoid region (Arrow). Balloon notch informed the exact position of sphincteral stricture at the bottom of the cricopharyngeal muscle (C) (S: sternothyroid muscle; T: thyroid). (B) A 28-mm strip-shaped longitudinal excision of the cricopharyngeal muscle (C) was made, followed by a subsequent myotomy of the upper esophagus (E). Note the protrusion of the underlying mucosa (M).
Please cite this article in press as: T. Watanabe, et al., Cricopharyngeal achalasia treated with myectomy and post-operative high-resolution manometry, Int. J. Pediatr. Otorhinolaryngol. (2014), http://dx.doi.org/10.1016/j.ijporl.2014.04.036
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Fig. 4. Postoperative manometric study and esophagram. (A) High-resolution manometry illustrated improved UES pressure with proper relaxation at deglutition (white arrow). The propagation of the luminal contents in the esophageal body and the LES relaxation (white dotted arrow) were confirmed. (B) Videofluoroscopic swallow study demonstrated an open lumen at the cricopharyngeal region without delayed transit, but small pharyngocele persisted.
Various forms of therapies have been employed for CPA. Balloon dilatation has been proven to be a safe and common first-line treatment in infants and small children with mild dysphagia [4,6,17,18], based on the premise of repeated dilatation or conversion to myotomy. Botulinum toxin injection was reported to provide temporary relief of symptoms, hence requiring subsequent myotomy [7]; however, recent case series have demonstrated the successful treatment of CPA with botulinum toxin alone [5,19]. At present, the accepted standard and curative treatment is surgical transcervical cricopharyngeal myotomy [3,4,10,20,21]. In our case, the use of an intraesophageal balloon catheter during surgery was a novel idea to localize the point of stricture by palpating the balloon notch, leading to successful myectomy. Endoscopic cricopharyngeal myotomy appears to be an emerging minimally invasive approach without risking laryngeal nerve injury [2,22]. Since the various techniques have shown success in the treatment of this disease, we propose further studies to standardize the treatment modalities. In summary, we presented a nine-year-old girl with CPA who is one of the oldest pediatric patients to have received myectomy. The radiologic features were distinctive; multiple diverticula in the pharynx and pouch-shaped piriform sinus with silent aspiration were involved, due to the patient’s long history of dysphagia. Cricopharyngeal myectomy resolved her symptoms allowing good weight gain, while high-resolution manometry further supported postoperative swallowing function. CPA should be recognized as one of the entities that causes dysphagia not only in neonates and infants, but young children as well. Consent Written informed consent was obtained from the patient for publication of this case report and accompanying images. Conflict of interest statement The authors have no funding, financial relationships, or conflicts of interest to disclose. Acknowledgment We would like to thank Dr. Julian Tang from the Department of Education for Clinical Research, National Center for Child Health and Development, for proofreading and editing this manuscript.
References [1] K.C. Huoh, A.H. Messner, Cricopharyngeal achalasia in children: indications for treatment and management options, Curr. Opin. Otolaryngol. Head Neck Surg. 21 (2013) 576–580. [2] R. Chun, M. Sitton, N.A. Tipnis, et al., Endoscopic cricopharyngeal myotomy for management of cricopharyngeal achalasia (CA) in an 18-month-old child, Laryngoscope 123 (2013) 797–800. [3] N. Martin, J.M. Prince, T.D. Kane, et al., Congenital cricopharyngeal achalasia in a 4.5-year-old managed by cervical myotomy: a case report, Int. J. Pediatr. Otorhinolaryngol. 75 (2011) 289–292. [4] T. Muraji, S. Takamizawa, S. Satoh, et al., Congenital cricopharyngeal achalasia: diagnosis and surgical management, J. Pediatr. Surg. 37 (2002) E12. [5] A. Messner, A.S. Ho, P.S. Malhotra, et al., The use of botulinum toxin for pediatric cricopharyngeal achalasia, Int. J. Pediatr. Otorhinolaryngol. 75 (2011) 830–834. [6] O. Erdeve, M. Kologlu, B. Saygili, et al., Primary cricopharyngeal achalasia in a newborn treated by balloon dilatation: a case report and review of the literature, Int. J. Pediatr. Otorhinolaryngol. 71 (2007) 165–168. [7] R.K. Sewell, N.M. Bauman, Congenital cricopharyngeal achalasia: management with botulinum toxin before myotomy, Arch. Otolaryngol. Head Neck Surg. 131 (2005) 451–453. [8] H.L. Utian, R.G. Thomas, Cricopharyngeal incoordination in infancy, Pediatrics 43 (1969) 402–406. [9] E. Richard, R.M.K. Bernan, Nelson Textbook of Pediatrics, 17th edition, Saunders, 2003 1220 pp. [10] A. Brooks, A.J. Millar, H. Rode, The surgical management of cricopharyngeal achalasia in children, Int. J. Pediatr. Otorhinolaryngol. 56 (2000) 1–7. [11] E. Raboei, R. Luoma, Neonatal cricopharyngeal achalasia – a case report, Eur. J. Pediatr. Surg. 10 (2000) 130–132. [12] M. Facco, P. Brun, I. Baesso, et al., T cells in the myenteric plexus of achalasia patients show a skewed TCR repertoire and react to HSV-1 antigens, Am. J. Gastroenterol. 103 (2008) 1598–1609. [13] F. Castex, F. Guillemot, N. Talbodec, et al., Association of an attack of varicella and an achalasia, Am. J. Gastroenterol. 90 (1995) 1188–1189. [14] C.S. Robertson, B.A. Martin, M. Atkinson, Varicella-zoster virus DNA in the oesophageal myenteric plexus in achalasia, Gut 34 (1993) 299–302. [15] E.B. Lindholm, F. Hansbourgh, J.R. Upp Jr., et al., Congenital esophageal diverticulum – a case report and review of literature, J. Pediatr. Surg. 48 (2013) 665–668. [16] A.J. Bredenoord, M. Fox, P.J. Kahrilas, et al., Chicago classification criteria of esophageal motility disorders defined in high resolution esophageal pressure topography, Neurogastroenterol. Motil. 24 (Suppl. 1) (2012) 57– 65. [17] D. Davis, M. Nowicki, H. Giles, Cricopharyngeal achalasia responsive to balloon dilation in an infant, South. Med. J. 98 (2005) 472–474. [18] M.A. Skinner, N.A. Shorter, Primary neonatal cricopharyngeal achalasia: a case report and review of the literature, J. Pediatr. Surg. 27 (1992) 1509– 1511. [19] M.A. Scholes, T. McEvoy, H. Mousa, et al., Cricopharyngeal achalasia in children: botulinum toxin injection as a tool for diagnosis and treatment, Laryngoscope (2013), doi:10.1002/lary.24464 in press. [20] M. Drendel, E. Carmel, P. Kerimis, et al., Cricopharyngeal achalasia in children: surgical and medical treatment, Isr. Med. Assoc. J. 15 (2013) 430–433. [21] V. Jain, V. Bhatnagar, Cricopharyngeal myotomy for the treatment of cricopharyngeal achalasia, J. Pediatr. Surg. 44 (2009) 1656–1658. [22] M. Pitman, P. Weissbrod, Endoscopic CO2 laser cricopharyngeal myotomy, Laryngoscope 119 (2009) 45–53.
Please cite this article in press as: T. Watanabe, et al., Cricopharyngeal achalasia treated with myectomy and post-operative high-resolution manometry, Int. J. Pediatr. Otorhinolaryngol. (2014), http://dx.doi.org/10.1016/j.ijporl.2014.04.036
International Journal of Pediatric Otorhinolaryngology xxx (2014) xxx–xxx
Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Case Report
Cricopharyngeal achalasia treated with myectomy and post-operative high-resolution manometry Toshihiko Watanabe a, * , Takahiro Shimizu a , Masataka Takahashi a , Kaori Sato a , Michinobu Ohno a , Yasushi Fuchimoto a , Takanobu Maekawa b , Katsuhiro Arai c , Kunio Mizutari d , Noriko Morimoto d , Yutaka Kanamori a a
Division of Surgery, Department of Surgical Specialties, National Center for Child Health and Development, Tokyo, Japan Division of Pediatrics, Department of General Pediatrics and Interdisciplinary Medicine, National Center for Child Health and Development, Tokyo, Japan Division of Gastroenterology, Department of Medical Subspecialties, National Center for Child Health and Development, Tokyo, Japan d Division of Otolaryngology, Department of Surgical Specialties, National Center for Child Health and Development, Tokyo, Japan b c
A R T I C L E I N F O
A B S T R A C T
Article history: Received 6 March 2014 Received in revised form 16 April 2014 Accepted 20 April 2014 Available online xxx
Cricopharyngeal achalasia is an uncommon cause of dysphagia in neonates or children. A nine-year-old female patient was referred to us with a long history of dysphagia, recurrent pulmonary infection and growth stunting. A gastrostomy was introduced to improve her nutritional condition and to minimize potential inflammation in the pharynx. Subsequently, cervical cricopharyngeal myectomy was conducted. The surgical intervention allowed prompt resolution of symptoms without complications. High-resolution manometry post myectomy demonstrated a significant reduction in upper esophageal pressure together with proper relaxation at deglutition. The patient was able to consume solid food and liquid normally, and remained asymptomatic without medications six months after the surgery. ã 2014 Elsevier Ireland Ltd. All rights reserved.
Keywords: Cricopharyngeal achalasia Cricopharyngeal myectomy Dysphagia Pharyngocele Motility High-resolution manometry
1. Introduction
2. Case report
Cricopharyngeal achalasia (CPA) is a rare clinical entity with little information available concerning etiology, pathogenesis and the best management [1]. The inappropriate contraction of the cricopharyngeal muscle results in an inability to relax the upper esophageal sphincter (UES) during deglutition, leading to dysphagia, nasopharyngeal reflux during feeding, salivation, choking and failure to thrive [2–4]. Treatment options include botulinum toxin injection, mechanical dilation and cricopharyngeal myotomy [2,5– 7]. Here, we describe the clinical case of a pediatric CPA patient with a history of dysphagia, recurrent pulmonary infection and growth stunting, who was successfully treated by cervical cricopharyngeal myectomy.
Our patient is a nine-year-old female with a long history of dysphagia and recurrent pneumonia. She was born at full-term gestation after an uncomplicated pregnancy. She had no congenital abnormalities and her family history was unremarkable. No feeding problem was observed throughout infancy. At two years of age, she suffered from sudden onset of dysphagia after varicella infection, which resulted in intermittent fever and weight loss. Tumorous obstruction was initially suspected by magnetic resonance imaging. Nasogastric tube feeding was introduced to maintain her nutritional condition. Rehabilitation program for dysphagia resulted in some subjective improvement in her swallowing function, and daily tube feeding was withdrawn in six months. She was followed-up at her regional hospital for a period of time. At seven years of age, swallowing difficulty occurred which led to insufficient consumption of food. She displayed peculiar eating behavior; tilting the head to one side in order to bring food from the pharynx down to the esophagus. Oftentimes, residues of a meal would be regurgitated, with only partially chewed food ingested. Due to frequent choking or laryngopharyngeal gurgling with excessive salivation, expectorant and bronchodilating agents were
* Corresponding author at: 2-10-1 Okura, Setagaya-ku, Tokyo 157-8535, Japan. Tel.: +81 3 3416 0181; fax: +81 3 3416 2222. E-mail addresses: [email protected], [email protected] (T. Watanabe). http://dx.doi.org/10.1016/j.ijporl.2014.04.036 0165-5876/ ã 2014 Elsevier Ireland Ltd. All rights reserved.
Please cite this article in press as: T. Watanabe, et al., Cricopharyngeal achalasia treated with myectomy and post-operative high-resolution manometry, Int. J. Pediatr. Otorhinolaryngol. (2014), http://dx.doi.org/10.1016/j.ijporl.2014.04.036
G Model PEDOT 7104 No. of Pages 4
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T. Watanabe et al. / International Journal of Pediatric Otorhinolaryngology xxx (2014) xxx–xxx
Fig. 1. Preoperative videofluoroscopic swallow study. (A) Persistent narrowing at the level of cricopharyngeal muscle (arrow) was present during deglutition. (B) A marked delay in pharyngeal transit results in stasis of the contrast medium in the pharynx. Note the multiple pharyngocele formation (asterisk) and pouch-shaped piriform sinus (P). Trace silent aspiration is seen (T: trachea; E: esophagus).
required, suggesting chronic microaspiration. Bedside laryngoscopy performed by a pediatric otolaryngologist revealed the stasis of massive saliva in the pharynx, and a tentative diagnosis of pharyngocele was made. Subsequently, the patient was referred to a surgical department due to refractory dysphagia, recurrent episodes of pneumonia and failure to thrive at nine years of age. Her height (121 cm, 1.6 SD) and weight (19 kg, 1.7 SD) revealed growth stunting. Fluoroscopic image showed persistent narrowing at the level of cricopharynx with a pouched pharynx and multiple pharyngoceles; a likely cause of poor clearance and aspiration of oral contents (Fig. 1). An attempt to insert a probe into her esophagus for 24-h pH monitoring and manometric study failed. Endoscopic examination under general anesthesia revealed a strikingly narrowed lumen in the region of the cricopharyngeous muscle, and a small-diameter endoscope (5.0 mm) was unable to pass through (Fig. 2B). However, under endoscopic view, a pressure probe was successfully inserted into the esophagus. The pressure of the upper esophageal sphincter (UES) was extremely hypertensive (715.7 mmHg), whereas that of the lower esophageal sphincter (LES) was normal (13.2 mmHg). A diagnosis of CPA was made at that stage, and a percutaneous gastrostomy tube was introduced to provide a means of feeding. In order to improve the affected lesion of pouched pharynx and to stabilize potential inflammation of the affected lesion, only minimum liquid meals were allowed. Cricopharyngeal myectomy was performed after the pharynx was stabilized. Successful weight gain was achieved in three months. With the assistance of endoscopy, a balloon catheter was introduced, and a notch formation was evident (Fig. 2A). Access to the pharynx and upper esophagus was achieved through a right cervical incision centered on the radiological sign of the balloon notch. Exposure of the cricopharyngeal muscle was facilitated by a left-lateral retraction of the carotid sheath and sternohyoid muscle, together with a dissection of the superior belly of the omohyoid muscle. Anterior retraction of the thyroid tissue provided wide operative field. The thickened transverse fibers of the cricopharyngeal muscle covered the upper esophagus circumferentially in the cricoid region (Fig. 3A). The exact position of the sphincteral stricture was confirmed by palpating the balloon notch at the bottom of the muscle. A strip-shaped longitudinal excision on the lateral aspect of the cricopharyngeal muscle was done, followed by myotomy down to the upper esophagus. Pathological assessment of the excised specimen demonstrated fibrous change with degenerated muscular tissue. Upon completion of myectomy,
the underlying mucosa was protruded, which allowed an endoscope to pass through the esophagus. The balloon notch was no longer observed (Figs. 2C, 2D and 3B). The postoperative course was uneventful and the patient had a quick recovery. She was orally fed on the second day after surgery and was discharged on the ninth day, swallowing solids and liquids without difficulty. Two months post-myectomy, gastrostomy button was removed as no dysphagia was reported. As well, preferable weight gain was recorded. Esophagram revealed an open lumen at the cricopharyngeal region with no delayed transit (Fig. 4B), while high-resolution manometry (Star Medical Inc., Tokyo, Japan) showed a UES pressure at the normal level of 19.0 mmHg with proper relaxation at deglutition (Fig. 4A). Thereafter, the patient continued to consume solid food and liquid orally with no difficulty, and experienced a weight gain of 5.0 kg. She remained asymptomatic without medication four months after cricopharyngeal myectomy. 3. Discussion To date, fewer than 50 pediatric CPA cases have been reported, with the first cases reported in 1969 by Utian and Thomas [8]. While the Nelson Textbook of Pediatrics has included descriptions of this condition, the amount of information was limited [9]. The lack of awareness of this entity leaves patients untreated [10], and surgical interventions, such as gastrostomy or tracheostomy misdirected [4,11]. Our case involved one of the oldest pediatric CPA patients ever reported to have undergone surgery. The etiology of CPA remains largely unknown and multifactorial, involving central nervous system disorders, neuromuscular disorders, or infections [10]. The esophagus is a unique alimentary tract lined by the squamous epithelium. Herpes simplex virus-1 or varicella-zoster virus are neurotropic viruses with a predilection of squamous epithelium, and have been proposed to be potential antigens that cause achalasia [12–14]. Given the association of varicella infection and achalasia, the onset of symptoms emerging right after varicella infection in our case cannot be overlooked. Differential diagnoses of CPA include congenital esophageal stenosis, esophageal web or ring, dysphagia lusoria (retroesophageal subclavian artery), tracheoesophageal fistula and laryngo-tracheo-esophageal cleft [3]. Upper gastrointestinal endoscopy and videofluoroscopic swallow studies may be employed for diagnosis. While endoscopic finding is non-specific, tight spasm at the level of UES, which failed to relax under general anesthesia,
Please cite this article in press as: T. Watanabe, et al., Cricopharyngeal achalasia treated with myectomy and post-operative high-resolution manometry, Int. J. Pediatr. Otorhinolaryngol. (2014), http://dx.doi.org/10.1016/j.ijporl.2014.04.036
G Model PEDOT 7104 No. of Pages 4
T. Watanabe et al. / International Journal of Pediatric Otorhinolaryngology xxx (2014) xxx–xxx
3
Fig. 2. Balloon catheter installation and endoscopic findings. (A) Balloon catheter was installed under direct vision of endoscopy, which demonstrated an evident notch formation. (B) Endoscopy revealed strikingly narrowed lumen in the region of the cricopharyngeous muscle, which prevented access through the esophagus. (C) Balloon notch disappeared post myectomy. (D) Post-myectomy endoscopy demonstrated a widely patent lumen.
may be characteristic [3,10]. Typical findings at videofluoroscopic swallow study include dilated pharynx with holdup of the contrast bolus accompanied by visible cricopharyngeal prominence – (termed posterior bar) [9,10]. Only a small amount of contrast enters the esophagus in the presence of nasal reflux or aspiration, as seen in our patient. Our case is unique as multiple diverticula in the pharynx with the pouch-shaped piriform sinus was present, suggesting end-stage configuration of CPA due to long-term exposure by high-pressure pharynx. A cricopharyngeal spasm may sometimes be severe enough to produce posterior pharyngeal diverticulum (Zenker’s diverticulum) above the obstructive sphincter. However, such entity is extremely rare in children [9,15], and it was not observed in our patient either. Hence, we performed the surgery based on this perspective together with prior careful observation at endoscopy.
The Chicago Classification of esophageal motility was developed to facilitate the interpretation of clinical highresolution manometry, concurrent with the widespread adoption of this technology into clinical practice [16]. As far as the UES is concerned, manometry is still complementary to radiology for the diagnosis of disorders. Previous literature on CPA has pointed out the importance of manometric study [2]. In our case, the patient had experienced throat discomfort with a number of diverticula in the pharynx for many years without an actual diagnosis of CPA. Post-operative highresolution manometry demonstrated improved resting pressure and proper relaxation of cricopharyngeal muscle with normal pharyngeal contraction pressure and esophageal function, although the patient was predisposed to double swallowing in most wet deglutition.
Fig. 3. Operative view of cricopharyngeal myectomy. (A) The thickened transverse fibers of the cricopharyngeal muscle covered the upper esophagus circumferentially in the cricoid region (Arrow). Balloon notch informed the exact position of sphincteral stricture at the bottom of the cricopharyngeal muscle (C) (S: sternothyroid muscle; T: thyroid). (B) A 28-mm strip-shaped longitudinal excision of the cricopharyngeal muscle (C) was made, followed by a subsequent myotomy of the upper esophagus (E). Note the protrusion of the underlying mucosa (M).
Please cite this article in press as: T. Watanabe, et al., Cricopharyngeal achalasia treated with myectomy and post-operative high-resolution manometry, Int. J. Pediatr. Otorhinolaryngol. (2014), http://dx.doi.org/10.1016/j.ijporl.2014.04.036
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Fig. 4. Postoperative manometric study and esophagram. (A) High-resolution manometry illustrated improved UES pressure with proper relaxation at deglutition (white arrow). The propagation of the luminal contents in the esophageal body and the LES relaxation (white dotted arrow) were confirmed. (B) Videofluoroscopic swallow study demonstrated an open lumen at the cricopharyngeal region without delayed transit, but small pharyngocele persisted.
Various forms of therapies have been employed for CPA. Balloon dilatation has been proven to be a safe and common first-line treatment in infants and small children with mild dysphagia [4,6,17,18], based on the premise of repeated dilatation or conversion to myotomy. Botulinum toxin injection was reported to provide temporary relief of symptoms, hence requiring subsequent myotomy [7]; however, recent case series have demonstrated the successful treatment of CPA with botulinum toxin alone [5,19]. At present, the accepted standard and curative treatment is surgical transcervical cricopharyngeal myotomy [3,4,10,20,21]. In our case, the use of an intraesophageal balloon catheter during surgery was a novel idea to localize the point of stricture by palpating the balloon notch, leading to successful myectomy. Endoscopic cricopharyngeal myotomy appears to be an emerging minimally invasive approach without risking laryngeal nerve injury [2,22]. Since the various techniques have shown success in the treatment of this disease, we propose further studies to standardize the treatment modalities. In summary, we presented a nine-year-old girl with CPA who is one of the oldest pediatric patients to have received myectomy. The radiologic features were distinctive; multiple diverticula in the pharynx and pouch-shaped piriform sinus with silent aspiration were involved, due to the patient’s long history of dysphagia. Cricopharyngeal myectomy resolved her symptoms allowing good weight gain, while high-resolution manometry further supported postoperative swallowing function. CPA should be recognized as one of the entities that causes dysphagia not only in neonates and infants, but young children as well. Consent Written informed consent was obtained from the patient for publication of this case report and accompanying images. Conflict of interest statement The authors have no funding, financial relationships, or conflicts of interest to disclose. Acknowledgment We would like to thank Dr. Julian Tang from the Department of Education for Clinical Research, National Center for Child Health and Development, for proofreading and editing this manuscript.
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Please cite this article in press as: T. Watanabe, et al., Cricopharyngeal achalasia treated with myectomy and post-operative high-resolution manometry, Int. J. Pediatr. Otorhinolaryngol. (2014), http://dx.doi.org/10.1016/j.ijporl.2014.04.036