Boegli, Yann

de Buys Roessingh, Anthony

Dolci, Mirko

El Ezzi, Oumama

Ikonomidis, Christos

Tooh, Mengu Ma

Journal of Pediatric Surgery Case Reports

English

Serveur académique lausannois

This article appeared in a journal published by Elsevier. The attachedcopy is furnished to the author for internal non-commercial researchand education use, including for instruction at the authors institutionand sharing with colleagues.Other uses, including reproduction and distribution, or selling orlicensing copies, or posting to personal, institutional or third partywebsites are prohibited.In most cases authors are permitted to post their version of thearticle (e.g. in Word or Tex form) to their personal website orinstitutional repository. Authors requiring further informationregarding Elsevier’s archiving and manuscript policies areencouraged to visit:http://www.elsevier.com/authorsrightsAuthor's personal copyDuplication of the cervical esophagus: An unusual cause of respiratoryarrest in a childqMengu Ma Tooh a, Oumama El Ezzi a, Mirko Dolci b, Yann Boegli b, Christos Ikonomidis c,Anthony de Buys Roessingh a,*aDepartment of Pediatric Surgery, Centre Hospitalier Universitaire Vaudois, CH-1011 Lausanne, SwitzerlandbDepartment of Anaesthesia, Centre Hospitalier Universitaire Vaudois, Lausanne, SwitzerlandcDepartment of Otorhinolaryngology, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerlanda r t i c l e i n f oArticle history:Received 21 May 2013Received in revised form28 July 2013Accepted 7 August 2013Key words:DuplicationEsophagusAirways compressionRespiratory distressa b s t r a c tDuplications of the cervical esophagus are rare malformations. When the malformation is situated in thethoracic part of the alimentary tract, symptoms can be dyspnea, regurgitation, cough, epigastricdiscomfort and anorexia. We report the history of a ten-month-old child who presented progressiveonset of snoring and stridor, in the course of an upper respiratory tract infection, not responding toantibiotics and drug therapy. The baby developed acute respiratory distress at the hospital in supineposition, due to major airway compression, leading to respiratory arrest. Resuscitation was provided andthe child underwent an emergency decompression of the cystic mass. 2013 The Authors. Published by Elsevier Inc. All rights reserved.Digestive duplications may occur anywhere from the mouth tothe anus. They can be tubular, cystic, diverticular, isolated or mul-tiple. The small intestine is themost common site of duplication andthe esophagus the second [1]. Differential diagnoses are broncho-genic cysts, branchial cysts or vascularmalformations. A duplicationsituated in the thoracic part of the alimentary tract is potentiallydangerous because it may induce respiratory distress, due tocompression and/or tracheomalacia [2,3]. Esophageal duplicationsmay induce respiratory distress in children and therefore should bementioned in the differential diagnosis of airway obstruction.1. Case reportA ten-month-old girl, without previous medical history, con-sulted at the pediatric emergency service for difficulty in breathingand unusual respiratory sounds. Symptoms had started 2 weeksbefore with snoring and coughing without fever. She was firsttreated with antibiotics by her pediatrician. Two weeks later, thebaby seemed to have more difficulty in breathing. There was nohistory of epigastric discomfort, cough or regurgitation. Because thesymptoms were becoming more and more severe, she was taken tothe closest emergency unit by the parents. Physical examinationshowed that the baby’s breathing was noisy with a slight stridor.Her respiratory rate was 44/min. Supine position seemed to in-crease respiratory distress while prone position did not. Duringclinical evaluation, the baby had a respiratory arrest; respiratoryresuscitation was achieved without intubation. A chest X-rayshowed an enlarged mediastinum with important tracheal devia-tion (Fig. 1). She was then transferred to our hospital for furtherinvestigations.She was apathetic and tachypneic. Oxygen saturation was be-tween 97% and 100% under 50% of oxygen support by mask. As shewas definitely better in prone position, with the head turned onthe right side, we decided not to move her for the clinical exam-ination and first investigations.Local status showed a left cervical mass, mobile, painless atpalpation and visible in the left cervical region next to the sterno-cleidomastoid muscle, with no visible cutaneous sinus. It measured4  5 cm. A computed tomography (CT) scan showed a cystic massfull of liquid in the parapharyngeal area, going from the left sub-mandibular region to the upper mediastinum space, situated inq This is an open-access article distributed under the terms of the CreativeCommons Attribution License, which permits unrestricted use, distribution, andreproduction in any medium, provided the original author and source are credited.* Corresponding author. Tel.: þ41 79 556 37 67; fax: þ41 21 314 30 71.E-mail address: anthony.debuys-roessingh@Chuv.ch (A. de Buys Roessingh).Contents lists available at ScienceDirectJournal of Pediatric Surgery CASE REPORTSjournal homepage: www.jpscasereports .com2213-5766/$ e see front matter  2013 The Authors. Published by Elsevier Inc. All rights reserved.http://dx.doi.org/10.1016/j.epsc.2013.08.009J Ped Surg Case Reports 1 (2013) 311e313Author's personal copydepth and close to the carotid artery and jugular vein with majorcompression and deviation of the trachea (Figs. 2 and 3).An emergency drainage of the mass was organized. Anesthesiawas induced by inhalation of Sevoflurane, in prone position. Shewas positioned onher left side to allowmore efficient ventilation. Asshe was still breathing spontaneously, without airways obstruction,we positioned her after a few minutes in a supine position andproceeded to an endotracheal intubation, without any drugs otherthan the volatile agent. The puncture of the cyst under ultrasound(US) control obtained a whitish milky fluid. A left dissection of theneckwas realized to expose thewall of the cyst whichwas biopsied.A catheter was left in the cyst. Oxygenation and ventilation wereuneventful throughout theprocedure. The childwas transferred, stillintubated, to the intensive care unit.Pathologyconfirmed thediagnosis of esophageal duplication anda second surgery was performed 2 weeks later for the completeremoval of the malformation through a cervicotomy. At the sametime,weperformed a laryngoscopy to exclude thepresence of a tractoriginating from the left pyriform fossa seen inpyriform sinus cyst. Afistulous connection between the cyst and the native esophaguswasidentified, ligated and covered with a flap of fibrous tissue. Macro-scopically, themasswas soft,weighed29g andmeasured3.53 cm.Microscopic examination showed that the innerwall was linedwithstratified squamous epithelium. The outer wall consisted of a thickband of smooth muscle fibers surrounded by skeletal muscle fasci-cles. The epithelium was strongly positive for various cytokeratins,especially for cytokeratins 19 and 125. These last two are usuallyseen in the gastro-intestinal epithelium. Neither cartilaginous tissuenor lymphoid tissuewere present. The structure of thewall, with itstype of epithelium, smooth muscle and skeletal muscle bands, wascompatible with esophageal duplication.A contrast study of the esophagus five days later showed noleakage. A transitory palsy of the left recurrent laryngeal nerve wasobserved. The child left the hospital 5 days after the operation, bywhich time she had completely recovered. One month after theoperation, the baby ate normally and pre-operative stridor haddisappeared.2. DiscussionThis case describes anunusual cause of respiratoryarrest in a ten-month-old girl suffering from esophageal duplication. The fact thatthe child had no history of chronic respiratory symptoms, nodigestive tract disturbances andnopalpable cervicalmass,when shewas first admitted, made the evaluation of the risk of respiratoryarrest a real challenge.Duplications of the alimentary tract are rare malformations andthe cervical portion of the esophagus is rarely concerned. More than80% of the cases are diagnosed before the age of two years. Since thefirst description by Balasius in 1711, only few cases have been re-ported in the literature [3e5]. Multiple theories have been proposedto account for esophageal duplication origin, but no single theoryadequately explains all the known duplications [6]. The most com-mon anatomical presentation is a single cyst, located on the lowerpart of the esophagus [7,8]. However, Robinson et al. reported a caseof multiple cysts in one patient [9]. These malformations can beassociated with other malformations such as esophageal atresia,tracheoesophageal fistula, spinal deformities, congenital heart dis-ease, vertebral abnormalities, malrotation of the bowel, diaphrag-matic hernia and Meckel’s diverticulum [10].The clinical presentation is usually nonspecific and depends onthe anatomic localization, size and nearby organs. An ectopic gastricmucosa is found in approximately two thirds of the duplications,Fig. 1. Chest X-ray, with deviation of trachea (arrow) and widening of the uppermediastinum.Fig. 2. Initial computed tomography (CT) scan, with a mass (*) creating a majorcompression of the airway (arrow).Fig. 3. Computed tomography (CT) scan image, in prone position, showing an ovoidcervical lesion (*) next to the esophagus and the neck vessels displaced laterally(arrows).M.M. Tooh et al. / J Ped Surg Case Reports 1 (2013) 311e313312Author's personal copypredisposing the mucosa to ulceration and perforation [11]. In thecase of cervical esophageal duplications, the clinical sign is thepresence of a cervicalmass. Early surgical intervention is required toprevent complications of the duplication [12].The interest of our case is the presentation of an anterior medi-astinal mass, with an initial diagnosis of upper respiratory tract viralinfection and a sudden respiratory arrest influenced by the positionof the baby. As the acute onset of stridor after an episode of upperrespiratory tract viral infection may be very common, the baby wasfirst treated conservatively. In the literature, such cases have eitherrespiratory symptoms or the presence of a cervical mass. Moultonet al. described a case of a 13month old boywith a history of chronic(10 months) biphasic stridor and dyspnea while feeding [2], andWootton-Georges et al. described the case of a newbornwith a largecervical mass [5], both being duplications of the cervical esophagus.As we did not have a diagnosis for the mass, we therefore planned asurgical drainage of the cyst in order to secure the airway tract, evenif this could increase the risk of bleeding, infection and distortion ofthe anatomy.Induction of anesthesia in patients with anterior mediastinalmass can lead to cardiorespiratory collapse [13]. Inhalation induc-tion and spontaneous ventilation maintenance are widely recom-mended [14,15]. The position of the patient can be supine, lateraldecubitus, semi-Fowler or prone [13,16]. In case of airwaycompression during the procedure, the first measure is the reposi-tioning of the patient in the position that caused the least symptomspre-operatively [15]. As our patientwasbreathing freely in theproneposition, we didn’t reposition her for the induction of anesthesia.Depending on the position of themass, rigid bronchoscopy could beanother option. In case of persistent life-threatening cardiovascularissue, the use of cardiopulmonary bypass is often recommended inthe literature, but a canulation e even with a dedicated team onstand-by emay require too much time and impede a resuscitation.Immediate sternotomy and elevation of the mass by the surgeon isprobably a better option [15].Wedescribe here a true duplication of the esophaguswith amassthatwas attached to the native esophagus and an identifiedfistulousconnection to the native esophagus that was ligated. Thewall of thecyst lined by a stratified squamous epithelium, encircled by bands ofsmoothmuscle aswell as skeletalmusclefibers are all features of theupper part of the esophagus. The embryological etiology of thismalformation is not completely understood, but both esophagealduplication and bronchogenic cysts are believed to occur at the timeof thedivisionof the embryonic foregut [17].Histological similaritiessupport their common origin, but a true esophageal duplicationcontains all the layers of a normal esophagus (mucosa, submucosaandmuscularis propria), but no cartilaginous tissue. The presence ofcartilaginous tissue or lymphoid tissue in the malformation aswell the type of epithelium seenmay suggest anothermalformationsuch as a bronchogenic or a branchial cyst (third or fourth). Theclassic location of bowel duplication is on the mesenteric side,sharing a commonmusclewall andblood supplywith the associatedbowel [18].3. ConclusionAlthough they are very rare conditions, esophageal duplicationsshould be mentioned in the differential diagnosis of children withrespiratory distress not responding to the usual medical treatmentand without defined etiology. Furthermore, according to the clinicalpresentation of this pathology, the preoperative management canbecome a challenge for the various medical teams.Financial/nonfinancial disclosureAt the time of submission of this research paper, there has beenno duplicate publication or submission elsewhere, the material isoriginal. All authors have read and approved the manuscript. Thereis no ethical problem or conflict of interest.References[1] Puligandla PS, Nguyen LT, St-Vil D, Flageole H, Bensoussan AL, Nguyen VH,et al. Gastrointestinal duplications. J Pediatr Surg 2003;38:740e4.[2] Moulton MS, Moir C, Matsumoto J, Thompson DM. Esophageal duplicationcyst: A rare cause of biphasic stridor and feeding difficulty. Int J Ped Oto-rhinolaryngol 2005;69:1129e33.[3] de Buys Roessingh AS, Rosato L, Meagher-Villemure K, Hohlfeld J. Cervicaloesophageal duplication causing respiratory distress in a child. J PediatrGastroenterol Nutr 2005;41:259e62.[4] Vasseur Maurer S, Schwab G, Osterheld MC, Reinberg O, Joseph JM. A 16month-old boy vomits a double tongue: intraluminal duplication of the cer-vical oesophagus in children. Dis Esophagus 2008;21:186e8.[5] Wootton-Gorges SL, Eckel GM, Poulos ND, Kappler S, Milstein JM. Duplicationof the cervical esophagus: a case report and review of literature. Pediatr Radiol2002;32:533e5.[6] Nobuhara KK, Gorski YC, La Quaglia MP, Shamberger RC. Bronchogenic cystsand oesophageal duplications: common origins and treatment. J Pediatr Surg1997;32:1408e13.[7] Carachi R, Azmy A. Foregut duplications. Pediatr Surg Int 2002;18:371e4.[8] Sharma K, Ranka P, Meratiya S. Isolated cervical esophageal duplication:a rarety. J Pediatr Surg 2005;40:591e2.[9] Robison RJ, Pavlina PM, Scherer LR, Grosfeld JL. Multiple esophageal duplica-tion cyst. J Thorac Cardiovasc Surg 1987;94:144e7.[10] Olajide AR, Yisau AA, Abdulraseed NA, Kashim IO, Olaniyi AJ, Morohunfade AO.Gastrointestinal duplications: Experience in seven children and a review ofthe literature. Saudi J Gastroenterol 2010;16:105e9.[11] Cioffi U, Bonavia L, De Simone M. Presentation and surgical management ofbronchogenic and cervical duplication cysts in adults. Chest 1998;113:1492e6.[12] Holcomb 3rd GW, Gheissari A, O’Neill Jr JA, Shorter NA, Bishop HC. Surgicalmanagement of alimentary tract duplications. Ann Surg 1989;209:167e74.[13] Cheung SL, Lerman J. Mediastinal masses and anesthesia in children. Anaes-thesiol Clin N Am 1998;16:893e910.[14] Hammer GB. Anaesthetic management for the child with a mediastinal mass.Pediatr Anaesth 2004;14:95e7.[15] Slinger P, Karsli C. Management of the patient with a large anterior medias-tinal mass: recurring myths. Curr Opin Anaesthesiol 2007;20:1e3.[16] Ferrari LR, Bedforth RF. General anesthesia prior to treatment of anteriormediastinal masses in pediatric cancer patients. Anesthesiology 1990;72:991e5.[17] Yasufuku M, Hatakeyama T, Maeda K, Yamamoto T, Iwai Y. Bronchopulmonaryforegut malformation: a large bronchogenic cyst communicating with anoesophageal duplication cyst. J Ped Surg 2003;38: E2.[18] De Fazio M, Guglielmi A, Catalano G. Communicating oesophageal duplication:a case report. G Chir 2003;24:243e5.M.M. Tooh et al. / J Ped Surg Case Reports 1 (2013) 311e313 313

Duplication of the cervical esophagus: An unusual cause of respiratory arrest in a child

Duplication of the cervical esophagus: An unusual cause of respiratory arrest in a child

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