Congenital esophageal diverticulum – A case report and review of literature

Congenital esophageal diverticulum – A case report and review of literature

Journal of Pediatric Surgery (2013) 48, 665–668 www.elsevier.com/locate/jpedsurg Congenital esophageal diverticulum – A case report and review of li...

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Journal of Pediatric Surgery (2013) 48, 665–668

www.elsevier.com/locate/jpedsurg

Congenital esophageal diverticulum – A case report and review of literature Erika B. Lindholm a,⁎, Faith Hansbourgh a , James R. Upp Jr. a , Rafael Cilloniz b , John Lopoo a a

Department of Pediatric Surgery, Louisiana State University Health Sciences Center, New Orleans, LA, USA Department of Pediatric Pulmonology, Our Lady of the Lake, Baton Rouge, Louisiana, New Orleans, LA, USA

b

Received 13 July 2012; revised 5 December 2012; accepted 8 December 2012

Key words: Congenital esophageal diverticula; Zenker's diverticula

Abstract Zenker's Diverticulum (ZD) is a false diverticulum located in the posterior esophagus between the cricopharyngeus and inferior constrictor. Few cases have been reported in the pediatric population. Congenital esophageal diverticulum is commonly associated with additional esophageal anatomical deformities or iatrogenic injury in the early post-natal period. TD was born at 39 5/7 weeks gestational age and did well at home with the exception of “choking during feeds 2–4 times per day” with mild cyanosis. Flexible bronchoscopy performed under sedation revealed a retropharyngeal mass protruding towards the epiglottis resulting in upper airway obstruction. Esophogram demonstrated upper esophageal diverticulum. Patient underwent left neck dissection with cervical myotomy from the cricopharyngeus to the level of the thoracic inlet. A postoperative repeat esophogram illustrated resolution of the diverticulum. Currently, the patient is 9 months postoperative and is tolerating feeds by mouth, gaining weight and has no pulmonary symptoms. There have been a few reports of ZD in children occurring in combination with anatomical abnormalities or esophageal manipulation. This case is one of the youngest reported and occurs in a patient without any previous manipulation of the esophagus. This condition is exceedingly rare but should be included in the differential diagnosis for both pharyngoesophageal and tracheal symptoms. Published by Elsevier Inc.

Esophageal diverticulae are characterized according to their pathogenesis as either traction or pulsion. Traction diverticulae are true diverticula that occur in the mid esophagus secondary to inflammatory processes in the mediastinum causing an external force on the esophagus. There Abbreviations: ZD, Zenker diverticulum; EGD, esophagogastroduodenoscopy; CCD, congenital crico-pharyngeal diverticulum.

⁎ Corresponding author. LSUHSC Department of Surgery, New Orleans, LA 70112. Tel.: +1 985 705 3115; fax: + 1 504 568 4633. E-mail address: [email protected] (E.B. Lindholm).

0022-3468/$ – see front matter. Published by Elsevier Inc. http://dx.doi.org/10.1016/j.jpedsurg.2012.12.037

are two types of pulsion diverticulae, Zenker Diverticulae (ZD) and epiphrenic diverticulae, which occur with increased pressure against the upper and lower esophageal sphincters, respectively. ZD is a false diverticulum of the posterior esophagus between the cricopharyngeus and inferior constrictor. This area of anatomical weakness is known as Killian's Triangle. ZD occurs with high intraluminal pressures created during swallowing. ZD is rare with the incidence estimated at 2 per 100,000 [1]. It is most commonly diagnosed between the sixth to eight decades of life and predominately occurs in the male

666 population. ZD is more likely to occur with increased size of Killian's triangle which may explain why this pathology is more prevalent in males [2]. Patients typically experience both pharyngoesophageal and tracheal symptoms. Common symptoms include orophyarngeal dysphagia, regurgitation, aspiration, and halitosis. Over time, patients may present cachectic owing to severe dysphagia. Few cases of ZD have been reported in the pediatric population [1–7]. The majority of these cases involve anatomical esophageal deformities or iatrogenic injury in the early post-natal period. We report one case of a congenital esophageal diverticulum in an otherwise healthy 8 day old patient who presented with poor feeding and stridor. The patient had no instrumentation of the esophagus prior to presentation. The diagnosis was made on barium swallow and the patient was treated with an esophageal myotomy without complication. Esophageal diverticulum is a rare pathology that should be included in the differential diagnosis of dysphagia and stridor in all age groups.

1. Case TD was born at 39 5/7 weeks gestational age and was released from the hospital within 48 hours. He did well at home with the exception of “choking during feeds 2–4 times per day” with mild cyanosis. He was referred to the pediatric pulmonology clinic and scheduled for bronchoscopy on day of life 8 for continued symptoms. Flexible bronchoscopy performed under sedation revealed a retropharyngeal mass protruding towards the epiglottis resulting in upper airway obstruction (Fig. 1). Computed tomography of the neck illustrated an ovoid fluid

Fig. 1 Flexible bronchoscopy demonstrating a retropharyngeal mass protruding towards the epiglottis resulting in upper airway obstruction.

E.B. Lindholm et al.

Fig. 2 Computed tomography of the neck illustrating an ovoid fluid collection measuring 1.4 × 1.1 × .9 cm from C2 to C5.

collection measuring 1.4 × 1.1 × .9 cm extending from C2 to C5 (Fig. 2). Esophogram demonstrated upper esophageal diverticulum and mild aspiration of thin fluids (Fig. 3). Patient was taken to the operating suite where he underwent rigid bronchoscopy, esophagogastroduodenoscopy (EGD), and esophageal myotomy under general anesthesia. Rigid bronchoscopy demonstrated normal anatomy without laryngeal clefts. EGD was within normal limits, showing no evidence of diverticulum. The patient was repositioned for left neck dissection where a cervical myotomy was performed from the cricopharyngeus to the level of the thoracic inlet. On postoperative day number seven, repeat esophogram illustrated resolution of the diverticulum with continued mild

Fig. 3 Preoperative esophogram with upper esophageal diverticulum.

Congenital esophageal diverticulum

Fig. 4 Postoperative esophogram demonstrating resolution of the diverticulum.

aspiration of thin liquids (Fig. 4). A transpyloric nasogastric feeding tube was placed transiently and the patient was discharged home on goal tube feeds. Follow-up swallow showed resolution of aspiration and the patient was restarted on oral feeds. Repeat bronchoscopy shows resolution of diverticulum (Fig. 5). Currently, the patient is 9 months postoperative and is tolerating feeds by mouth, gaining weight and has no pulmonary symptoms.

2. Discussion The patient described above presented with a congenital esophageal diverticulum in the neonatal period. There is debate over the nomenclature, specifically whether this disorder should be classified as congenital versus acquired. Zenker's type diverticulum is classically considered to be

Fig. 5 Postoperative bronchoscopy demonstrating resolution of the diverticulum.

667 acquired owing to the high pressures created during swallowing. We postulate this condition can be acquired from propulsive forces created while swallowing in utero. There is no scientific evidence to support this hypothesis. Historically, pediatric diverticulae were classified according to their physiology by Meadows in 1970. He described four types of diverticulum that occur above the pharyngoesophageal junction. These are esophageal duplications, traumatic injury, pulsion, and congenital crico-pharyngeal diverticulum (CCD). CCD is true diverticulum that contains all layers of esophagus [8]. Pulsion diverticulae, which include ZD, are distinguished from CCD according to histological examination only. The first reported case of a true pulsion diverticulum in a pediatric patient was by Rush in 1929 [1,6]. A one day old male presented with respiratory distress and was diagnosed with an upper esophageal diverticulum on post-mortem analysis. More recently, a 6 month old child with esophageal stenosis following pneumonia was found to have developed an esophageal diverticulum secondary to increased intraluminal pressure [2]. Histological examination demonstrated mucosa with little muscular coating. There are additional case reports which diagnosis of diverticulum is preceded by pathological cause; in example, esophageal stenosis as above. Pressure leading to pulsion diverticulae has always presumably been owing to a combination of uncoordinated swallowing and impaired relaxation and spasm of cricopharyngeal muscles. Recent manometric pressure recordings have attempted to prove this hypothesis; however, study results have been inconclusive [9–11]. Technical difficulties and distortion of the original anatomy with arrival of diverticulum have resulted in conflicting data. Current hypotheses are more comprehensive and include abnormal esophageal motility disorders, which create increased pressure at Killian's triangle via impaired bolus passage [12]. We believe our patient most likely had impaired relaxation of the sphincter which leads to the in utero formation of the esophageal diverticulum. This theory is based on observations following induction of anesthesia with resulting relaxation of the upper esophageal sphincter causing the diverticulum to be relaxed. On the contrary, during esophogram, the increased pressure during swallowing was able to demonstrate a large posterior diverticulum. American Gastroenterological Association guidelines for the adult diagnosis of ZD include barium swallow as the gold standard [13]. In most cases, the diverticulum protrudes posterior and may be superimposed on the esophagus, therefore rotating the patient during exam is imperative. In addition, a second smaller diverticulum is found in 1–2% patients [4]. In the patient described above, the diverticulum was first noticed on bronchoscopy performed under sedation, which is not uncommon in the pediatric population. Endoscopic examination can be dangerous owing to increased risk of perforation with misdirection of the scope into the diverticula and should be undertaken with careful precaution.

668 There is similar risk with passage of nasogastric tubes and thus these should be placed under direct visualization. Myotomy has been the mainstay of treatment and is supported by numerous studies observing favorable outcome in 80–100% of patients and reduced recurrence rate [14]. Left anterior cervical incision is utilized to visualize the esophagus during cricopharyngeal myotomy. The decision between diverticulectomy and diverticulopexy is based on the patient co-morbidities and size of diverticulum. Diverticulopexy is a cephalad tacking of the diverticulum to the prevertebral fascia to prevent food content from entering the pouch during deglutition, thus preventing potential complications of ZD. Newer approaches to treatment are based on an intraluminal approach via esophagoscopy. This technique, which was pioneered in the 1960s, is based on disruption of the wall shared between the diverticula and the esophagus [15]. A number of different approaches exist; the most common being coagulation followed by division. More recently, in the 1990s endoscopic stapling devices have been utilized to transect the shared wall [16]. The intraluminal approaches are safe and significantly reduce hospital stay when compared to open techniques. This procedure is not feasible in nearly 30% patients owing to anatomical differences which limit exposure [17]. Endoscopic treatment is not recommended in patients with large diverticula because of incomplete emptying of pouch remnants. Both treatments have been described in the pediatric population. Endoscopic methods have been gaining popularity with case reports demonstrating success of minimally invasive treatments. Under the guidance of laryngoscope, a 5 year old male received a myotomy via carbon dioxide laser therapy without complication [7]. Additional case reports demonstrate feasibility of the argon plasma coagulation system in two teenagers with esophageal diverticula [18]. The feasibility of minimally invasive therapies in a neonate is unlikely owing to size limitations with current instrumentation.

3. Conclusion There have been few reports of ZD in children ranging from newborns to 10 years old in combination with additional anatomical abnormalities or esophageal manipulation. This case is one of the youngest reported and occurs in a

E.B. Lindholm et al. patient without any previous manipulation of the esophagus. This condition is exceedingly rare but should be included in the differential diagnosis in patients with both pharyngoesophageal and tracheal symptoms.

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