Gastroenterology & Hepatology: Open Access
Hiatal Hernia Complicated by Benign Esophageal Stricture in a Developmentally Delayed Male Case Report
Abstract Background: Benign esophageal strictures can be defined as a lumen-narrowing lesion of the esophagus with no associated malignancy. Hiatal hernias can cause severe reflux and have been identified as a cause of esophageal strictures in adults. Here, we report a case of esophageal stricture secondary to hiatal hernia in a developmentally delayed adolescent, making this one of the few-reported cases of esophageal stricture secondary to hiatal hernia in the pediatric population.
Case Report: A 15-year-old male with Autism Spectrum Disorder presented with a four month history of vomiting and weight loss. On admission, his upper GI series revealed a distal esophageal stricture and hiatal hernia. He was treated with Omeprazole and underwent 5 sequential endoscopic dilatations with resolution of symptoms. Discussion: Esophageal strictures can be classified as simple or complex based on the depth of injury and magnitude of fibrosis. Chronic acid reflux leading to chronic inflammation can predispose to stricture formation. The treatment of esophageal stricture includes gastric acid suppression and esophageal dilatation. The goal of any dilation is to establish and maintain a patent lumen compatible with patient’s life style with lowest risk to the patient. Complications associated with dilations include bleeding, perforation and infection.
Volume 7 Issue 3 - 2017 Department of Pediatrics, Westchester Medical Center, USA Center for Digestive Health and Nutrition, Arnold Palmer Children’s Hospital, USA 3 Department of Pediatric Gastroenterology, Westchester Medical Center, USA 1 2
*Corresponding author: Jennifer Deluty Apsan, Westchester Medical Center, 36 Laurel Hill Terrace apt 5F, NY, NY 10033, Tel: (516) 314-1134; Fax: (888) 980-5993; Email: Received: May 17, 2017 | Published: August 07, 2017
Conclusion: Hiatal hernias and secondary esophageal strictures are rare in the pediatric age group and thus are not well characterized. Esophageal stricture should be considered as a cause of emesis and weight loss in the pediatric population.
Keywords: Hiatal Hernia; Benign Esophageal Stricture
Introduction
Case Report
Benign esophageal strictures can be defined as a lumennarrowing lesion of the esophagus with no associated malignancy. Severe reflux is the most common asosiated etiology of stricture formation in adults. Hiatal hernias can cause severe reflux and have been identified as a cause of esophageal strictures in adults [1]. Sliding hiatal hernias are characterized by the weakening of diaphragmatic crura and a resulting projection of the stomach, through the diaphram. Hiatal hernias in children are almost exclusively congenital in which case repair is warranted early in life for symptomatic cases to ensure adequate nutritional tolerance and growth. Most sliding hiatal hernias in adults remain largely asymptomatic. Acquired hiatal hernias are only symptomatic less than 10% of the time [2]. Here, we present a case of a developmentally delayed adolescent male who presents with weight loss and recurrent vomiting, found to have a hiatal hernia and esophageal stricture. This case underscores the importance of considering and pursuing strictures and underlying reflux pathologies in cases of recurrent persistent oral intolerance and weight loss, especially in developmentally delayed children who are unable to give an accurate or reliable history of symptoms.
A 15-year-old male with a history of Bipolar Disorder Autism Spectrum Disorder, Global Developmental Delay, Attention Deficit Hyperactivity Disorder, Impulse Control Disorder and Intellectual Disability presented with a five pound weight loss associated with three to four months of retching and vomiting. Three to four months prior to presentation, the patient’s mother noticed some food regurgitation after oral intake. This behavior progressed to consistent emesis after any solid food intake. Eventually, he was only able to tolerate small sips of liquid. Therapy with omeprazole was started without improvement. He was hospitalized for hydration and further evaluation. On admission, physical examination revealed that he weighed 40 kg, was 1.52 m in height, had a thin body habitus, and non specific dysmorphic facial features. His vital signs revealed tachycardia to 102 beats per minute, blood pressure of 118/74 and body temperature of 36.1 °C. The remainder of the physical examination was unremarkable.
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An upper GI was performed which showed a distal esophageal stricture and hiatal hernia. The remainder of the exam was normal except for the presence of gastro-esophageal reflux (Figure 1). Endoscopy (EGD) confirmed the presence of a stricture in
Gastroenterol Hepatol Open Access 2017, 7(3): 00239
Hiatal Hernia Complicated by Benign Esophageal Stricture in a Developmentally Delayed Male
the distal esophagus. This only permitted for entrance of an Olympus XP190 upper endoscope with a 5.4 mm diameter. The patient underwent five sequential endoscopies with progressive dilatations from 6 to 15 mm using through-the-scope balloon dilatations. Biopsies distal to the stricture revealed chronically inflamed gastric cardiac mucosa, confirming the presence of a hiatal hernia.
Figure 1: Esophagram 2/11/16. Esophogram: Hiatal Hernia and Stricture. High-grade gastroesophageal reflux was observed during the course of the examination. Arrows point to stricture.
The patient was treated with omeprazole 40 mg twice daily and slowly progressed back to a full diet after dilatations were preformed. He continued to do well without any recurrent episodes of emesis and was tolerating a regular diet in a 6 month follow-up period.
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cause subsequent stricture [4]. Prior trachea-esopheal fistula repair is a risk factor for stricture formation in the pediatric population [5]. Consideration must also be given to primary dermatologic disorders like scleroderma and epiidermolysis bullosa dystrophica, which can cause progressive erosive mucosal injury, healing with overlying fibrosis and scaring. Similarly, eosinophillic esophagitis is known to causes significant stricture and food impaction secondary to untreated inflammation [6]. Finally, external compression of the esophagus is a known cause of stricture formation. Mediastinal pathology from tuberculosis or idiopathic fibrosing mediastintis are among causes of mediatsinal changes that physically compress the esophagus [3,4]. Strictures can be classified as simple or complex based on the depth of injury and the magnitude of fibrosis present. Complex strictures are characterized by a length of 2 cm or greater, a severely narrowed luminal diameter or an angulated or irregular contour [7]. Typically, 1to 3 dilations are sufficient to relieve dysphagia in simple strictures where 5 dilations is the upper limit in > 85% of patents [8]. The Kochman criteria better define characteristics of recurrent and refractory strictures. They are characterized by extensive fibrosis or luminal compromise that anatomically restricts the luminal width of the esophagus, inability to dilate 14 mm over 5 sessions, inability to maintain patency to 14 mm over subsequent 4 weeks [9]. Cases also must exclude both neuromuscular dysfunction and inflammatory states that cause strictures. Once the stricture is classified, a therapeutic plan can be initiated with regards to dilation frequency and a determination of prognosis can be made [9,10] (Figure 2 & 3).
Discussion
An esophageal stricture is defined as a decrease in the caliber of the esophagus secondary to fibrotic contraction or deposition of abnormal tissue. The etiology of most benign esophageal strictures involves persistent chronic acid reflux in about 90% of cases in adults. The esophagus is normally exposed to low amounts of gastric acid, mediated by the competence of the lower esophageal stricture and pace of clearance by esophageal peristalsis. Any defect in these mechanisms, including hiatal hernias that compromise lower esophageal sphincter function, can lead to chronic buildup of gastric contents. In the esophageal lumen The exposure of the esophagus to refluxed gastric contents, leads to chronic inflammation secondary to exposure to acid, pepsin and pancreatic enzymes and ultimately stricture formation of the tissue. Thus, 7-23% of patients with significant reflux esophagitis will have concurrent esophageal stricture formation [1].
Though less common, multiple other etiologies exist for the formation of an esophageal stricture. These pathologies may be thought of as intrinsic versus extrinsic esophageal disease. For example, any history of prior surgical intervention, radiation exposure or caustic ingestion may contribute to significant intrinsic fibrosis upon healing, thus leading to stricture formation [3]. Anatomic causes like congenital esophageal atresia can
Figure 2: 2/16/16 Stricture dilation session 1 of 5. GIF 190 XP scope was passed through the stricture with sequential dilatation to 7mm, 8mm and 9 mm for 1 minute each. After the end of 9 mm dilatation, GIF 160 scope was passed with ease through the stricture site.
The goal of any dilation is always to establish and maintain a patent lumen compatible with patient’s life style with the lowest associated cost and risk to the patient. Options for dilations include balloon dilation and bougie dilation. The bougie exerts both a circumferential and shearing force that dilates from the proximal to the distal end of the stricture. Maloney dilators are mercury-weighted bougies in which the bougie is blindly inserted in sequentially increasing size until dilation is accomplished. A Savary Gilliard is a similar bougie but introduced, over a spring tipped guide wire via the working channel of an endoscope. In contrast, a through the scope (TTS) balloon dilation delivers radial forces simultaneously throughout the stricture.
Citation: Apsan JD, Pandey A, Bostwick H (2017) Hiatal Hernia Complicated by Benign Esophageal Stricture in a Developmentally Delayed Male. Gastroenterol Hepatol Open Access 7(3): 00239. DOI: 10.15406/ghoa.2017.07.00239
Hiatal Hernia Complicated by Benign Esophageal Stricture in a Developmentally Delayed Male
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outcomes for each patient. Frequent follow-up and chronic medical management of reflux is essential to good long term clinical outcomes.
Acknowledgement
Special thanks to the Division of Pediatric Gastroenterology and Pediatric Residency Program at the Maria Fareri Children’s Hospital of Westchester Medical Center.
Conflict of Interest
No conflict of Interests exist in the above work.
References Figure 3: 3/28/16 Stricture dilation session 4 of 5. GIF 160 endoscopes was passed through the stricure, and with the TTS balloon, the stricture was sequentially dilated to 12 mm, 13.5 mm and 15 mm each for 1 minute.
In head to head studies, there does not seem to be a superior dilatation method. Furthermore, there does not seem to be increased post procedure complications in trials of balloon versus bougie dilations. [9] . There are cases, such as epidermolysis bullosa where longitudinal shear force should be avoided, thus making the balloon method more advantageous [3]. Complications asosiated with dilations include bleeding, perforation and infection. Guidelines follow the “rule of three” and advise not to increase a luminal diameter by more than 3 millimeters per session to minimize perforation risk [6]. There is debate with regard to the efficacy of steroid injection and its ability to prevent recurrence of stricture in refractory cases. Studies to date remain small, uncontrolled and involve strictures of differing etiologies, making it difficult to make evidence based conclusions [5]. In one published randomized study, 13% of patients who received steroids needed repeat dilation versus 60% who did not receive a steroid injection, pointing to the possible efficacy of this intervention [12]. Incisional therapy is an option for a safe alternative if dilatation does not prove effective. Some studies show efficacy with application of mitomycin C, an antineoplastic agent, though long-term effects are poorly studied [13]. In a prospective study, effectiveness with endoscopic ultrasound was demonstrated by its ability to define the extent of wall involvement in benign esophageal strictures and to accurately predict the response to endoscopic dilatation [14].
Conclusion
Acquired hiatal hernia and secondary strictures are rare in the pediatric age group and thus are not well characterized or defined. Clinical suspicion must remain high to rule out organic causes of emesis and weight loss in developmentally delayed children. Persistence of symptoms or severity of presentation may warrant consultation with pediatric gastroenterology and endoscopic evaluation. If stricture is confirmed, simple, complex and refractory strictures must be classified and dilatations methods must be clinically determined to optimize clinical
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Citation: Apsan JD, Pandey A, Bostwick H (2017) Hiatal Hernia Complicated by Benign Esophageal Stricture in a Developmentally Delayed Male. Gastroenterol Hepatol Open Access 7(3): 00239. DOI: 10.15406/ghoa.2017.07.00239
Hiatal Hernia Complicated by Benign Esophageal Stricture in a Developmentally Delayed Male
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14. Surinder S Rana, Deepak K Bhasin, Kartar Singh (2011) Role of endoscopic ultrasonography (EUS) in management of benign esophageal strictures. Ann Gastroenterol 24(4): 280-284.
Citation: Apsan JD, Pandey A, Bostwick H (2017) Hiatal Hernia Complicated by Benign Esophageal Stricture in a Developmentally Delayed Male. Gastroenterol Hepatol Open Access 7(3): 00239. DOI: 10.15406/ghoa.2017.07.00239
