Sharp recanalization of a short esophageal occluding stricture in a patient with epidermolysis bullosa Raymond H. Thornton, MD, Melvin B. Heyman, MD, MPH, Mark W. Wilson, MD, Ali Zarrinpar, PhD, Robert K. Kerlan, MD, Jeanne M. LaBerge, MD, Maurice Zwass, MD, Roy L. Gordon, MD San Francisco, California, USA
Background: Although esophageal strictures caused by epidermolysis bullosa are often treated with balloon dilations, complete obstruction has few effective therapies except esophagectomy with colonic replacement. Objective: Resolution of esophageal obstructive lesion without surgical intervention. Design: Case study. Setting: Interventional radiology. Patient: Epidermolysis bullosa with esophageal stricture. Intervention: Endoscopic- and guidewire-guided sharp recanalization. Main Outcome Measurement: Radiologic evidence of stricture resolution. Results: Successful recanalization. Limitations: Experience of operators (anesthesiologist, endoscopist, interventional radiologist). Conclusions: Sharp recanalization of a complete stricture in patients with epidermolysis bullosa is feasible in a controlled setting.
Epidermolysis bullosa comprises a constellation of epidermal fragility disorders characterized by blistering, photosensitivity, progressive poikiloderma, and esophageal stenosis.1 Minor trauma, such as that caused by swallowing solid food, can result in blister formation and scarring. Though the skin, and GI, respiratory, and genitourinary systems are frequently involved, the most debilitating manifestation is the development of esophageal strictures, which may cause profound dysphagia and even complete obstruction.2 Because of the pathologic fragility of epithelial surfaces, the optimal therapy for esophageal strictures has not yet been established. Nevertheless, several methods have been proposed and investigated, including colonic replacement of the esophagus3-6 and balloon dilation.7-9 We describe a case of epidermolysis bullosa that caused complete occlusion of the upper esophagus and that was successfully recanalized by using the Rosch-Uchida needle set (Cook, Bloomington, Ind),10 followed by balloon dilation.
PATIENT AND METHOD
Copyright ª 2006 by the American Society for Gastrointestinal Endoscopy 0016-5107/$32.00 doi:10.1016/j.gie.2006.07.029
A 33-year-old man with epidermolysis bullosa presented for dilation of an esophageal stricture. He had undergone his first such dilation 3 years earlier at another institution. Two years earlier, a gastrostomy tube was placed to alleviate increasing dysphagia. By the following year, he was unable to swallow even his saliva. An upper-GI series revealed complete occlusion of the upper esophagus. In the interventional radiology suite, the patient was intubated, and general anesthesia was obtained. Endoscopy through the gastrostomy revealed complete occlusion of the esophagus 25 cm cephalad from the gastroesophageal junction. Methylene blue was injected under pressure from below the obstruction. The endoscope was then passed through the mouth, and the obstruction was encountered 2 to 3 cm distal to the cricopharyngeus. No methylene blue had passed through the obstruction. With the endoscope in place from above, the interventional radiologist passed a catheter and a 0.035-inch guidewire through the gastrostomy for access cephalad to the occlusion. The guidewire was not visualized by endoscopy (Fig. 1). A catheter and a guidewire were then passed by mouth down to the obstruction and directed toward a 30-mm Amplatz gooseneck snare (Microvena Corp,
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Sharp recanalization of a short esophageal occluding stricture
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Capsule Summary What is already known on this topic d
Epidermolysis bullosa presents with blistering, photosensitivity, progressive poikiloderma, and esophageal strictures that may cause dysphagia.
What this study adds to our knowledge d
A complete esophageal stricture was successfully recanalized in a patient with epidermolysis bullosa by using the Rosch-Uchida needle set, followed by balloon dilation.
White Bear Lake, Minn), which had been positioned distal to the obstruction. The composite fluoroscopic and endoscopic assessment demonstrated that the occluded segment spanned only several millimeters and appeared to be weblike in nature. After extensive consideration by all involved and in view of limited surgical options, the decision was made to attempt sharp recanalization of this short-segment, weblike occlusion. The needle was passed under fluoroscopic guidance by using a C-arm fluoroscope that was alternately positioned in the anteriorposterior and lateral positions. The needle was not
visualized before or after the puncture was made, although it was well seen by fluoroscopy. The Rosch-Uchida needle set, typically used in the transjugular intrahepatic portosystemic shunt (TIPS) procedure, was deployed proximal to the occlusion over a 0.035-inch guidewire. The set has a 14F metal cannula with a gently curved tip. A sharp trocar stylet (0.38-inch diameter) was passed coaxially through a 5F catheter; these fit as a unit coaxially through the stiff cannula. By using the cannula for direction, the inner assembly was passed through the web and was grabbed by using the snare. (Fig. 2). This allowed a 0.035-inch guidewire to be pulled through the esophagus and out the gastrostomy. A long, 5F sheath was placed over this wire and was pulled back during injection of radiopaque contrast material. No esophageal perforation was identified (Fig. 3). The stenosis was then dilated over the wire by using 6-, 12-, and 16-mm balloons, with good fluoroscopic results. The balloon size was chosen based on the estimated diameter of the esophagus. The dilation was proceeding smoothly, and we determined that the optimal dilation should leave a large enough lumen that would not be obstructed by postdilation edema or bullae formation. Before the wire was removed, a nasogastric tube was placed over the wire, and it was left in place after the procedure to serve as a stent across the area of intervention. Postprocedure odynophagia was effectively managed with a viscous lidocaine swish-and-swallow solution. On postprocedure day 4, the patient was tolerating a soft diet, and the nasogastric tube was removed. He was discharged home in stable condition, able to swallow his own secretions. Five months later, the patient returned for routine surveillance dilation. He reported that within 1 month of the initial procedure, he had returned to a full diet and routinely enjoyed steak dinners, and, at the time of this follow-up procedure, he was not having any dysphagia. After anesthesia was obtained, a Berenstein catheter (Boston Scientific Corp, Natick, Mass) and 0.035-inch hydrophilic guidewire were passed by mouth through the
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Figure 1. Fluoroscopic spot film in the lateral projection shows the endoscope in the esophagus from above and the curled guidewire in the esophagus passed retrograde via the gastrostomy. The endotracheal tube is seen anteriorly.
Figure 2. A snare is passed retrograde up the esophagus. A Rosch-Uehida needle is passed antegrade from above through the narrow occlusion.
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Sharp recanalization of a short esophageal occluding stricture
Figure 3. A guidewire was passed through the needle from above, engaged by the snare below, and pulled through the gastrostomy. Contrast agent injected from above shows the narrowed esophagus but no extravasation of contrast. The wire is in the lumen of the esophagus.
esophagus and into the stomach. Balloon dilations were easily performed over the wire by using 12- and 16-mm balloons (Fig. 4), with significant improvement in the degree of esophageal narrowing compared with 5 months earlier. After a brief observation period, he was discharged home in stable condition. Since then he has continued to enjoy regular food. Repeat dilations have successfully been implemented approximately every 12 months, by which time he develops a recurrent stricture manifested by dysphagia to solid foods. To avoid the development of another complete obstruction, he contacts us at the first sign of dysphagia. Endoscopy has not been performed at the follow-up dilations, because the dilation procedure is readily and safely performed under fluoroscopic guidance.
Figure 4. Esophageal narrowing. A, Before full inflation of the 16-mm balloon. B, After full inflation of the 16-mm balloon, without residual waist.
Esophageal strictures are common among patients with epidermolysis bullosa. Clinically significant dysphagia and esophageal strictures, defined by impaired nutrient intake, aspiration, pneumonia, or total esophageal obstruction with intolerance to one’s own secretions, require esophageal dilations. Medical therapy with corticosteroids or phenytoin7,11 has been ineffective, and bougienage has been associated with esophageal injury and perforation.9,12 Although balloon dilation has been established as the mainstay of treatment for recurrent esophageal strictures, patients with severe esophageal obstruction have had little choice other than esophageal replacement with colonic interposition.3,4,6
Because of the exquisite sensitivity of the epithelial surfaces to even minimal trauma, special care must be taken during interventions on patients with epidermolysis bullosa. Considerations include the use of graduated balloon dilations, short inflation times, and fluoroscopic monitoring to minimize the possibility of esophageal rupture.9 Sharp recanalization is recognized as useful for crossing central venous occlusions, and the use of the RoschUchida needle set has been specifically described in this setting.13 Endoscopic retrograde puncture of total esophageal stenoses caused by radiation has been described,14 including a recent report of a similar method used in a radiation-associated complete obstruction.15 However, the application of this technique to even a short-segment esophageal occlusion raises special concerns for the potentially lethal sequelae of esophageal perforation. The
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DISCUSSION
Sharp recanalization of a short esophageal occluding stricture
operators in this case determined that a single attempt at sharp recanalization might afford the patient considerable clinical benefit, potentially obviating esophageal replacement. Although sharp esophageal recanalization cannot be recommended as a routine interventional therapy for esophageal occlusion, its dramatic success in this case raises the possibility of its application in carefully selected circumstances, particularly in patients with epidermolysis bullosa and other disorders of collagen formation. DISCLOSURE M. Heyman is supported, in part, by NIH grant DK 060617.
REFERENCES 1. Yeh SW, Ahmed B, Sami N, et al. Blistering disorders: diagnosis and treatment. Dermatol Ther 2003;16:214-23. 2. Ergun GA, Lin AN, Dannenberg AJ, et al. Gastrointestinal manifestations of epidermolysis bullosa. A study of 101 patients. Medicine (Baltimore) 1992;71:121-7. 3. Demirogullari B, Sonmez K, Turkyilmaz Z, et al. Colon interposition for esophageal stenosis in a patient with epidermolysis bullosa. J Pediatr Surg 2001;36:1861-3. 4. Elton C, Marshall RE, Hibbert J, et al. Pharyngogastric colonic interposition for total oesophageal occlusion in epidermolysis bullosa. Dis Esophagus 2000;13:175-7. 5. De Leon R, Mispireta LA, Absolon KB. Five year follow-up of colonic transplants in patients with epidermolysis bullosa producing esophageal obstruction. Med Ann Dist Columbia 1974;43:241-4. 6. Absolon KB, Finney LA, Waddill GM Jr, et al. Esophageal reconstruction: colon transplant in two brothers with epidermolysis bullosa. Surgery 1969;65:832-6.
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Thornton et al 7. Castillo RO, Davies YK, Lin YC, et al. Management of esophageal strictures in children with recessive dystrophic epidermolysis bullosa. J Pediatr Gastroenterol Nutr 2002;34:535-41. 8. Fujimoto T, Lane GJ, Miyano T, et al. Esophageal strictures in children with recessive dystrophic epidermolysis bullosa: experience of balloon dilatation in nine cases. J Pediatr Gastroenterol Nutr 1998; 27:524-9. 9. Heyman MB, Zwass M, Applebaum M, et al. Chronic recurrent esophageal strictures treated with balloon dilation in children with autosomal recessive epidermolysis bullosa dystrophica. Am J Gastroenterol 1993;88:953-7. 10. Uchida BT, Putnam JS, Ro¨sch J. ‘‘Atraumatic’’ transjugular needle for portal vein puncture in swine. Radiology 1987;163:580-1. 11. Caldwell-Brown D, Stern RS, Lin AN, et al. Lack of efficacy of phenytoin in recessive dystrophic epidermolysis bullosa. Epidermolysis Bullosa Study Group. N Engl J Med 1992;327:163-7. 12. Feurle GE, Weidauer H, Baldauf G, et al. Management of esophageal stenosis in recessive dystrophic epidermolysis bullosa. Gastroenterology 1984;87:1376-80. 13. Farrell T, Lang EV, Barnhart W. Sharp recanalization of central venous occlusions. J Vasc Interv Radiol 1999;10:149-54. 14. Lew RJ, Shah JN, Chalian A, et al. Technique of endoscopic retrograde puncture and dilatation of total esophageal stenosis in patients with radiation-induced strictures. Head Neck 2004;26:179-83. 15. Baumgart DC, Veltzke-Schlieker W, Wiedenmann B, et al. Successful recanalization of a completely obliterated esophageal stricture by using an endoscopic rendezvous maneuver. Gastrointest Endosc 2005;61:473-5.
Received April 22, 2006. Accepted July 11, 2006. Current affiliations: Section of Interventional Radiology, Memorial SloanKettering Cancer Center, New York, New York (Dr Thornton), Department of Pediatrics (M.B.H., R.K.K., J.M.L., M.Z., R.L.G.), Section of Interventional Radiology, Department of Radiology (M.W.W.), School of Medicine (Dr A.Z.), Department of Anesthesia (M.Z.), University of California, San Francisco, California, USA. Reprint requests: Melvin B. Heyman, MD, Department of Pediatrics, 500 Parnassus Ave, MU 4-East, University of California, San Francisco, CA 94143-0136.
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