Ann Thorac Surg 2006;81:1858-1862
© 2006 The Society of Thoracic Surgeons
Original article: General thoracic
Long-Term Observation and Functional State of the Esophagus After Primary Repair of Spontaneous Esophageal Rupture
Xavier Benoit D'Journo, MD
a
,
Christophe Doddoli, MD
a
,
b
,
Jean Philippe Avaro, MD
a
,
Pascal Lienne, MD
a
,
Marc A. Giovannini, MD
c
,
Roger Giudicelli, MD
a
,
Pierre A. Fuentes, MD
a
,
Pascal A. Thomas, MD
a
,
b
,
*
a Department of Thoracic Surgery, Ste. Marguerite University Hospital, Marseille, France
b Unité Propre de Recherche et d'Enseignement Supérieur, Equipe d'Acceuil (UPRES EA 2001), Institut Fédératif de Recherche, Jean Roche, Marseille, France
c Endoscopic Unit, Paoli-Calmettes Institute, Marseille, France
Accepted for publication December 13, 2005.
* Address correspondence to Dr Thomas, Department of Thoracic Surgery, Ste Marguerite Hospital-CHU Sud, 270 Bd Ste Marguerite, 13274 Marseille Cedex 9, France (Email: pascal-alexandre.thomas{at}mail.ap-hm.fr).
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Abstract
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BACKGROUND: Long-term outcome of patients treated for a spontaneous esophageal rupture (Boerhaave's syndrome) is seldom reported.
METHODS: From 1989 to 2004, 62 esophageal perforations were treated in a single institution. Eighteen patients presented with a spontaneous esophageal rupture. Among them, 15 could be treated with a transthoracic primary repair and constituted the material of the present study. A chart review was performed with special attention to survival, residual symptoms, and anatomic and motility disorders.
RESULTS: Three patients died postoperatively (20%). At last follow-up, 10 patients were alive and 2 had died from unrelated causes. At a median delay of 13 months (3 to 74), 7 patients accepted to undergo complementary investigations. None of them had any anatomic abnormality as checked by barium swallow. Six patients complained of mild symptoms from gastroesophageal reflux. Six patients (85%) presented with esophageal motility disorders on manometry and 4 (54%) had nocturne chronic reflux disease on pH monitoring. Two patients underwent endoscopic ultrasonography, of which one presented with a focal absence of one layer of the esophageal wall within the area of the suture. With time, no patient experienced recurrence, but one developed a cancer in the cervical esophagus.
CONCLUSIONS: These results suggest that esophageal functional disorders are the rule after primary repair of a Boerhaave's syndrome. Whether or not these findings are causal, coincidental, or related to the surgical treatment remains unclear. However, performance of routine postoperative explorations is strongly encouraged for a better understanding of this challenging condition.
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Introduction
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Spontaneous esophageal rupture (Boerhaave's syndrome) is a potentially lethal condition for which multiple treatment options exist; with growing evidence favoring conservative techniques, assuming that the rupture is intrinsically an accident arising on a healthy viscera [1]. The rupture occurs on the occasion of a sudden rise in intraluminal pressure, but its true pathophysiology remains unclear even if various factors including regular consumption of irritating substances, focal defect of the muscularis mucosa, preexisting gastroesophageal reflux (GERD), or other functional disorders are suspected predisposing factors [1–4]. Although clinical presentation and early management have been well-documented, long-term outcome and natural course of the disease have seldom been investigated in survivors. Indeed, 30-day survival still is the primary endpoint of most reported studies dealing with spontaneous rupture of the esophagus given that it remains the most lethal perforation of the gastrointestinal tract. Marginally, esophageal stricture and GERD are mentioned as possible long-term sequels [5–7]. Since recurrences have been reported [8, 9], we hypothesized that some form of preexisting anatomical and/or functional abnormalities may persist after a successful esophageal repair [10]. The aim of this study was thus to report on a single institution experience with the long-term outcome of patients treated for a spontaneous esophageal rupture, with special attention to survival, residual symptoms, and anatomic and motility disorders.
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Patients and Methods
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This retrospective trial was conducted according to the current regulations for clinical research in France. There was no intent of research at the time of data collection and all tests were proposed in the frame of medical care, with a presumed individual benefit for the patients. From 1989 to 2004, a total of 62 patients underwent surgical treatment for esophageal perforation at our institution. Among them, 18 patients (28% of all esophageal rupture referred to our hospital) suffered from a spontaneous esophageal rupture, without any preexisting trauma or endoscopic investigation. There were 13 males and 5 females, with a mean age of 64 ± 10 years. Nine patients were treated between 1989 and 1997 and 6 between 1998 and 2004, resulting in an observed incidence of roughly one case per year. Three patients were treated by bipolar exclusion or resection of the esophagus, and were not included in the study. Fifteen patients could be treated with a transthoracic primary repair and constituted the material of the present study. In those 15 patients, median time from injury to surgical repair was 36 hours (range, 6 hours–12 days). Principles of surgical repair included a left thoracotomy, extensive pleural and mediastinal lavage with debridement of necrotic tissue, exposition of the retracted edges of the tear that was located in the lower one-third of the thoracic esophagus in all cases, a double layer interrupted absorbable suture (mucosal and muscular plan), and reinforcement of the suture by a pleural or a pericardial flap in some cases. Thirty-day and in-hospital mortality rates were 7% (1 patient) and 20% (3 patients), respectively.
At last follow-up in June 2005, 10 patients were alive, 2 having died from unrelated causes 73 and 103 months, respectively, after surgery. Individual informed consent was obtained in 7 patients who accepted to undergo complementary investigations. The delay between surgery and follow-up examination ranged from 3 to 74 months (median, 13 months).
In the obvious absence of baseline objective data, patients were interviewed regarding the presence of possible symptoms of GERD or esophageal motility disorders prior to the rupture. Follow-up examination comprised a dedicated questionnaire to assess general well-being, acid complaints, abdominal pain, diarrhea, nausea, sleep disturbances, and other complaints such as cough. Barium swallow, esophageal manometry, long-term pH-monitoring and fiberscopy were performed in all cases. Endoscopic ultrasonography was performed in the two last patients. Esophageal manometry consisted of progressive stationary withdrawal from the stomach of a four-catheter probe infused by a low compliance Mui hydraulic pump (Mui Scientific, Mississauga, Ontario, Canada). The four distal side-hole recording orifices were located at 5 cm intervals with a 45 degree offset angle from each other to allow complete assessment of the esophageal body and sphincters; ie, lower esophageal sphincter (LES) and upper esophageal sphincter (UES). Signals were recorded by four Siemens (Munich, Germany) pressure gauges and transmitted to a Gould (Gould Electronics, Cleveland, OH) electrostatic recorder by an amplifier. Readings were expressed in millimeters of mercury (mm Hg). Long-term (18–24 hours) ambulatory measurement of acid reflux was performed with a Proxima pH-monitor (Proxima Light 2, Synectics AB, Stockholm, Sweden) connected to an INGOLD (Columbus, OH) glass electrode probe. Readings were expressed in DeMeester values [11].
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Results
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Characteristics and early outcome of the patients at the time of the primary surgical repair are summarized in Table 1. Two patients had preexisting symptoms suggesting the presence of reflux (28%). None complained from dysphagia, paroxystic chest pain, or other symptoms suggestive of esophageal disorders.
Table 2
details the long-term esophageal function evaluation. Six patients (85%) complained from mild symptoms of gastroesophageal reflux whereas only one was really symptom-free. None of them had any anatomic abnormality as checked by barium swallow. Endoscopy disclosed a grade II reflux esophagitis in one patient, with contiguous erosions of the superficial epithelium and whitish exudates surrounded by red halos of erythema above the squamocolumnar junction. At 24-hour pH monitoring (Table 3), a pathologic gastroesophageal reflux (defined as a DeMeester composite score greater than 14.7) was observed in four cases (57%). Long-lasting reflux periods were observed in these four cases, suggesting poor esophageal clearance. Six patients (85%) presented with corporeal esophageal motility disorders at manometry (Table 4). Manometry revealed a lack of propulsive peristaltic waves and muscular coordination, especially in the upper part of the esophagus, mimicking those abnormalities seen in nonspecific esophageal motility disorders (NEMD), as well as a marked decline of the lower esophageal sphincter pressure. Endoscopic ultrasonography was performed in the two last patients: one (patient 6) presented with a focal absence of one layer of the esophageal wall within the area of the suture.
None of the patients of the present series experienced recurrent esophageal rupture. One patient (patient 7) was readmitted 3 years after surgery because of the occurrence of acute dysphagia due to the development of a squamous cell carcinoma in the cervical esophagus. One, five, and ten year survival rates were 86%, 66%, and 36%, respectively (Fig 1).
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Fig 1. Long-term survival in 7 patients having survived a spontaneous esophageal rupture (bold black line = survival function; vertical lines = censored.)
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Comment
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Boerhaave's syndrome, improperly named "spontaneous rupture of the esophagus," results from an acute barotrauma with a sudden pressure rise in the esophagus at the occasion of protracted forceful vomiting in most cases [10]. The vomiting mechanism is a succession of coordinated reflexes with an acute increase in intraabdominal pressure due to convulsive contraction of the abdominal muscles and diaphragm, creating an expulsive discharge of gastric contents through the relaxed sphincters and esophageal body. Lack of synchronization of these reflexes with offbeat closure due to an esophageal or cricopharyngeal spasm could lead to sufficient intraesophageal pressure to cause a full thickness rupture [1–4].
Predilection for left-side perforations may be caused by the lack of adjacent supporting connective tissues such as a visceral serosa, thinning of musculature in the lower esophagus, and anterior angulation of the esophagus at left diaphragmatic crus [12]. Based on histologic examination of surgical specimens from two patients, Kuwano and colleagues [13] have reported the absence of muscularis mucosa around and along the longitudinal extension of the sites of rupture. The role of muscularis mucosa is unclear but it could act as a protective bumper. It has been demonstrated that the organization of the lamina muscularis mucosae varied considerably among the cervical, the thoracic, and the abdominal part of the esophagus [14]. Near the cardia, the muscular bundles in the lamina muscularis mucosae usually ran in various directions forming a reticular configuration. It may be hypothesized that this specific density and arrangement aims to compensate the relative vulnerability of the viscera in this area, where the esophagus is widest and its bursting point can easily be achieved according to Laplace's law. Echoendoscopy has been shown to allow good visualization of all parietal structures including the muscularis mucosa which appears as the third hypoechogenic layer [15] (Fig 2A). The two last patients included in our study underwent endoscopic ultrasonography to assess the structure of the esophagus. One of these two patients presented with a focal absence of the third layer of the esophageal wall within the area of the suture (Fig 2B). The meaning of this finding is unclear. If not coincidental, it would have been anticipated as a constant clue if related to the esophageal wall repair. It is thus attractive, but obviously not demonstrated, to propose that lack of muscularis mucosa in the esophageal wall may be one cause of spontaneous rupture of the esophagus, as Kuwano and colleagues [13] did previously.
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Fig 2. (A) Normal structure of the esophagus with five echogenic layers. The muscularis mucosa appears as the third hypoechogenic layer. (B) Focal absence of one layer of the esophageal wall within the area of the suture.
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Gastroesophageal reflux disease is the main complaint of patients surviving a Boerhaave's syndrome [5–7]. None of our patients presented with a peptic stenosis in the long run, although as high as 27% incidence has been reported by others [5]. Although GERD is commonly considered as a sequel of the rupture and/or its treatment, GERD may also contribute to the origin of the rupture. This assumption has been already emphasized by Salo and colleagues [10], who concluded on the basis of a retrospective study of 5 patients that survivors of a spontaneous esophageal rupture have a long-term severe disturbance of esophageal motility caused by chronic reflux esophagitis. In our experience, 6 of 7 patients (85%) presented with symptoms of GERD postoperatively, whereas only 2 (28%) of them had a remote history of symptomatic GERD. This observation strongly suggests the prevailing responsibility of the surgical repair in the determinism of symptomatic refluxes experienced by our patients, but it does not exclude a possible preexisting silent GERD in the absence of baseline data. Of note is the fact that the incidence of GERD documented by pH-monitoring was lower in our study (4/7) than in that from Salo and colleagues (4/5) [10], and only one patient presented with esophagitis on fiberscopy versus 4. However, similar to what was observed by Salo and colleagues, pH-monitoring disclosed long-lasting single reflux periods, suggesting poor esophageal clearance in these patients. Underlying chronic inflammation due to GERD may add to weaken the esophageal wall and/or induce some form of esophageal motility dysfunction.
Motility disorders were found in 6 of 7 patients of our study (85%). None of these patients met strict criteria for established esophageal motility disorders such as achalasia, diffuse esophageal spasm, or nutcracker esophagus. Abnormalities included triple-pick contractions, low-amplitude peristalsis (less than 30 mm Hg), nontransmitted contractions, spontaneous contractions, and prolonged spontaneous contractions. While motor disorders or hypotonic lower esophageal sphincter were found in all 6 patients, only 3 could be considered as NEMD because of the presence of up to three criteria [16]. In the series by Salo and colleagues [10], 4 of 5 patients were considered to have NEMD. Nonspecific esophageal motility disorder is a vague category used to include patients with poorly defined esophageal contraction abnormalities. It has been demonstrated that NEMD was not a manometric curiosity but a disorder characterized by selective impairment of semisolid emptying [17]. In this variety of disease, patients may be symptomatic with chest pain and/or dysphagia, and should be treated by an approach similar to that used in patients with readily classifiable motility disturbances.
As for GERD, however, whether or not these motility disorders are causal or related to the surgical treatment remains unclear. Nevertheless, our findings clearly show that esophageal functional disorders, as a whole, are frequent and perhaps the rule after surgical repair of spontaneous esophageal perforation. Because preexisting functional abnormalities are also presumed predisposing factors of so-called spontaneous esophageal rupture, persistent ones have been incriminated in the occurrence of delayed recurrences after an initial successful repair [8, 9]. Such an event did not happen in any of the patients of the present series. Long-term survival was similar to that related to the patient's age at the time of diagnosis and treatment. At follow-up, one patient developed a squamous cell carcinoma in the cervical esophagus 3 years after the esophageal rupture, and was treated with combined radiochemotherapy. This fate probably reflects the common association with overindulgence in alcohol in both conditions.
Primary repair, emerging as the treatment of choice of Boerhaave's syndrome leaves in place a possibly diseased esophagus. Our findings demonstrate that esophageal functional disorders, among which are GERD and NEMD, are frequent and perhaps the rule. Whether or not these findings are causal, coincidental, or related to the surgical treatment remains unclear. Whether or not their treatment may prevent a potentially devastating recurrence is speculative. However, performance of routine postoperative explorations is strongly encouraged for a better understanding of this challenging condition.
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References
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|---|
- Jougon J, McBride T, Delcambre F, Minniti A, Velly JF. Primary esophageal repair for Boerhaave's syndrome whatever the free interval between perforation and treatment Eur J Cardiothorac Surg 2004;25:475-479.[Abstract/Free Full Text]
- Pate JW, Walker WA, Cole Jr FH, Owen EW, Johnson WH. Spontaneous rupture of the esophagusa 30-year experience. Ann Thorac Surg 1989;47:689-692.[Abstract]
- Kollmar O, Lindemann W, Richter S, Steffen I, Pistorius G, Schilling MK. Boerhaave's syndromeprimary repair vs.esophageal resection—case reports and meta-analysis of the literature. J Gastrointest Surg 2003;7:726-734.[Medline]
- Lawrence DR, Ohri SK, Moxon RE, Townsend ER, Fountain SW. Primary esophageal repair for Boerhaave's syndrome Ann Thorac Surg 1999;67:818-820.[Abstract/Free Full Text]
- Saabye J, Nielsen HO, Andersen K. Long-term observation following perforation and rupture of the esophagus Scand J Thorac Cardiovasc Surg 1988;22:79-80.[Medline]
- Justicz AG, Symbas PN. Spontaneous rupture of the esophagusimmediate and late results. Am Surg 1991;57:4-7.[Medline]
- Kumar P, Sarkar PK. Late results of primary esophageal repair for spontaneous rupture of the esophagus (Boerhaave's syndrome) Int Surg 2004;89:15-20.[Medline]
- Reeder LB, Warren SE, Ferguson MK. Recurrent spontaneous perforation of the esophagus Ann Thorac Surg 1995;59:221-222.[Abstract/Free Full Text]
- Khan OA, Barlow CW, Weeden DF, Amer KM. Recurrent spontaneous esophageal rupture Eur J Cardiothorac Surg 2005;28:178-179.[Abstract/Free Full Text]
- Salo JA, Seppala KM, Pitkaranta PP, Kivilaakso EO. Spontaneous rupture and functional state of the esophagus Surgery 1992;112:897-900.[Medline]
- DeMeester TR, Wang CI, Wernly JA, et al. Technique, indications, and clinical use of 24 hour esophageal pH monitoring J Thorac Cardiovasc Surg 1980;79:656-670.[Abstract]
- Mackler SA. Spontaneous rupture of the esophagus; an experimental and clinical study Surg Gynecol Obstet 1952;95:345-356.[Medline]
- Kuwano H, Matsumata T, Adachi E, et al. Lack of muscularis mucosa and the occurrence of Boerhaave's syndrome Am J Surg 1989;158:420-422.[Medline]
- Nagai K, Noguchi T, Hashimoto T, Uchida Y, Shimada T. The organization of the lamina muscularis mucosae in the human esophagus Arch Histol Cytol 2003;66:281-288.[Medline]
- Kimmey MB, Martin RW, Haggitt RC, Wang KY, Franklin DW, Silverstein FE. Histologic correlates of gastrointestinal ultrasound images Gastroenterology 1989;96:433-441.[Medline]
- In: Castell DO, Richter JE, Dalton CD, editors. Esophageal motility testing. New York, NY: Elsevier Science; 1987. pp. 61-78.
- Hsu JJ, O'Connor MK, Kang YW, Kim CH. Nonspecific motor disorder of the esophagusa real disorder or a manometric curiosity?. Gastroenterology 1993;104:1281-1284.[Medline]
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Abstract
Introduction
