What is the relationship between the Mallory-Weiss syndrome and spontaneous rupture of the lower esophagus?
B. Gayet, T. Perniceni (Paris)
Boerhaave's syndrome, the Mallory-Weiss syndrome and also rare spontaneous intramural hematomas are all seen as forms of esophageal rupture that usually occur during vomiting. Other circumstances of onset have been described: the lifting of a heavy weight, straining at stool, difficult labor, attack of asthma, epileptic seizure, or any event accompanied by a marked and often sudden elevation of abdominal pressure [1-4]. This is why these ruptures have been described as "barogenic", "emetogenic", or strain ruptures. Subjacent esophagogastric or neurological disease may predispose to these accidents.
"Spontaneous" rupture and the Mallory-Weiss syndrome have a number of points in common which at first sight suggest that it is reasonable to group them together, especially since succession of these two syndromes in the same patient at an interval of a few months has been described. In both instances there is abdominal hyperpressure, usually with vomiting. Usually, the clinical picture is reported to include alcoholism (although its acute or chronic nature is rarely stated). Numerous concurrent esophagogastric disorders have been described although their prevalence and their role have not be clearly defined : hiatal hernia, esophagitis, diffuse esophageal spasm, mid-thoracic or epiphrenic diverticulum [5, 6]. Defects of the vomiting mechanisms have also been reported, especially for "spontaneous" ruptures. The location of the lesion in the lower esophagus or cardia and the longitudinal appearance of the lesion whether it is panmural as in Boerhaave's syndrome or purely mucosal or even submucosal as in the Mallory-Weiss syndrome, are other arguments in favor of grouping these two syndromes . In addition, associated mucosal lesions of focalized necrosis type have been observed in cases of "spontaneous" rupture. Finally, the literature reports a male predominance for both these types of accidents.
In practice many differences have been observed between these two entities. Although excess abdominal pressure is initially involved in both disorders, their pathophysiology is not the same. In Boerhaave's syndrome the rise in pressure is intraesophageal and, in accordance with Laplace's law, it is the most distensible segment that tears, i.e. the left edge of the lower thoracic esophagus or, very rarely, the abdominal esophagus. This increase in intraesophageal pressure is related to an increase in abdominal pressure in a patient with an incompetent lower esophageal sphincter (LES) whose cricopharyngeus muscle is in spasm. It can be seen how defective synchronization of the physiological mechanisms of vomiting, and especially failure of the cricopharyngeus muscle to open, could be responsible, along with the possible involvement of alcoholism. During vomiting, when the vomitus is expelled from the stomach through contraction of the abdominal muscles and the diaphragm, there is synchronous relaxation of the lower esophageal sphincter and the cricopharyngeus muscle. Defective coordination at this level can account for rupture of the esophagus as a complication of the abdominal bruising. In the Mallory-Weiss syndrome the lesions are gastric or cardiac and very rarely solely esophageal [8, 9]. The increase in abdominal pressure is only transmitted to the stomach in patients with a competent LES. Some believe that the topography of the lesions should be gastric in the presence of a hiatal hernia and esophageal in its absence . According to the literature, the prevalence of hiatal hernia in the Mallory-Weiss syndrome ranges between 30 and 100%. Although the lesion is longitudinal and single in both syndromes (although multiple lesions have been described in patients with Mallory-Weiss syndrome), it is always panmural in the case of spontaneous rupture and always purely mucosal or, at most, submucosal in the Mallory-Weiss syndrome. Crushing of the cardiac region against the crura of the diaphragm has also been incriminated in the Mallory-Weiss syndrome. Experimentally, it has been shown that it is the muscular layer which tears first in the esophagus whereas it is the mucosa in the stomach. Because of its anatomic structure, the muscular layer of the abdominal or lower thoracic esophagus would seem to be a weak area.
Overall, despite these differences, from the pathophysiological point of view it makes sense to classify these two syndromes together in the same category of barogenic lesions of the esophagogastric junction even if the lesions associated with Boerhaave's syndrome and the Mallory-Weiss syndrome require completely different therapeutic management and carry a different prognosis.
3. Richelme P, Bourgeon A, Ferrari CH, Michels-Galy M. Quatre observations de rupture spontanée de l'sophage : déductions diagnostiques et thérapeutiques de l'analyse détaillée de 100 observations publiées dans la littérature. Chirurgie 1981;107:158-169.