0000000000240114
AUTHOR
V. F. Eckardt
Etiopathological aspects of achalasia: lessons learned with Hirschsprung's disease
SUMMARY The etiology of primary esophageal achalasia is largely unknown. There is increasing evidence that genetic alterations might play an important but underestimated role. Current knowledge of the genetic base of Hirschsprung's disease in contrast is far more detailed. The two enteric neuropathies have several clinical features in common. This association may also exist on a cellular and molecular level. The aim of this review is to enlighten those etiopathogenetic concepts of Hirschsprung's disease that seem to be useful in uncovering the pathological processes causing achalasia. Three aspects are looked at: (i) the genetic base of Hirschsprung's disease, particularly its major suscept…
Autonomic dysfunction in patients with achalasia.
It has been previously shown that patients with achalasia may have motor abnormalities of the stomach, small bowel and biliary system. This study investigates whether a disturbance of extraintestinal autonomic function occurs. Autonomic function studies were performed in 15 patients with achalasia and 15 age- and sex-matched healthy controls. Pupillo-grams were obtained during darkness, light exposure and after pilocarpine administration. Cardiovascular function studies included determinations of heart rate variation during deep breathing and orthostasis. In addition, we determined blood pressure changes in response to sustained handgrip, cold exposure and orthostasis. Neurohormonal functio…
Zweizeitige Ösophagusresektion mit ischämischer Präkonditionierung des Schlauchmagens bei dekompensiertem Dolichomegaösophagus im Endstadium der Achalasie
Development of a megaesophagus with a sigmoid-shaped distal part in patients with achalasia--even in the course of successful myotomy with reduction of the resting pressure of the lower esophageal sphincter--is often the expression of an irreversible progression of the disease. Management of patients with end-stage achalasia and aperistaltic, dilated "burned-out" esophagus--with or without peptic stenosis--is a therapeutic challenge for gastroenterologists and surgeons. We report on a 37-year-old female patient with decompensated dolichomegaesophagus following multiple endoscopic and operative interventions at the lower and upper esophageal sphincters presenting with severe dysphagia and we…
Achalasie im Kindesalter: Eine separate Entität?
Background Achalasia in childhood is rare, also the etiology and the pathogenesis of the early onset ort he disease is practically unknown. Little is known about the neuropathological changes in structure of the esophageal wall in non-hereditary, sporadic achalasia in children and ist differentiation to that in adults. The aim of our study was to examine the morphological properties or high-pressure zone of the lower esophageal sphincter in children who had undergone a Heller myotomy because of achalasia as well as to compare them with the pathological findings in adults. Methods Muscle biopsies of the smooth musculature, a 20 x 10 mm long segment of the myenteric of the distal esophagus (l…
Esophageal transmural potential difference in patients with symptomatic gastroesophageal reflux.
Esophageal electrical potential difference (PD) was studied in 9 patients with symptomatic gastroesophageal reflux and in 9 healthy control subjects. None of the patients revealed gross mucosal damage by radiography or endoscopy, but all of them showed positive acid perfusion studies. In the stomach and across the lower esophageal sphincter PD profiles were remarkably similar in patients and controls. Throughout the lower esophagus however, PD values were slightly higher in patients with symptomatic reflux than in healthy volunteers. These data are in contrast to a previous investigation, in which patients with reflux-induced gross mucosal damage revealed a decreased PD in the lower esophag…
Ösophagusresektion bei unspezifischer Motilitätsstörung der Speiseröhre - Bedeutung der neuropathologischen Befunde
A 47-year-old patient presented with a history of dysphagia for solid food for almost 10 years and weight loss of more than 50 kg. Non-resecting surgical as well as endoscopic procedures (laparoscopic cardiomyotomy with secondary antireflux operations, balloon dilation, Botulinum-toxin injection) were without success. A barium esophagogram showed a confinement of the distal esophagus with a filiform passage of the contrast medium and undigested food in the prestenotic dilated esophageal corpus. Manometry displayed a hypertensive lower esophageal sphincter with a resting pressure of 43.8 mmHg - although completely relaxing. The tubular esophagus was aperistaltic with 100 % simultaneous and r…
Achalasie bei zirkumskripter Sklerodermie
HISTORY: A 38-year-old man had been suffering from circumscribed scleroderma for 12 years. Dysphagia had been diagnosed 5 years ago and for the last 2 years he had retrosternal dysphagia for solid and liquid food. His symptoms had increased markedly 6 months before presenting at our hospital and the patient had lost 15 kg of weight. 2 months ago, a percutaneous endoscopic gastrostomy (PEG) had been inserted at another hospital. INVESTIGATIONS: The patient presented in a reduced general and nutritional state. The routine laboratory tests and tumor markers were within normal range. Endoscopy showed a moderately dilated esophagus with food remnants. It was not possible to pass the cardia witho…