Olfactory stimuli provoke diffuse esophageal spasm: reversal by ipratropium bromide

Diffuse esophageal spasm (DES) is a motor disorder of the esophageal smooth muscle characterized by multiple spontaneous contractions and by swallow-induced contractions that are of simultaneous onset, large amplitude, long duration, and repetitive occurrence. Although the pathogenesis of DES is unknown, provocative studies with cholinergic stimulation, esophageal balloon distention, or acid instillation have suggested involvement of both sensory and motor mechanisms. This report describes a patient with DES who would predictably become symptomatic with dysphagia and chest pain upon inhalation of perfume or other strong odors. Using esophageal scintigraphy to quantitate and analyze esophageal transit in this patient, we report for the first time that olfactory stimulation triggers episodes of DES and that such phenomena are mediated through the vagus nerve, because they can be ameliorated by the administration of ipratropium bromide. These observations suggest a new (sensory) pathway for the induction of DES and raise the intriguing possibility that inhaled anticholinergics may have a therapeutic role in the management of spastic esophageal motility disorders.     

Hypomagnesemia following correction of metabolic acidosis: a case of hungry bones

Severe symptomatic hypomagnesemia (0.15 mmol/L [0.3 mEq/L]) and hypocalcemia (1.47 mmol/L [5.9 mg/dL]) occurred in a 4-week-old infant coincidental with correction of a severe renal tubular acidosis with alkali therapy. The patient had no evidence of gastrointestinal abnormality and magnesium (Mg) intake was adequate for age and weight. Extreme renal conservation of Mg was observed, supporting the presence of Mg depletion. We suggest that Mg depletion in this infant occurred due to acidosis-induced bone demineralization and that symptomatic hypomagnesemia was precipitated by rapid remineralization accompanying correction of systemic acidosis. This patient represents a novel case of hungry bone syndrome (HBS). Since HBS has not been described previously in patients with acidosis undergoing therapy, several other factors may have contributed to this patient's severe hypomagnesemia, namely, prematurity, twin status, severity of acidosis, rapidity of correction of acidosis, catch-up growth and calcium supplementation. Clinicians should be vigilant for HBS in infants with severe acidosis undergoing alkali therapy.     

Patterns of esophageal motility in patients with stable bulimia

OBJECTIVES: A high prevalence of GI motility disturbances, including deranged esophageal motility, has previously been reported in patients with eating disorders; altered esophageal and gastric motility have been suggested to play an important role in these disease processes. We sought to confirm this association in an independent patient population. METHODS: We performed esophageal manometry in 12 patients with eating disorders (eight patients with bulimia and four with overlap syndromes, including both anorexia and bulimia features) and 12 sex-matched controls. All subjects completed a symptom questionnaire. Motility testing was performed in the standard fashion using a low compliance water perfusion catheter and computerized digital data capture. Coded tracings were interpreted in a blinded fashion. RESULTS: All 24 studies showed normal motility patterns [95% confidence interval for abnormal esophageal motility in eating disorders patients (0, 0.27)]. Mean lower esophageal sphincter pressure was 24.4 mm Hg in patients and 21.8 in controls; all relaxed normally. Mean esophageal body contraction amplitude 3 cm above the lower esophageal sphincter was 82.6 mm Hg in patients and 84.3 in controls; waveform morphology and progression met normal criteria in all studies. Eight of 12 patients reported dysphagia, odynophagia, or both, compared with 1 of 12 controls; patients demonstrated a pattern of increased overall GI symptomatology compared to controls. CONCLUSIONS: We conclude that disordered esophageal motility is uncommon among stable eating disorder outpatients with bulimic features, and that dysphagia and odynophagia are rarely associated with disordered motility in this group.     

Sneddon''s syndrome: apropos a case

The case of a 76 year old patient diagnosed of severe dysphagia by familiar oculopharyngeal muscular dystrophy is presented. Central venous catheterization (right internal jugular) was required for parenteral nutrition. A few minutes after catheterization, the patient developed respiratory arrest and coma and later cerebral vascular accident with left flaccid hemiplegia, with the ischemic lesion being focalized in the right parietooccipital region as well as in the left of the posterior fossa were observed. Aneurismatic dissection of the bilateral vertebral artery during lateralization of the head may be a cause of the lesions presented by the patient associated to arterial spasm or not. In central venous catheterization the possible complications derived from the position of the neck may carry must be taken into account.     

Periodic downbeat nystagmus

The authors observed a periodic downbeat nystagmus with a cycle of 3 minutes 30 seconds, beating downward for a period of 90 seconds every 2 minutes. It lasted 86 hours in a patient with severe hypomagnesemia associated with complications from scleroderma. There was no lesion on brain imaging, and an EEG performed during the nystagmus was normal, indicating that the most likely explanation for this periodic nystagmus is metabolic. This form of nystagmus may have resulted from severe hypomagnesemia, possibly associated with thiamine deficiency.     

Severe dysphagia, dysmotility, and unusual saccular dilation (diverticulum) of the esophagus following excision of an asymptomatic congenital cyst

Iatrogenic dysmotility syndromes, particularly achalasia-like conditions, occasionally complicate esophageal and paraesophageal surgery. We describe a patient who developed a very unusual (and as far as we know unreported) syndrome characterized by severe dysphagia, esophageal dysmotility (segmental simultaneous contractions of the distal esophagus), and very large saccular outpouching (diverticulum) involving the right wall of the distal half of the esophagus as a consequence of excision of an asymptomatic congenital cyst. The cyst had been discovered as an incidental finding on a preemployment chest x-ray. Our patient's dysphagia did not improve with nonsurgical treatments that are usually successful for idiopathic and iatrogenic achalasia.     

Diurnal variation of adrenocortical activity in chronic fatigue syndrome

The nutcracker esophagus, a primary motor disorder, is frequently associated with noncardiac chest pain. However, there are no data on whether its diagnosis, as in other esophageal motility disorders, is delayed. Since the disorder is frequently heralded by alarming symptoms such as chest pain and dysphagia, diagnosis should be made as soon as possible. In this study we assessed the diagnostic delay, if any, in patients with the nutcracker esophagus. Moreover, we were interested in whether the abnormalities described in the distal esophagus could also involve the entire viscus. Fifty-four subjects (age range 23-78 yr) with the nutcracker esophagus were assessed for clinical and manometric variables as an overall group and after dividing them into subgroups according to their symptoms. The manometric variables were compared with those obtained in 61 controls (age range 21-67 yr). Overall, a diagnosis of nutcracker esophagus was made after an average period of 36 +/- 6 months, and surprisingly, this was not different in the various subgroups complaining of either chest pain, dysphagia, or both. Analysis of manometric variables showed that the mean amplitude of contractions was significantly higher in the patients' group at all esophageal body levels, even in the proximal portions. Again, there were no significant differences among the subgroups of nutcracker esophagus with respect to the symptoms. Notwithstanding the presence of alarming symptoms, such as chest pain and dysphagia, the nutcracker esophagus is diagnosed on average after 3 years from the onset of symptoms. Manometric assessment seems to confirm that this entity may indeed represent a primary esophageal motor disorder. The major dysfunction is due to an abnormal increase of contraction amplitude of the entire esophageal body.     

Neurofibroma of the esophagus

Benign esophageal tumors occur infrequently, with leiomyomas accounting for approximately 70% of cases. Benign neural tumors of the esophagus account for 200 cases reported in the literature and rarely require operative resection. The case of a 58-year-old woman with a 4-month history of progressive dysphagia and odynophagia is presented. A large intramural esophageal mass was resected through a right thoracotomy, and the esophagus was primarily repaired. Histologic examination revealed a neurofibroma.     

Stricture related to an inlet patch of the esophagus

A 64-yr-old female was referred for evaluation of dysphagia. An esophagram showed a stricture in the proximal esophagus. On esophagogastroduodenoscopy, the patient was found to have a 3-cm stricture beginning at 14 cm from the incisors. This was initially dilated to 45-French with the Savary-Gilliard dilators. Biopsies and cytology of the stricture showed benign columnar epithelium. Subsequent dilations over the next 2 months resulted in dilation to 60-French with the esophageal dilators. Ulcerations related to this strictured area were healed over this period, with a sucralfate slurry, and the area of abnormality after healing appeared to be that of an esophageal inlet patch.     

Fresh osteochondral allografts: a 6-10-year review

OBJECTIVE: To analyze the use of self-expanding metal coated esophageal stents a palliative therapy of malignant dysphagia. METHOD: We performed a one year prospective study placing 12 stents in 10 patients with malignant dysphagia. RESULTS: The insertion mean time was lower than 25 min, and most of our patients (70%) reported a good tolerance. Dysphagia score improved immediately and it lasted until death with no occlusions. Complications appeared early, 17% at the time of placement, with one patient having a life-threatening complication. The probability for these patients not to develop any complications after the three first weeks will be about 60%. CONCLUSIONS: Self-expanding metal coated esophageal stents provide a fast and lasting relief in malignant dysphagia, with a good tolerance.     

Treatment of advanced gastroesophageal reflux disease with Collis gastroplasty and Belsey partial fundoplication

OBJECTIVE: To examine the factors affecting outcome in patients with advanced gastroesophageal reflux disease. DESIGN: Retrospective analysis. SETTING: University tertiary referral center. PATIENTS: Thirty-seven patients with advanced gastroesophageal reflux disease and no previous antireflux surgery. INTERVENTIONS: Thirty patients underwent Collis gastroplasty for esophageal lengthening and Belsey partial fundoplication. Seven patients with esophageal stricture and global loss of esophageal body motility who underwent primary esophagectomy and reconstruction were used as a comparison group. OUTCOME MEASURES: Symptomatic outcome in all 37 patients was assessed by questionnaire at a median of 25 months (range, 5-156 months) after surgery. In a subset of 11 patients undergoing the Collis-Belsey procedure, outcome was measured using 24-hour pH and results of motility studies. RESULTS: The Collis-Belsey procedure was successful in relieving symptoms of gastroesophageal reflux in 21 (70%) of the 30 patients. The outcome was excellent or good in 16 (89%) of 18 patients who presented with symptoms other than dysphagia, but only in 5 (42%) of 12 patients with dysphagia (P = .01). The outcome was particularly poor if dysphagia was associated with a previously dilated esophageal stricture. Persistent or induced dysphagia was the reason for failure in all but 1 patient. Results of 24-hour esophageal pH studies were returned to normal in 8 (73%) of 11 patients undergoing postoperative evaluation. Contraction amplitudes in the distal esophagus and the prevalence of simultaneous contractions in these segments did not change after the operation. All 7 patients who underwent primary esophagectomy were classified as having an excellent or good outcome and were relieved of their reflux symptoms, including dysphagia. Six of these could eat 3 meals per day and enjoyed an unrestricted diet. CONCLUSIONS: The outcome of the Collis-Belsey procedure in patients with advanced gastroesophageal reflux disease without dysphagia is excellent. It is less so in patients with dysphagia as a preoperative symptom. Esophagectomy can provide a good outcome in patients who have a combination of dysphagia stricture and a profound loss of esophageal motility.     

Laparoscopic Toupet fundoplication for gastroesophageal reflux disease with poor esophageal body motility

Impaired esophageal body motility is a complication of chronic gastroesophageal reflux disease (GERD). In patients with this disease, a 360-degree fundoplication may result in severe postoperative dysphagia. Forty-six patients with GERD who had a weak lower esophageal sphincter pressure and a positive acid reflux score associated with impaired esophageal body peristalsis in the distal esophagus (amplitude <30 mm Hg and >10% simultaneous or interrupted waves) were selected to undergo laparoscopic Toupet fundoplication. They were compared with 16 similar patients with poor esophageal body function who underwent Nissen fundoplication. The patients who underwent Toupet fundoplication had less dysphagia than those who had the Nissen procedure (9% vs.44%; P=0.0041). Twenty-four-hour ambulatory pH monitoring and esophageal manometry were repeated in 31 Toupet patients 6 months after surgery. Percentage of time of esophageal exposure to pH <4.0, DeMeester reflux score, lower esophageal pressure, intra-abdominal length, vector volume, and distal esophageal amplitude all improved significantly after surgery. Ninety-one percent of patients were free of reflux symptoms. The laparoscopic Toupet fundoplication provides an effective antireflux barrier according to manometric, pH, and symptom criteria. It avoids potential postoperative dysphagia in patients with weak esophageal peristalsis and results in improved esophageal body function 6 months after surgery.     

Gastric cancer occurring in a patient with Plummer-Vinson syndrome: report of a case

We report herein the unusual case of a 59-year-old woman with Plummer-Vinson syndrome who developed gastric cancer. The patient had a longstanding history of dysphagia and iron deficiency anemia, for which she had sporadically taken iron supplements that improved the dysphagia to some extent, but not completely. Owing to her tolerance of the dysphagia, she had not been taking iron supplements for the past 17 years. On admission, she was in fair nutritional condition and not anemic. Blood chemistry results were all normal, including the serum iron level. Gastrointestinal radiographic series demonstrated cervical esophageal webs and advanced gastric cancer. Her dysphagia was successfully treated by endoscopic bougienage through the webs, and a distal partial gastrectomy with nodal dissection was performed. Histology of the resected stomach revealed atrophic mucosal change and, by chance, an adenomatous lesion in addition to adenocarcinoma. Her postoperative course was uneventful and she is now well, without any signs of recurrence. Although Plummer-Vinson syndrome is known to be associated with upper alimentary tract cancers, gastric cancer is extremely rare. A discussion on the etiology of Plummer-Vinson syndrome and its link with potential carcinogenesis follows this case report.     

Median nerve F-wave study derived by flexor carpi radialis

Oropharyngeal dysphagia in adults is secondary to either a structural lesion or neuromuscular disorder of the upper esophageal sphincter. In cricopharyngeal achalasia (incomplete relaxation of the upper esophageal sphincter), the etiology is usually either related to neck surgery or other neuromuscular disorders. We report on a rare case of neuromuscular oropharyngeal dysphagia secondary to bone metastases to the base of the skull. The patient is an 81-year old man with prostate cancer with metastases to the sacrum. A gastroscopy was attempted to discern the etiology of his dysphagia, but the endoscope could not be advanced. A barium swollow showed cricopharyngeal achalasia, and an magnetic resonance image of the brain demonstrated bone destruction to the floor of the left posterior fossa in the region of the jugular foramen and foramen magnum. The bone destruction caused disruption of the glosso-pharyngeal and vagus nerves. Selective radiotherapy resulted in rapid improvement in his symptoms. The primary treatment of cricopharyngeal achalasia is to correct the underlying process, if possible. This case illustrates an unusual presentation of secondary cricopharyngeal achalasia caused by cranial nerve involvement secondary to bone metastases.     

Esophageal manometry: a comparison of findings in younger and older patients

OBJECTIVE: We sought to determine the utility of esophageal manometry in an older patient population. METHODS: Consecutively performed manometry studies (470) were reviewed and two groups were chosen for the study, those > or = 75 yr of age (66 patients) and those < or = 50 years (122 patients). Symptoms, manometric findings (lower esophageal sphincter [LES], esophageal body, upper esophageal sphincter [UES]) and diagnoses were compared between the groups. RESULTS: Dysphagia was more common (60.6% vs 25.4%), and chest pain was less common (17.9 vs 26.2%) in older patients. In the entire group, there were no differences in LES parameters. Older patients with achalasia had lower LES residual pressures after deglutition (2.7 vs 12.0 mm Hg), but had similar resting pressures (31.4 vs 35.2 mm Hg) compared with younger achalasia patients. Duration and amplitude of peristalsis were similar in both groups, whereas peristaltic sequences were more likely to be simultaneous in the older group (15% vs 4%). The UES had a lower resting pressure in the older patients (49.6 vs 77.6 mm Hg) and a higher residual pressure (2.0 vs -2.7 mm Hg). The older patients were less likely to have normal motility (30.3% vs 44.3%) and were more likely to have achalasia (15.2% vs 4.1%) or diffuse esophageal spasm (16.6% vs 5.0%). When only patients with dysphagia were analyzed, achalasia was still more likely in the older group (20.0% vs 12.9%). CONCLUSION: When older patients present with dysphagia, esophageal manometry frequently yields a diagnosis to help explain their symptoms.     

Falsely high ionized magnesium results by an ion-selective electrode method in severe hypomagnesemia

Changes in serum total and ionized magnesium (Mg and Mg2+) and calcium (Ca and Ca2+) were monitored in three patients who transiently developed severe (total Mg < 0.50 mmol/l) to profound hypomagnesemia (total Mg < 0.35 mmol/l) due to cisplatin or interleukin-2 therapies. Mg2+ and Ca2+ were measured with the Nova ion-selective electrodes at 37 degrees C and all results were normalized to pH 7.40. Independent of the etiology, the Mg2+ fraction (Mg2+/total Mg) increased as the concentration of the serum total Mg decreased in all three patients. When the total Mg was around or below 0.35 mmol/l the Mg2+ approached or exceeded total Mg, suggesting an error in the measurement of Mg2+. The findings were extended by including a group of 31 additional patients whose serum total Mg, Mg2+, total Ca, and Ca2+ concentrations varied from abnormally low to above normal. The serum total and ionized concentrations strongly correlated for both Mg (r2 = 0.88) and Ca (r2 = 0.92). The Mg2+ fraction rapidly increased with a fall in the total Mg concentration (r2 = 0.76) and total Mg/total Ca ratio (r2 = 0.71). In fact, with decreasing total Mg concentrations or total Mg/total Ca ratios, the Mg2+ fraction progressively increased to 93-128% of the total, confirming an error in the Mg2+ determinations. The Ca2+ fraction showed a slight and insignificant decrease with falling total Ca concentrations and total Mg/total Ca ratios. The Mg2+ concentration was directly related (r2 = 0.62), whereas the Ca2+ concentration showed a complex relationship to the total Mg/total Ca ratio. Whether this latter relationship represents a technical artifact or a true biological phenomenon requires further study. The apparent overestimation of Mg2+ at very low total Mg concentrations, and in the presence of a very low total Mg/total Ca ratio, could be due to improper chemometric correction of the Ca effect on the Mg electrode, non-linearity, and inadequate calibration. Whatever the mechanism, the failure of this method to correctly measure very low serum Mg2+ concentrations in the sera of patients with severe hypomagnesemia, or likely in any patient with an unusually low total Mg/total Ca ratio, erodes its diagnostic usefulness.     

Dietary magnesium deficiency increases Gi alpha levels in the rat heart after myocardial infarction

OBJECTIVE: Magnesium (Mg) is crucial for the function of G proteins which play important roles in mediating the inotropic effects of beta adrenergic agonists in the heart and are altered in heart failure. This study was performed to determine whether or not dietary Mg deficiency alters functional activity and levels of the two major ventricular G proteins, Gi alpha and Gs alpha in the heart after myocardial infarction (MI). METHODS: Six week old rats were fed an Mg adequate or deficient diet for 6 weeks. At the end of week 3, MI was induced by coronary artery ligation. A sham operation was performed as control. After surgery, surviving animals were maintained on their assigned diets for another 3 weeks. Then, cardiac function was measured, plasma and tissue were collected. RESULTS: Severe hypomagnesemia and increased plasma catecholamine level were observed in all animals fed the Mg deficient diet. A significant reduction of myocardial Mg concentration accompanied by elevated plasma and myocardial calcium concentrations was observed in MI animals with existing Mg deficiency vs. animals fed the Mg adequate diet. Cardiac function was impaired in MI rats and further reduced in MI rats with existing Mg deficiency. Gi alpha level was not altered by either Mg deficiency or MI alone, but was dramatically elevated in animals with combined Mg deficiency and MI (9.9 +/- 0.7 arbitrary unit.mg-1 protein) as compared to MI alone (5.8 +/- 0.6, P < 0.05) and Mg deficiency alone (6.1 +/- 0.8, P < 0.05). Gs alpha level did not differ between groups. GppNHp-, but not fluoride-stimulated adenylyl cyclase activity was slightly reduced in MI animals with existing Mg deficiency. CONCLUSION: The findings suggest that dietary Mg deficiency increases the expression of Gi alpha in the heart after MI, while levels and function of Gs alpha are not compromised during dietary Mg deficiency either with or without MI.     

Use of the polymerase chain reaction for detecting Trypanosoma cruzi in triatomine vectors

Two patients with squamous cell carcinoma arising within an epiphrenic diverticulum are reported bringing the total reported in the English-language literature to fifteen. The majority of patients with carcinoma arising in an epiphrenic diverticulum are elderly males who have been symptomatic for years with a known diverticulum. This is the first report of multiple cases of carcinoma within an epiphrenic diverticulum from a single institution and the first report in a female patient. Similar to other reports, these two patients presented with several months of symptoms related to their esophageal diverticulum. It is important to entertain the diagnosis of esophageal carcinoma early in the evaluation of patients with signs and symptoms suggestive of epiphrenic diverticula as early intervention may be the only chance of cure.     

Gingival lesions in a patient with dermochondrocorneal dystrophy (Fran?ois syndrome). A case report

This report is concerned with gingival manifestations associated with a case of dermochondrocorneal dystrophy (DCCD) or Fran?ois syndrome occurring in a 42-year-old woman. Our Department treated this patient for 15 years. Oral examination of this case revealed a diffuse enlargement and severe inflammation of the attached gingiva. Systemic findings were similar to those reported in the literature for patients with DCCD. Firm papules 3 mm wide, localized on the face and on the dorsal surface of the hands, were associated with corneal involvement and progressive and severe articular disorder. Because they recurred after surgical ablation, the gingival lesions became an important problem in the management of the patient. After 10 years of unsuccessful treatment limited to scaling, oral hygiene control and mouth rinses with 0.2% chlorexidine solution, the patient was submitted to extraction of the remaining teeth, remodelling osteoplasty and cutaneous graft. An acrylic full denture was inserted. In a follow-up of 7 years, good results for the oral health of the patient were seen.     

Lack of effect of hypomagnesemia on elevated plasma 1,25-dihydroxycholecalciferol in acutely phosphate-deprived rats

The effect of hypomagnesemia occurring in phosphate deprivation on elevated levels of plasma 1,25-dihydroxycholecalciferol (1,25(OH)2D3) was evaluated in rats. Phosphate deprivation was associated with an elevated plasma 1,25(OH)2D3, hypophosphatemia, and hypomagnesemia. With oral magnesium supplement during phosphate deprivation, plasma magnesium did not fall, but plasma inorganic phosphate fell and 1,25(OH)2D3 rose to comparable levels seen in phosphate-deprived rats lacking magnesium supplement. These results indicate that a rise in plasma 1,25(OH)2D3 during phosphate deprivation is not due to concomitant hypomagnesemia and support the postulate that phosphate deprivation per se is responsible for the elevated plasma 1,25(OH)2D3.