William C. Orr

Desmoplastic malignant melanoma (A rare variant of spindle cell melanoma)

This is a detailed report of seven cases presenting a peculiar sequence of events which, starting from inconspicuous superficial melanotic lesions, generally located in the head and neck regions leads to the production of bulky subcutaneous tumefactions. These have the clinical and histologic appearance of locally invasive fibrous tumors. However, the elongated neoplastic cells are cytologically different from neoplastic fibroblasts, and these tumors behave as highly malignant stubbornly recurring and often metastasizing neoplasms. Some of the recurrences, as well as some of the metastases, are histologically acceptable as malignant melanoma of the more usual type. This appears to be a hitherto undescribed clinicopathologic entity, for which we suggest the term “desmoplastic malignant melanoma.”.

The effect of a modified functional appliance on obstructive sleep apnea

This study combined the use of cephalometrics and overnight polysomnographic monitoring to analyze the effects of a modified functional appliance on airway, sleep, and respiratory variables in patients with obstructive sleep apnea (OSA). Twelve patients without overt anatomic or pathologic evidence of obstruction were selected on the basis of an initial single night of polysomnographic monitoring, which confirmed the diagnosis of obstructive sleep apnea syndrome. The patients subsequently were fitted with a modified functional appliance designed to securely hold the mandible in an anterior-inferior position. A subsequent overnight polysomnographic study was obtained with each patient wearing the appliance. Lateral cephalometric radiographs with and without the appliance in place were also obtained. The mean vertical and horizontal changes in mandibular position while wearing the appliance were 8.49 mm and 2.28 mm, respectively. The findings indicate that 10 of the 12 patients had decreases in the rate of complete airway obstructions from a mean of 28.86 to 18.69 events per hour, and in the total apnea index from a mean of 53.81 to 35.99 events per hour. A reduction in the rate of obstructive events is attributed to the effect of the appliance on the oropharyngeal structures. Six cephalometric measurements are presented to provide a means of assessing effects of the appliance on the oropharynx and associated structures. The modified functional appliance is a conservative, successful treatment alternative that could benefit patients with obstructive sleep apnea syndrome.

Water Swallows Versus Food Ingestion as Manometric Tests for Esophageal Dysfunction

Data from 100 consecutive patients with chest pain or dysphagia, or both, who underwent esophageal testing with standard water swallows and upright food ingestion were retrospectively evaluated. In addition to having manometric patterns monitored, patients were asked to relate symptoms during testing. Of 77 patients with a history of dysphagia, significantly more had abnormal manometry during the test meal than with water swallows (79 vs. 43%, p less than 0.005). Additionally, dysphagia, although reported in only 8% of these patients during standard testing, occurred in 47% during the test meal (p less than 0.001). Of 60 patients with chest pain, symptoms were rarely reported (5%) with water or with food ingestion. We conclude that manometry with food ingestion should be used as a provocative test in anatomically normal patients with dysphagia.

Comparison of calcium channel blocking agents and an anticholinergic agent on oesophageal function.

The effects of oral doses of three calcium blockers and an anticholinergic drug on oesophageal function were compared. Nifedipine (20 mg) and hyoscyamine (0.25 mg) significantly reduced lower oesophageal sphincter (LES) pressure and oesophageal contractile pressure. Verapamil (120 mg) and diltiazem (60 mg) had no significant effect on any of the oesophageal variables measured. Oesophageal transit time and oesophageal contractile duration were not affected significantly by any of the agents. Only hyoscyamine significantly prolonged acid clearance time. The combination of nifedipine and hyoscyamine was no more effective in decreasing LES pressure or oesophageal contractile pressure than either agent alone. Either nifedipine or hyoscyamine would appear to be potentially effective for the treatment of oesophago‐spastic and other hypertensive motor disorders, but hyoscyamine may lead to prolongation of acid clearance from the oesophagus.

Anorectal functioning in fecal incontinence

Manometric testing was performed on three groups of subjects: 14 patients complaining of fecal incontinence, 14 age- and sex-matched continent patients, and 14 sex-matched younger normal controls. The younger group displayed significantly stronger contractions of the external anal sphincter and puborectalis than the two patient groups, which did not differ. No differences were found in the relaxation of the internal anal sphincter. The incontinent group required a significantly larger stimulus in order to detect rectal distension compared to either the continent patients or the younger normals. An additional group of unmatched normals and incontinent patients demonstrated significant differences in their ability to retain rectally infused saline. The patients leaked sooner and retained less;however, the performance of the normals was considerably reduced from that reported in previous studies. The aging process seems to result in weakening of the striated muscles of the anal canal, although fecal incontinence need not occur. The afferent limb of the anorectal sensorimotor mechanism does not necessarily deteriorate with aging. A lower threshold for sensation of rectal distension among continent individuals apparently helps them to avoid incontinent episodes, even though maximum contractile pressures in their anal canal are no different from a comparable group of incontinent individuals.

Utilization of Polysomnography in the Assessment of Sleep Disorders

Polysomnography is an indispensable tool in dealing with sleep disorders, but because of its inherent logistical complexity and expense, it must be used with discretion. Many sleep disorders can be diagnosed clinically by the astute physician and treated empirically with good results. On the other hand, there are numerous circumstances in which the best clinical judgment indicates a polysomnographic evaluation. The ubiquity of sleep complaints in medical practice, and the knowledge that they may in some instances be associated with life-threatening medical complications, compel the modern clinician to obtain a basic knowledge of the appropriate utilization of the sleep laboratory. Polysomnography is a useful adjunct to the clinician because it provides objective information to be integrated into the overall clinical picture in the evaluation of the patients complaint. Polysomnography should be utilized in cases where the results will clearly make a difference in the differential diagnosis or in the treatment plan. If a clinicians basic approach to the evaluation of the patient or his treatment will not be substantially altered by polysomnography, it should not be undertaken. The results of polysomnography should give the referring physician critical information concerning the type of disorder, its severity, appropriate treatment and necessary follow-up. Unfortunately, few, if any, hard and fast rules exist concerning the appropriate utilization of polysomnography. As always in medicine, the physician must take a careful history, and integrate it with other basic laboratory tests and the physical examination, and then decide whether polysomnography will provide useful additional information. The ability to assess physiological phenomena during sleep objectively has clearly advanced the study of sleep disorders to the point that it is now considered an integral part of internal medicine. Polysomnography has made available the objective study and documentation of sleep disorders which, coupled with increasing sophistication concerning the natural history of sleep disorders, have created a new and exciting discipline in clinical medicine.

Nonhypersomnolent Patients with Obstructive Sleep Apnea

Until recently, snoring had been considered both a medical enigma and a psychosocial problem. Snoring is now considered to be an acoustic phenomenon produced by vibration of the soft palate and the tonsillar pillars. We describe 20 patients with a clinical complaint of excessive snoring who were referred to rule out obstructive sleep apnea. All patients were without symptoms of daytime sleepiness and failure of the right heart. Twenty subjects were studied, 18 of whom were males. All subjects were monitored for one full night in the Sleep Laboratory. The apnea rate ranged from 9.0 to 94.0 incidents an hour with a mean of 30.0. Eight of the 20 subjects had obstructive episodes longer than 1 minute and three others had episodes longer than 55 seconds. Hypersomnolence, long thought to be a cardinal symptom, is not present in all patients with an ostensibly significant degree of obstructive sleep apnea.

Lower Esophageal Sphincter Pressure, Esophageal Body Motor Functioning, and Esophageal Acid Sensitivity

Esophageal acid sensitivity is believed to develop as a result of esophageal acid exposure, contributing factors being gastroesophageal reflux and delayed esophageal acid clearance. The relationship among lower esophageal sphincter pressure, motor functioning of the body of the esophagus, and esophageal acid sensitivity was examined by comparing the results from 912 patients and normal subjects studied with both esophageal manometric and Bernstein acid infusion tests. Positive acid infusions were statistically more closely associated with hypotensive lower esophageal sphincter pressures than with any motor abnormality in the body of the esophagus. Of the several esophageal body motor abnormalities considered, only feeble peristalsis had significantly more positive Bernstein tests than did normal esophageal body motor functioning. The findings from this study demonstrate that hypotensive lower esophageal sphincter pressure is more closely associated with an acid-sensitive esophagus than is impaired esophageal body motor functioning.

Studies of Esophageal Function During Waking and Sleep

Although the gut has long been recognized as the site of powerful but obscure influences from the central nervous system (CNS), interest has focused almost exclusively on CNS effects on the stomach and colon. There is a well-known and voluminous literature which has developed on the subject of the psychological influences on peptic disease, inflammatory bowel disease, and the irritible bowel syndrome, but until recently, the esophagus has been relatively ignored as an organ worthy of serious physiologic investigation, let alone scrutiny by the psychophysiologist.