Isolated non-compaction of left ventricular myocardium

Isolated non-compaction of the left ventricle is a distinct form of cardiomyopathy. It may be idiopathic or secondary to an identifiable disease. The condition usually presents with symptoms of cardiac failure. The present report is of a child with this disease who was diagnosed at birth and was relatively stable at 5 years of age.     

Anaesthetic considerations in a parturient with critical coronary artery disease and a drug-eluting stent presenting for caesarean section

A parturient presented with her first symptoms of coronary artery disease at 18 weeks' gestation. Following an angiogram, a drug-eluting stent was inserted, resulting in resolution of her symptoms. The patient was prescribed anti-platelet medication including clopidogrel. She was delivered by elective caesarean section at 35 weeks under general anaesthesia. The anaesthetic management is discussed and a review of the literature presented.     

Reconstruction of vertebral artery stenosis.

Eight patients with transient attacks of cerebrovascular insufficiency were treated by reconstruction of an atheromatious stenosis at the origin of a vertebral artery. The technique was relatively simple and safe, and was effective in relieving symptoms. Previous reviews suggest that the symptoms of vertebrobasilar insufficiency tend to be persistent, frequent and distressing, and that they can be a prelude to brain stem infarction. The collateral circulation to the vertebrobasilar system may be restricted by anatomical variations and by disease, particularly affecting the circle of Willis. Bilateral vertebral artery disease can cause appreciable ischaemia even without associated carotid artery disease. Carotid endarterectomy is not particularly effective in relieving vertebrobasilar symptoms. Vertebral artery reconstruction appears to be the appropriate treatment for vertebrobasilar insufficiency.     

Fibromuscular dysplasia of the carotid arteries

Fibromuscular disease of the carotid artery was identified in 30 patients, which represented 3.2 percent of all patients who had cerebral angiography at Brooke Army Medical Center in the 6 year period from 1978 to 1984. Focal neurologic events were the presenting symptoms in 63 percent of the patients. The majority of the patients were treated with antiplatelet therapy, and eight patients had a total of 10 carotid artery dilatations. The only patients with recurrent symptoms were those who received either no treatment or antiplatelet therapy. There were no recurrent symptoms in the operated patients. This study suggests that surgical treatment for the symptomatic patient may prevent recurrent symptoms with an acceptably low morbidity and mortality. There was, however, no indication that prophylactic dilation of the fibromuscular disease in the asymptomatic patient was beneficial. Fibromuscular dysplasia of the carotid arteries is often associated with intracranial aneurysms, and surgical therapy rather than antiplatelet therapy may be advisable in patients who have intracranial aneurysms. Patients with concomitant atherosclerosis of the carotid artery bifurcation should be treated like any patient with atherosclerotic disease and an endarterectomy should be performed with carotid dilatation when indicated. Fibromuscular disease of the carotid artery is an infrequent angiographic finding that is associated with focal and global neurologic symptoms. Most patients can be effectively treated with antiplatelet drugs with no recurrent symptoms, however, for persistent or progressive symptoms, some patients will require surgical dilatation of the carotid artery. Fibromuscular disease of the carotid artery may lead to catastrophic symptoms of stroke or intracranial hemorrhage if left undiagnosed or untreated.     

Gallbladder ejection fraction: an accurate evaluation of symptomatic acalculous gallbladder disease

The use of cholecystokinin stimulation during cholescintigraphy to calculate the gallbladder ejection fraction has been associated with variable clinical results as a preoperative indicator for chronic acalculous cholecystitis and postoperative relief of biliary symptoms. A series of 56 consecutive patients was analyzed to determine the accuracy of a decreased gallbladder ejection fraction as a preoperative indicator for acalculous cholecystitis. Each patient had symptoms compatible with biliary disease. Each patient had a decreased gallbladder ejection fraction calculated by cholescintigraphy. The gallbladder ejection fraction was calculated using a 30-minute intravenous infusion of cholecystokinin at a dose of 0.02 microg/kg during cholescintigraphy. There was a 100% correlation found in this series of patients between a decreased gallbladder ejection fraction during cholescintigraphy, preoperative symptoms of gallbladder disease, and postoperative pathology evidence of acute or chronic cholecystitis. Only 1 patient had less than a complete resolution of her preoperative symptomatology after laparoscopic removal of her gallbladder. This patient had irritable bowel disease, which was diagnosed postoperatively. Six symptomatic patients with a gallbladder ejection fraction between 35% and 60% were also treated by laparoscopic removal of the gallbladder with complete resolution of their preoperative symptomatology. The use of a 30-minute infusion of cholecystokinin at a dose of 0.02 microg/kg to calculate the gallbladder ejection fraction during cholescintigraphy is an accurate test to preoperatively predict acalculous cholecystitis and postoperative relief of biliary symptoms. The gallbladder ejection fraction of less than 35% was abnormal. Cholecystectomy may be considered for patients whose gallbladder ejection fractions were calculated to be between 35% and 60% if the patient's symptoms were classical for biliary disease and have been present for 1 year. The use of a 30-minute intravenous infusion of cholecystokinin at a dose of 0.02 microg/kg to calculate the gallbladder ejection fraction during cholescintigraphy is an accurate test to preoperatively predict acalculous cholecystitis and postoperative relief of biliary symptoms.     

RECONSTRUCTION OF VERTEBRAL ARTERY STENOSIS1

Eight patients with transient attacks of cerebrovascular insufficiency were treated by reconstruction of an atheromatous stenosis at the origin of a vertebral artery. The technique was relatively simple and safe, and was effective in relieving symptoms. Previous reviews suggest that the symptoms of vertebrobasilar insufficiency tend to be persistent, frequent and distressing, and that they can be a prelude to brain stem infarction. The collateral circulation to the vertebrobasilar system may be restricted by anatomical variations and by disease, particularly affecting the circle of Willis. Bilateral vertebral artery disease can cause appreciable ischæmia even without associated carotid artery disease. Carotid endarterectomy is not particularly effective in relieving vertebrobasilar symptoms. Vertebral artery reconstruction appears to be the appropriate treatment for vertebrobasilar insufficiency.     

Factors associated with risk of breast cancer and persistence of symptoms in women with breast problems

Risk factors in breast cancer were analysed in a series of 293 women who were first seen at the Surgical Outpatient Department of Savonlinna Central Hospital in 1982-1983 for breast problems and were alive and willing to participate in a control examination and interview 4-5 years later. In 1986 and 1987 the patients were examined clinically and underwent mammography and interview. They were divided into three groups: 1) 49 patients with breast cancer in 1982 and 1983, 2) 49 with breast symptoms after primary treatment of benign disease, and 3) 195 patients without breast symptoms after primary treatment of benign disease. The groups were compared in terms of 18 suggested risk factors for breast cancer. Two factors were significantly associated with breast cancer (compared with all benign breast diseases): higher age of the patient and higher age at first delivery. Use of contraceptives was significantly associated with the presence of breast symptoms after treatment of benign breast disease (as compared with the absence of symptoms) (RR = 1.70 P less than 0.001). Hysterectomy and/or oophorectomy was significantly associated with the absence of breast symptoms among patients with benign breast disease (RR = 0.76, P less than 0.05). Our results suggest that symptoms after treatment of benign breast disease are associated with the levels of female sex hormones.     

Neurogenic claudication secondary to vascular disease.

Neurogenic claudication is characterized by sensory symptoms which appear during exercise or while maintaining a fixed posture. They are paraesthetic in quality, may be associated with 'march' phenomena, and patients may have bowel and bladder disturbance. The problem is most commonly secondary to lumbar canal stenosis (LCS) but rarely due to aortic disease, as shown in this case report. This report concerns a 66 year old woman who presented with symptoms of paraesthesia radiating from the buttocks to the thigh and, intermittent loss of bladder and bowel function, all associated with walking. There were no associated symptoms of vascular claudication. Clinically, there was evidence of aorto-iliac obstruction with absence of femoral pulses and a vascular index of 0.4 at both ankles. Neurological examination was normal at rest but the left ankle jerk was absent immediately after exercise. Myelogram and computerized tomographic (CT) scan were normal. An aortogram revealed a very tight irregular stenosis of the aorta at the level of the renal and mesenteric arteries. Very few lumbar vessels were seen. An aortic endarterectomy via a thoraco-abdominal approach was performed and an aortobifemoral graft inserted. The patient's symptoms resolved following this procedure. We postulate that her symptoms were caused by a 'steal' from the blood supply to the cauda equinda due to the severe athromatous disease of her upper abdominal aorta.     

Self‐reported depressive symptoms and suicidal feelings in perianal Crohn’s disease

Aim  Crohn’s disease is a chronic inflammatory condition that has been associated with high rates of mental illness. Perianal lesions are prevalent; however, their specific impact on depression has not been studied. The aim of our study was to investigate the prevalence and associations of self‐reported depressive symptoms in the subset of Crohn’s patients with perianal disease. Method  Patients with perianal Crohn’s disease from one institution were surveyed to elicit the frequency of self‐reported depressive symptoms. Patients completed a questionnaire and consented to medical records audit. Results  Of the 130 patients invited, 69 (53%) returned a survey. Depressive symptoms were self‐reported at very high rates, with 73% reporting feeling depressed and 13% reporting feeling suicidal at some point. Associations were found between depressive symptoms and duration of disease, prior surgery, past or present stoma, and anal stenosis. Patients who self‐reported depressive symptoms had lower overall utilities, and were willing to trade very significant proportions (upwards of 15%) of their life expectancy for disease cure. Conclusion  This study suggests that many patients with perianal Crohn’s disease experience significant emotional distress that impairs their overall quality of life. Further controlled studies are required to assess the impact of perianal disease and to address the need to target interventions to meet the mental health needs of this population.     

Intrafamilial phenotypic expression of autosomal dominant polycystic kidney disease.

It has been suggested that the clinical expression of autosomal dominant polycystic kidney disease (ADPKD) is uniform among individuals of a given family. To test this hypothesis, intrafamilial variations in ages at onset of first symptoms, types of first symptoms, serum creatinine concentrations, and renal sizes were evaluated in 131 patients with ADPKD from 36 unrelated families. These parameters were compared in younger and older affected relatives in the same family at a single time, due to difficulties of following them longitudinally. Because the natural course of the disease is to progress with age, it was presumed that disease progression in a given family was nonuniform if older individuals had lower serum creatinine concentrations, and/or smaller kidneys than their affected younger relatives, or if relatives of similar ages had different serum creatinine concentrations and/or kidney sizes. Nonuniform progression was suggested in 38% of affected relatives by serum creatinine concentrations and in 53% by kidney sizes. Ages at onset of first symptoms and types of first symptoms were also different in patients from the same families. These data indicate that phenotypic expression of ADPKD may differ considerably among patients who belong to the same families.     

Avascular necrosis of the femoral head in sickle-cell disease. A series from the Guinea Savannah of Nigeria

Of 899 patients with sickle-cell disease, aged between 6 and 28 years, who attended clinics in the Guinea Savannah of Nigeria in 1982 and 1983, 29 had symptoms of avascular necrosis of the femoral head. This group was studied in detail. Twenty-eight patients had haemoglobin-SS electrophoretic patterns and one had haemoglobin-SC. The male to female ratio was 1 to 1.6, and most of the patients were aged between 6 and 15 years at the onset of hip symptoms. These symptoms correlated with the radiographic lesions, but were not related to the age or sex of the patient. The radiographic lesions varied widely and were related to the age at onset of hip symptoms. A new radiological classification is proposed.     

Buerger''s disease and cigarette smoking in Bangladesh.

Buerger's disease is rare in the West but common in parts of Asia and the Middle East. A total of 39 patients with Buerger's disease were investigated in the setting of a hospital in Bangladesh. All but one were male and the mean age at onset of symptoms was 34 years. All but two were current smokers with a mean duration of smoking history of 17 years before the onset of symptoms. No other risk factors were identified. The majority of patients had ulceration or gangrene at presentation, and all but one had palpable femoral pulses. Vascular reconstruction was not possible in this institution and the main treatment options adopted were antibiotics, analgesia, chemical sympathectomy and amputation.     

Clinical study of chronic arachnoiditis in the posterior fossa

Summary A series of 12 cases of chronic arachnoiditis in the posterior fossa is reported.The diagnosis in six patients was established at suboccipital craniectomy which brought about marked improvement in symptoms. The other six were diagnosed as having arachnoiditis in the posterior fossa from the facts that improvement in clinical symptoms was achieved by lumbar air insufflation, which has often been said to be effective in arachnoiditis, and no abnormal findings were revealed by pneumoencephalo-roulette tomography.By investigating the clinical features of these cases, we came to the following conclusions: many cases of this disease have had head trauma or chronic inflammatory disease in the past; the symptoms are apt to occur abruptly, and display repeated remission and aggravation; symptoms originating in the cranial nerves are diversified, and are often bilateral: peripheral vestibular disturbances and retrocochlear hearing losses are observed on neuro-otological examination. In the caloric test especially the patients show canal paresis (CP) on the more impaired side, but none of them appeared to be nonresponsive to the test; lumbar air insufflation is often an effective treatment for this disease; pneumoencephalo-roulette tomography is very useful for diagnosing pathological changes in the posterior fossa.     

Primary diffuse tracheobronchial amyloidosis

The case is reported of a woman who died at the age of 36 years from obstructive respiratory failure due to diffuse tracheobronchial amyloidosis which had caused symptoms for six years. When first seen her symptoms of wheezing cough and mucopurulent sputum sometimes streaked with blood were of recent onset, but on bronchogram and bronchoscopy her disease was already widespread. Appearances at bronchography were interpreted as malformation and at bronchoscopy as tracheobronchitis, possibly tuberculous. Bronchoscopic biopsy was necessary to make the diagnosis of amyloidosis. The amyloid gave the usual staining reactions with an apple-green birefringence in polarized light following staining with Congo red. At necropsy an extensive histological survey proved that the amyloid was confined to the trachea and main stem bronchi. There was no associated disease, no family history, and no upset in plasma proteins.     

Carotid endarterectomy. Long term follow-up with specific reference to recurrent stenosis, contralateral progression, mortality and recurrent neurological episodes

This study follows up 292 carotid endarterectomy operations in 256 patients using the colour coded carotid Doppler Imager and oculoplethysmography and reports the rate of ipsilateral restenosis, contralateral progression, late (after 30 days postoperatively) neurological symptoms and mortality in life table form. The restenosis rate was 15% at 5 years. No case of restenosis presented with symptoms and none required reoperation. The contralateral progression rate from less than 50% internal carotid artery stenosis to greater than 50% was 20% over 5 years. Significantly more symptoms were associated with cases of contralateral progression (p = 0.05). The late neurological symptom rate was 14% at 5 years and was associated with widespread intra/extracranial vascular disease rather than restenosis. The late death rate was 12% at 5 years of which 40% were due to myocardial infarction. The overall postoperative death rate over 5 years was the same as an age and sex matched population. A more aggressive approach to coronary artery disease does not appear justified. The value of noninvasive tests in the post-endarterectomy situation is stressed with particular reference to documentation of contralateral progression.     

NEUROGENIC CLAUDICATION SECONDARY TO VASCULAR DISEASE

Book reviews in this article: Neurogenic claudication is characterized by sensory symptoms which appear during exercise or while maintaining a fixed posture. They are paraesthetic in quality, may be associated with ‘march’ phenomena, and patients may have bowel and bladder disturbance. The problem is most commonly secondary to lumbar canal stenosis (LCS) but rarely due to aortic disease, as shown in this case report. This report concerns a 66 year old woman who presented with symptoms of paraesthesia radiating from the buttocks to the thigh and, intermittent loss of bladder and bowel function, all associated with walking. There were no associated symptoms of vascular claudication. Clinically, there was evidence of aorto-iliac obstruction with absence of femoral pulses and a vascular index of 0.4 at both ankles. Neurological examination was normal at rest but the left ankle jerk was absent immediately after exercise. Myelogram and computerized tomographic (CT) scan were normal. An aortogram revealed a very tight irregular stenosis of the aorta at the level of the renal and mesenteric arteries. Very few lumbar vessels were seen. An aortic endarterectomy via a thoraco-abdominal approach was performed and an aortobifemoral graft inserted. The patient's symptoms resolved following this procedure. We postulate that her symptoms were caused by a ‘steal’ from the blood supply to the cauda equinda due to the severe athromatous disease of her upper abdominal aorta.     

Natural history of carotid artery disease on the side contralateral to endarterectomy

The natural history of the nonoperated carotid artery opposite an endarterectomy was examined in 134 patients by means of ultrasonic duplex scanning over a period extending to 48 months. None of the nine deaths that occurred during follow-up was stroke related. A total of 22 arteries showed progression of disease over this period. By life-table analysis the mean annual rate of progression for all categories of disease was 12.6% and 7.4% for progression to a diameter reduction greater than 50%. Disease progression was more rapid in patients under 65 years of age. Symptoms occurred in 13 patients for an overall incidence of 10% and a mean annual rate estimated at 5%. All symptoms indicated transient ischemic attacks; there were no strokes. There was a strong relationship between the development of symptoms and stenoses greater than 80% either at the initial examination or secondary to progression. No correlation was found between the presence of bruits or their change over time and the progression or appearance of symptoms. Conservative management of nonoperated vessels opposite an endarterectomy appears appropriate until symptoms develop or a lesion greater than 80% is detected.     

Avoidable delay in the management of carcinoma of the right colon.

A retrospective study of 89 consecutive patients with carcinoma of the right colon presenting in a district general hospital over a 5-year period is reported. Of the patients, 74% were anaemic at the time of diagnosis and 27% of these had anaemia or a low mean corpuscular volume (MCV) for a significant time (mean 177 days, range 76-496 days) before developing symptoms. All doctors should be more vigilant towards anaemia or low MCV as presymptomatic indicators of possible colonic disease. Appropriate investigation is required in order to detect disease at an earlier stage and therefore influence survival. Significant delay in the diagnosis of symptomatic disease occurs before referral to hospital (mean 61 days vs 36 days, P < 0.05). Treatment delay is similar whether patients are referred to surgeons or physicians. The preoperative duration of symptoms for emergency admissions was significantly shorter than for elective admissions (mean 50 days vs 119 days, P < 0.05). The 30-day mortality was significantly higher for emergency admissions (20.7% vs 3.3%, P < 0.05). Earlier diagnosis of symptomatic disease may not reduce the proportion of emergency admissions (33%) or improve survival. Many tumours are at an advanced pathological stage (39% node positive) by the time symptoms develop.     

Differentiation of vascular and neurogenic claudication

Lower extremity pain caused by exercise but relieved by rest is usually a reliable symptom of chronic arterial insufficiency. However, similar discomfort often occurs in patients who have neurospinal compression. Furthermore, both arterial occlusive disease and neurogenic causes of lower extremity discomfort may present simultaneously. Forty patients with symptoms that suggested intermittent claudication comprised our study group. All had non-arterial cause of their complaint. The nonvascular origin of the symptoms was suggested initially by clinical evaluation in 30 patients and by noninvasive evaluation in 25 patients. The neurospinal origin of symptoms was obscured in 15 patients because of the concomitant presence of significant arterial occlusive disease as demonstrated by noninvasive arterial testing. Twelve of these 15 patients underwent arterial reconstruction, which did not relieve their symptoms. Subsequently, the neurospinal origin of their symptoms was proven by appropriate evaluation and therapy. Forms of evaluation that proved helpful in the differential diagnosis were lumbosacral spine x-rays, electromyography, nerve conduction velocity studies, computerized tomography, Doppler noninvasive assessment and, at times arteriography and contrast myelography.     

Giovan Battista Morgagni, a pioneer of clinical nephrology

In the 18th century, Giovanni Battista Morgagni was the first to propose that specific signs and symptoms are linked to particular anatomical changes at autopsy and that these changes were the cause of the disease. This paper describes the report by Morgagni wherein he linked the anatomic findings at autopsy, specifically atrophied kidneys, with the signs and symptoms of a disease now known as uremia. From these findings, Morgagni felt that he had identified the factors responsible for the disease as well as its clinical course.