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Biomedical subjects

C J Westermann

Publications and source records attributed to C J Westermann.

At least 37 records · Page 2Linked to original sources

Screening family members of patients with hereditary hemorrhagic telangiectasia.

PURPOSE: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant inherited disorder which may give rise to arteriovenous malformations in lungs and brain. When left untreated, these may cause serious complications. We screened family members of HHT patients for presence of the disease and associated pulmonary or cerebral arteriovenous malformations. PATIENTS AND METHODS: We investigated 98 family members of HHT patients on an outpatient basis. A stepped screening protocol was used based on prevalence of different manifestations of HHT. RESULTS: Thirty-six cases of HHT were found in the 98 persons screened. Pulmonary arteriovenous malformations were found in 12 of the 36 patients (33%), and cerebral arteriovenous malformations in 4 (11%). Therapy was recommended in 9 patients with pulmonary arteriovenous malformations and in 2 with cerebral arteriovenous malformations. CONCLUSIONS: Family members of known HHT patients should be encouraged to engage in a screening program, since the prevalence of potentially serious localizations is higher than previously thought.

Arteriovenous Malformations↗

Embolisation of pulmonary arteriovenous malformations: results and follow up in 32 patients.

BACKGROUND: Pulmonary arteriovenous malformations may cause a number of complications when left untreated. Embolisation of the feeding vessels is a relatively new approach and information concerning its efficacy and long term results is scarce. METHODS: Pulmonary arteriovenous malformations with feeding arteries of > 3 mm were treated by embolisation. Right to left shunt fraction and arterial oxygen pressure breathing air (PaO2) were measured before and after treatment. Six monthly measurement of shunt fraction was used for follow up. RESULTS: In 32 patients 92 pulmonary arteriovenous malformations were treated by coil embolisation. Mean shunt fraction decreased from 16.6% to 7.4% and PaO2 increased from 9.6 kPa to 11.5 kPa. Treatment was incomplete in two patients, one of whom was subsequently treated surgically. Serious complications occurred in one patient. Recanalisation of embolised vessels occurred in two cases after 22 and 28 months, respectively. Mean period of follow up was 25 months. CONCLUSIONS: Embolisation is a safe and efficacious treatment for most pulmonary arteriovenous malformations. Long term studies are necessary to determine the risk of recanalisation.

Adolescent↗

The scimitar syndrome: clinical spectrum and surgical treatment.

We present seven patients with the scimitar syndrome. The clinical and anatomical spectrum is described. Two different types of scimitar vein was recognized. a) simple classical vein running from the middle of the right lung to the cardiophrenic angle (5 patients) and b) double arched vein in the upper and lower lung zone, with ample drainage into the left atrium and inferior caval vein (2 patients). Four patients required surgical treatment. Indications, diagnostic procedures and surgical management are discussed. In two patients, thrombosis and fibrosis occurred in the scimitar vein that had been reimplanted in the left atrium, necessitating pneumonectomy.

Adult↗

Aortobronchial fistula after correction of congenital cardiovascular abnormalities.

Aortobronchial fistula (ABF) is a rare but highly lethal condition. Four patients with ABF, 10-25 years after surgical repair of a congenital cardiovascular abnormality are reported. All patients presented with haemoptysis. Computed tomographic (CT) scan and aortography were inconclusive in two, diagnostic in one and not performed in another. Three patients underwent operation: all survived and are free of symptoms with a follow-up of 2-8 yrs. The fourth patient died before operation due to massive haemorrhage into the lung. In all patients, the fistula was secondary to aortic interposition of patch grafts.

Adult↗

Relationship between lung tissue and blood plasma concentrations of inhaled budesonide.

In 11 patients, in whom a lung lobe or whole lung was to be resected, a single dose of 1.6 mg inhaled budesonide was given pre-operatively. In 9 of them, concentrations of the drug in both lung tissue and blood plasma were measured. Budesonide concentrations in lung tissue, at least 90 min after dosage, were 2.1-8.9 nmol kg-1. Concentrations in blood plasma (0.27-1.1 nmol kg-1) were 1/8th of those in lung tissue.

Administration, Inhalation↗

Unruptured intracranial arteriovenous malformations with hereditary haemorrhagic telangiectasia. Neurosurgical treatment or not?

Clinical decision analysis is applied to treatment decisions for three patients with unruptured familial intracranial arteriovenous malformations (AVMs) in association with hereditary haemorrhagic telangiectasia (HHT). The grades of the AVMs--according to Spetzler--were 1-0-0 (two patients aged 12 and 14) and 2-0-1 (one patient aged 43). The AVM in one patient (graded 1-0-0) was operated on without sequelae. In the decision analysis, life expectancy is used as an outcome measure, with and without quality of life adjustment and discounting for time preference. It is concluded that neurosurgical treatment is the optimal strategy for all three patients. Because no conclusive data are available concerning the natural history and neurosurgical treatment of unruptured AVMs, we recommend the prospective application and testing of AVM grading systems. General treatment guidelines balancing the lifetime risk of haemorrhage, risk of incomplete extirpation and surgical mortality and morbidity are given. The analysis suggests that patients with low-graded AVMs which have not bled should be treated surgically, unless they are older than 60 years and have an increased risk of incomplete extirpation.

Adolescent↗

Pulmonary resection after pneumonectomy in patients with bronchogenic carcinoma.

Eight patients with a previous pneumonectomy for bronchogenic carcinoma underwent an additional resection because of a second primary carcinoma in the remaining lung. One patient died of pulmonary embolism in the postoperative period. The postoperative course was otherwise uneventful except for prolonged air leak. Two patients died after 3 months (bone metastasis) and 5 months (recurrent small-cell carcinoma). Two patients were alive at the time this article was written but had evidence of recurrence after 18 months (distant metastasis) and 21 months (local recurrence at the site of positive resection margins). Three patients were alive and doing well without evidence of disease after 16, 17, and 40 months. After careful selection, even patients with a previous pneumonectomy may be good candidates for additional resection of a second primary bronchogenic carcinoma.

Aged↗

[Late manifestation of a right-sided diaphragmatic rupture].

Diaphragmatic rupture is commonly of traumatic origin and can complicate thoracoabdominal injuries. Some cases are diagnosed after a delay of years. We present three patients with a rupture of the right hemi-diaphragm with herniation of liver fragments, and discuss the diagnostic and therapeutic challenge.

Adolescent↗

Postpneumonectomy syndrome in adulthood. Surgical correction using an expandable prosthesis.

The postpneumonectomy syndrome is a rare complication occurring after right pneumonectomy and is seen mainly after pneumonectomy in childhood. The presenting symptoms are dyspnea, stridor, and recurrent pulmonary infections. The syndrome is caused by the shifting and rotation of the heart and mediastinum into the right hemithorax, and anterior herniation of the left lung. This causes tortuosity and stretching of the trachea and compression of the left main bronchus and left lower lobe bronchus, eventually resulting in secondary tracheobronchomalacia. This report reviews two cases of postpneumonectomy syndrome following pneumonectomy in adulthood. After implantation of an expandable prosthesis, an anatomic correction of the shifted mediastinum was achieved, which in both cases resulted in instantaneous and sustained relief.

Adult↗

A twenty-five-year follow-up of ninety-three resected typical carcinoid tumors of the lung.

From 1965 to 1990, 93 patients (57 women and 36 men) with typical bronchopulmonary carcinoids were operated upon. Patient ages ranged from 17 to 78 years, the mean age being 45.5 years. Central carcinoids were symptomatic in 80% of the patients. A correct preoperative diagnosis was made in 54 of 64 (84%) patients. Peripheral carcinoids were usually asymptomatic and a correct diagnosis was established in 4 of 29 patients (14%). The prognosis in the group of patients with bronchopulmonary carcinoids treated surgically was excellent. Seven patients died from nonrelated causes. The 5-, 10-, and 15-year survival rates for the remaining 86 patients are 100%. Only one patient died as a result of the tumor after 17 years and another patient is known to have had distant metastasis 9 years after resection. There was no hospital mortality. In the last decade a lung parenchyma-preserving attitude was adopted. Whenever possible, bronchoplastic surgery was applied for central carcinoids and resection of one segment or less was used for peripheral carcinoids. This approach was possible in 30 of 50 patients (60%). Nine patients were treated with preoperative endobronchial neodymium:yttrium-aluminum-garnet laser resection to facilitate a lung-preserving surgical resection. The prognosis of patients for whom a lung-preserving approach was adopted was as good as that of those with conventional resections. Involvement of regional lymph nodes (nine patients, 9.7%) or positive resection margins (two patients, 2.1%) had no influence on prognosis. We conclude that lung-preserving resections are often facilitated by preoperative neodymium:yttrium-aluminum-garnet laser treatment in central obstructing carcinoids.

Adolescent↗

[Tracheobronchial lesions after closed thoracic injuries].

We report the clinical picture and the outcome after medical and surgical treatment of eleven patients who had a traumatic rupture of the tracheobronchial tree between 1971 and 1990. The lesions occurred after a blunt chest trauma. All patients but one had evidence of extrapulmonary air leak on admission; one patient complained of hemoptysis. Seven patients underwent a fibroscopy on admission, which confirmed the rupture. One of them was operated immediately for tracheal rupture; four presented a small lesion which was medically treated; two were operated later on for secondary stenosis. Four patients didn't undergo a fibroscopy at first examination, one of them being asymptomatic. All four were operated secondarily. Of the six patients operated for secondary stenosis, three were operated within one month after the accident. This reflects the importance of other priorities at the initial stage. Of the three patients operated later on two didn't have a fibroscopy and it is impossible to assess the importance of the primary lesion. For the last patient operation was delayed because of the peripheral location of the lesion. The long term evolution was satisfactory in all cases.

Adolescent↗

Complications of endobronchial neodymium-Yag (Nd:Yag) laser application.

Endobronchial Nd:Yag laser therapy is an excellent tool to reopen the central airway(s) in patients with severe respiratory symptoms caused by endobronchial tumor growth. Rigid bronchoscopy should be the method of choice. Usually general anesthesia is necessary. Complication rate depends on the condition of the patient, the localization of the tumor, patient selection criteria, method of endoscopy, method of anesthesia, method of laser energy application, and last but not least, the operator's skills and the treating team's cooperation. A very careful monitoring of the patient's condition during and after the operative procedure is mandatory. Only when all these conditions are fulfilled can endobronchial Nd:Yag laser therapy have a great benefit for the patient with a low grade, acceptable complication rate.

Bronchoscopes↗