Rare diseases of the chest area Therapy

Congenital pulmonary arteriovenous malformation (AVM)

Diagnostics

Computed tomography is usually sufficient. MRI or perfusion scintigraphy can also contribute to the diagnosis. Classical angiography is particularly important due to the possibility of simultaneous therapy.

Treatment

All symptomatic and asymptomatic AVMs above a certain size require treatment. Multiple AVMs can be treated adequately using angiographic procedures with coils or embolization. Complex or large AVMs, or if angiography is not effective, are treated by resection.

Congenital cystic adenomatoid malformation (CCAM)

Diagnostics

Conventional chest X-ray and computed tomography show a mass, usually localized in the lower lobes. The CT often (not always!) also shows the arterial inflow.

Treatment

As a rule, resection is recommended for intralobar sequestration. In symptomatic disease with hemotpoea and recurrent infections, surgery is the treatment of choice. Asymptomatic patients should also be operated on to prevent the symptoms mentioned.
Extralobar sequestration as an incidental finding can be monitored provisionally in the absence of symptoms.

Bronchogenic cyst

Diagnostics

The conventional X-ray image usually already shows the cystic structure and the computer tomography confirms the diagnosis.

Treatment

Resection of the cyst is the treatment of choice. The operation can often be performed minimally invasively.

Congenital lobar emphysema

Diagnostics

The diagnosis can often be made prenatally using ultrasound or MRI. Later, a computer tomography is performed primarily after the conventional X-ray. Perfusion scintigraphy may rarely be necessary for better diagnosis.

Treatment

The treatment of choice is resection, although a lobectomy is often necessary in newborns. Adolescents usually require a partial resection in the sense of a lung volume reduction.

Rare tumors of the pleura

Diagnostics

A rare tumor of the pleura is the solitary fibrous tumor. It is difficult to differentiate the lesion from the lung tissue using conventional radiology. Computed tomography is the examination of choice, but often little additional information can be obtained. SFTs with a pedicle often show a changing intrathoracic position when the patient changes position. The density of the tumor is very heterogeneous, and the larger lesions may well lack evidence of a pleural tumor on CT.

Treatment

Treatment consists of resection in healthy tissue, either thoracoscopically or via thoracotomy, depending on the size of the tumor. 80% of SFTs are benign. Most arise from the visceral pleura and have a defined stalk. Tumors that grow into the lung or arise from the parietal pleura at the mediastinum or diaphragm are more frequently malignant.

Tumors of the trachea

Diagnostics

Computed tomography usually shows the tracheal mass, bronchoscopy shows the endoluminal findings and confirms the histological diagnosis. In addition, other malignant diseases must be ruled out or treated. recognize pathologies (lung, thyroid, esophagus, ear, nose, throat, etc.).

Treatment

As a rule, primary surgery is sought. The affected segment of the trachea is resected and an end-to-end anastomosis is performed. Adjuvant radiotherapy is usually recommended for malignant processes. For the palliation of inoperable tumors, bronchoscopic laser application can often provide short- to medium-term symptom relief. Bronchoscopically inserted stents also play a major role in the palliative setting.

Tracheal stenosis

Diagnostics

While computed tomography usually already shows the narrowing and excludes or confirms possible external causes, bronchoscopy is essential for the diagnosis.

Treatment

The treatment of choice is primary resection with end-to-end anastomosis. Depending on the extent and type of stenosis, laser or stents can also be used.

Esophagotracheal fistulas

Diagnostics

A conventional radiologic esophageal passage with contrast medium is often able to visualize the fistula. Computed tomography does not make a major contribution to the diagnosis of a fistula, but remains an examination of choice for clarifying and staging suspected tumors. Fistulas can usually be diagnosed endoscopically. Bronchoscopy in particular has proven its worth here. A simultaneous esophagoscopy can be helpful.
Both procedures are also used for tissue preservation in cases of suspected malignancy and for germ preservation.

Treatment

The therapy differs fundamentally depending on the type of fistula and the underlying disease. In the non-ventilated patient without malignant disease as the cause of the fistula, the initial focus is on adequate nutrition and correct treatment of pulmonary complications. In most cases, a one-sided operative procedure is the therapy of choice. The fistula is resected, the defect closed and secured with an interposition flap (e.g. muscle flap).

An esophagotracheal fistula after intubation can also often be treated with a single-stage operation, although partial tracheal resections are often necessary.
Traumatic fistulas are often accompanied by serious infections, which affect management accordingly. In principle, the indication for esophagostomy should be given generously. While a temporary esophagostomy is often necessary for postoperative fistulas after esophageal resection, a complete esophagectomy is often recommended for severe necrotizing inflammation.
In the case of malignant diseases, palliative stents are the treatment of choice, as surgery is rarely expedient due to the poor prognosis of the underlying disease and the high surgical morbidity and mortality.

Swyer-James syndrome

Diagnostics

The conventional X-ray image and the computer tomography show the hypertransparent lung sections. As a rule, an entire page is affected. An important differential diagnosis is congenital lobar emphysema.

Treatment

Medication measures such as bronchodilators and steroids and the treatment of any infections are the main focus alongside active pulmonary rehabilitation. Surgical resection is rarely performed, mainly to treat possible complications. Depending on the findings, the operation can be performed to reduce the lung volume.

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