Bronchopulmonary Sequestration
Hydrops Fetalis
Bronchopulmonary Dysplasia
Aspergillosis, Allergic Bronchopulmonary
Angiography, Digital Subtraction
Ventilation-Perfusion Ratio
Technetium Tc 99m Aggregated Albumin
Pulmonary Artery
Diagnosis of pulmonary sequestration by spiral CT angiography. (1/92)
The diagnosis of pulmonary sequestration traditionally requires arteriography to identify abnormal systemic vessels feeding the abnormal portion of the lung. Non-invasive imaging techniques have recently been used to replace arteriography. Conventional computed tomographic (CT) scanning is, however, at a disadvantage because of its inability to obtain multiplanar images. The combination of slip ring CT scanning and computerised three-dimensional reconstruction (spiral CT angiography) can be used to visualise the anatomical detail of a wide range of vessels within the lung. Four cases of pulmonary sequestration are reported which were successfully diagnosed using spiral CT angiography. Spiral CT scanning allows simultaneous imaging of anomalous vessels and lung parenchyma in a single examination and is particularly useful in the diagnosis and assessment of pulmonary sequestration. (+info)Direct intrauterine fetal therapy in a case of bronchopulmonary sequestration associated with non-immune hydrops fetalis. (2/92)
Bronchopulmonary sequestration associated with non-immune hydrops fetalis is generally recognized as a uniformly fatal fetal condition without fetal surgical intervention. We describe here the first case of such a condition treated successfully with direct intrauterine fetal therapy using digoxin and frusemide. (+info)Management of antenatally diagnosed pulmonary sequestration associated with congenital cystic adenomatoid malformation. (3/92)
BACKGROUND: Sequestration with associated cystic adenomatoid malformation is rare. A study was undertaken to determine whether pulmonary sequestration associated with congenital cystic adenomatoid malformation has a more favourable natural history than that of sequestration without associated cystic adenomatoid malformation. METHODS: An outline of the postnatal work up leading to the management of extralobar or intralobar pulmonary sequestration with congenital cystic adenomatoid malformation diagnosed antenatally as pulmonary malformation is presented and the indications for surgical intervention are discussed. RESULTS: In five infants in whom an antenatal ultrasound scan had detected a congenital lung malformation at 18-19 weeks gestation a final diagnosis of extralobar or intralobar pulmonary sequestration with congenital cystic adenomatoid malformation was made postnatally. Postnatal ultrasound and computerised axial tomographic scans confirmed the diagnosis of sequestration by delineating anomalous vascular supply. Cystic changes were also observed in the basal area of the sequestration in all patients. Four children remained asymptomatic and one infant presented at 10 months of age with pneumonia. The mean age at surgical resection was 6.8 months (range 2-10). Histopathological examination confirmed intralobar pulmonary sequestration with associated Stocker type 2 congenital cystic adenomatoid malformation in two patients and extralobar pulmonary sequestration with associated Stocker type 2 congenital cystic adenomatoid malformation in three patients. The mean period of follow up was four years (range 1-8). The children remain well and are developing normally. CONCLUSIONS: The importance of seeking an anomalous blood supply in children with congenital lung lesions is emphasised. Pulmonary sequestration and congenital cystic adenomatoid malformation probably share a common embryogenesis despite diverse morphology. The natural history of antenatally diagnosed lung masses is variable. Early postnatal surgical resection of pulmonary sequestration with cystic adenomatoid malformation is recommended. Surgical excision should be conservative, sparing the normal lung parenchyma. (+info)A familial case of pulmonary arterial sequestration. (4/92)
The cases of a mother and infant son are reported, both with a rare type of pulmonary sequestration where the arterial supply to the lung arises from the systemic circulation. This is a familial case of arterial sequestration. In both patients, the lung parenchyma was radiologically normal. (+info)Primary lymphoepithelioma-like carcinoma within an intralobular pulmonary sequestration. (5/92)
A 31-yr-old Chinese female, a nonsmoker, presented with digital clubbing and coughing. Diagnostic evaluation revealed a pulmonary sequestration in the left lower lobe. During surgery a tumour was discovered, which turned out to be a lymphoepithelioma-like carcinoma. These tumours are mainly found in Asians, and are associated with the Epstein Barr virus and not with smoking. They are thought to have a better prognosis and to be more chemosensitive. The need for surgical treatment of pulmonary sequestration and the recognition of lymphoepithelioma-like carcinoma as a distinct clinicopathological entity is emphasized. (+info)Contrast enhanced MR angiography in pulmonary sequestration. (6/92)
OBJECTIVE: To evaluate the feasibility of contrast enhanced three-dimensional (3D) magnetic resonance angiography (MRA) in identifying the systemic blood supply in pulmonary sequestration. METHODS: Three patients who were suspected of pulmonary sequestration were examined by contrast enhanced 3D MRA. MR images were compared with surgical findings. RESULTS: Contrast enhanced 3D MRA clearly demonstrated systemic arteries from the descending thoracic aorta supplying the basilar segments of the lower lobe in each case. Pulmonary veins from these segments, which drained into the left atrium in two cases, were also detected. MRA images were consistent with those observed in surgery. CONCLUSION: Contrast enhanced 3D MRA allows accurate diagnosis of pulmonary sequestration and may obviate the need for more invasive investigations. (+info)Intralobar pulmonary sequestration presenting increased serum CA19-9 and CA125. (7/92)
A 39-year-old man was admitted to our hospital for further evaluation of a consolidated shadow and clarification of the cause of serum tumor marker elevation (CA19-9 496.2 U/ml, CA125 160.6 U/ml). Chest computed tomography revealed a well-defined homogeneous nodule in the left S(10). Angiography showed one aberrant artery, branching from the ascending aorta. Intralobar pulmonary sequestration was diagnosed and the sequestrated lung was resected. Microscopic findings of the sequestrated lung showed a mucus-containing cystically dilated bronchus, which was covered with ciliated cylindrical epithelium. Immunohistochemical staining showed positive staining for CA19-9 and CA125 in both the ciliated cylindrical epithelium and mucus. Serum values of tumor markers returned to their normal range after surgery. (+info)Pulmonary sequestration associated by Mycobacterium intracellulare infection. (8/92)
A case of a 29-year-old woman with intralobar pulmonary sequestration infected with Mycobacterium intracellulare is presented. A chest CT scan revealed a density in the posterior segment of the left lower lobe, and an acid-fast bacillus sputum culture yielded Mycobacterium intracellulare. After 3 months of treatment with clarithromycin, streptomycin, rifampicin and ethambutol, the patient underwent partial resection of the left lower lobe. At the 6-month follow-up the patient's clinical status is excellent. A review of the literature revealed only three case reports of pulmonary sequestration associated with Mycobacterium avium-intracellulare complex infection. (+info)Also found in: Dictionary, Thesaurus, Medical, Encyclopedia.
Bronchopulmonary Sequestration (BPS) is a rare congenital abnormality that affects the lungs and airways. It occurs when a portion of the lung develops abnormally during fetal development, resulting in a separate compartment or "sequestrum" that is not connected to the main airway. This sequestrum is surrounded by a membrane and contains blood vessels, air cells, and other structures found in normal lung tissue.
The symptoms of BPS can vary depending on the size and location of the sequestrum, but may include:
* Difficulty breathing (dyspnea)
* Coughing up blood (hemoptysis)
* Chest pain (toracalgia)
* Fatigue
* Shortness of breath (dyspnea)
* Recurrent respiratory infections
BPS can be diagnosed using imaging tests such as chest X-rays, computed tomography (CT) scans, and magnetic resonance imaging (MRI). A lung biopsy may also be performed to confirm the diagnosis.
Treatment for BPS typically involves surgery to remove the sequestrum and repair any damage to the surrounding lung tissue. In some cases, surgery may not be possible or may not completely resolve the symptoms, in which case other treatments such as oxygen therapy, respiratory therapy, and medications may be used to manage the condition.
Overall, Bronchopulmonary Sequestration is a rare and complex congenital abnormality that requires careful evaluation and management by a team of medical professionals, including pulmonologists, surgeons, and other specialists. With appropriate treatment, many individuals with BPS can experience improved respiratory function and quality of life.
The term "hydrops" refers to the excessive accumulation of fluid in the body, and "fetalis" indicates that the condition occurs during fetal development. The condition is often diagnosed during the second or third trimester of pregnancy, and it can be associated with other congenital anomalies or genetic disorders.
The symptoms of hydrops fetalis can vary depending on the underlying cause, but they may include:
* Enlargement of the fetus
* Increased amniotic fluid levels
* Poor fetal growth
* Abnormalities in the ultrasound examination
* Premature birth or stillbirth
Hydrops fetalis is a serious condition that requires close monitoring and management by a multidisciplinary team of healthcare providers, including obstetricians, maternal-fetal medicine specialists, and perinatologists. Treatment options may include:
* Close monitoring of the pregnancy to detect any complications early
* Medications to help manage symptoms such as high blood pressure or heart failure
* Surgical interventions, such as amnioreduction or fetoscopy, to reduce fluid accumulation and improve fetal growth
* In some cases, delivery of the baby may be necessary, either through cesarean section or vaginal delivery.
The prognosis for hydrops fetalis is generally poor, with high rates of stillbirth and neonatal mortality. However, with early diagnosis and appropriate management, the outcome can be improved. It is important for pregnant women to seek medical attention immediately if they experience any symptoms or abnormalities that may indicate hydrops fetalis.
The exact cause of BPD is not fully understood, but it is thought to be related to a combination of genetic and environmental factors. Babies who are born prematurely or have low birth weights are at higher risk for developing BPD.
Symptoms of BPD can include rapid breathing, difficulty breathing, and bluish color of the skin (cyanosis). Diagnosis is typically made through a combination of physical examination, medical history, and diagnostic tests such as chest X-rays or blood tests.
There is no cure for BPD, but treatment options are available to help manage symptoms and improve lung function. These may include oxygen therapy, respiratory therapy, and medications such as bronchodilators or steroids. In severe cases, babies with BPD may require mechanical ventilation.
Long-term outcomes for babies with BPD can vary widely, depending on the severity of the disease and other individual factors. Some children may experience ongoing breathing problems and developmental delays, while others may recover fully with time. With appropriate treatment and support, however, many babies with BPD are able to lead healthy lives.
The prognosis for BPD is generally better for babies who are born at later gestational ages and have fewer other health problems. However, even with appropriate treatment, some babies with BPD may experience ongoing breathing difficulties and other complications throughout their lives. These may include:
* Respiratory infections: Babies with BPD are at higher risk for developing respiratory infections such as pneumonia, which can be serious and potentially life-threatening.
* Chronic lung disease: BPD can lead to long-term breathing problems and chronic lung disease, which can require ongoing medical treatment.
* Developmental delays: Babies with BPD may experience developmental delays and learning disabilities, particularly if they spent a significant amount of time in the neonatal intensive care unit (NICU).
* Behavioral and emotional problems: Some children with BPD may experience behavioral and emotional problems, such as anxiety and depression, which can be related to their medical history and experiences.
Overall, while babies with BPD face a higher risk for ongoing breathing problems and other complications, many are able to recover fully with appropriate treatment and support. It is important for parents and caregivers to work closely with healthcare providers to monitor their child's condition and address any ongoing concerns or complications.
The main cause of ABPA is exposure to airborne spores of the fungus Aspergillus, which are commonly found in soil and decaying organic matter. Individuals with a pre-existing allergic condition may be more susceptible to developing an allergic reaction to these spores, leading to inflammation and damage to the airways.
Diagnosis of ABPA typically involves a combination of physical examination, medical history, and diagnostic tests such as chest X-rays, CT scans, and bronchoscopy with biopsy. Treatment for ABPA typically involves corticosteroids to reduce inflammation and antifungal medications to treat any underlying infection. In severe cases, hospitalization may be necessary to provide oxygen therapy and other supportive care.
Prevention of ABPA includes avoiding exposure to known allergens and maintaining good respiratory hygiene. This can involve regularly cleaning and disinfecting surfaces and objects, using HEPA filters in air purifiers, and wearing a mask when working with or around potentially contaminated materials.
Prognosis for ABPA is generally good if treated promptly and effectively, but untreated cases can lead to serious complications such as respiratory failure and other organ damage. With proper management and prevention strategies in place, individuals with ABPA can lead active and fulfilling lives.
The symptoms of pulmonary embolism can vary, but may include shortness of breath, chest pain, coughing up blood, rapid heart rate, and fever. In some cases, the clot may be large enough to cause a pulmonary infarction (a " lung injury" caused by lack of oxygen), which can lead to respiratory failure and death.
Pulmonary embolism can be diagnosed with imaging tests such as chest X-rays, CT scans, and ultrasound. Treatment typically involves medications to dissolve the clot or prevent new ones from forming, and in some cases, surgery may be necessary to remove the clot.
Preventive measures include:
* Avoiding prolonged periods of immobility, such as during long-distance travel
* Exercising regularly to improve circulation
* Managing chronic conditions such as high blood pressure and cancer
* Taking blood-thinning medications to prevent clot formation
Early recognition and treatment of pulmonary embolism are critical to reduce the risk of complications and death.
Pulmonary sequestration
Congenital pulmonary airway malformation
Hydrops fetalis
EXIT procedure
BPS
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Extralobar pulmonary sequestration3
- 2. Prenatal ultrasound characterization of the suprarenal mass: distinction between neuroblastoma and subdiaphragmatic extralobar pulmonary sequestration. (nih.gov)
- 3. Extralobar pulmonary sequestration mimicking neuroblastoma. (nih.gov)
- 20. Infradiaphragmatic extralobar pulmonary sequestration masquerading as an intra-abdominal, suprarenal mass. (nih.gov)
Intralobar4
- A sixty-three-year-old male patient was newly diagnosed with intralobar bronchopulmonary sequestration. (ctsnet.org)
- The pathology showed varying amounts of fibrous cystic changes and inflammation consistent with intralobar sequestration. (ctsnet.org)
- Konecna J, Karenovics W, Veronesi G, Triponez F. Robot-assisted segmental resection for intralobar pulmonary sequestration. (ctsnet.org)
- On investigation, she was diagnosed as a case of intralobar sequestration of the lung. (medscicaserep.com)
Congenital3
- Bronchopulmonary sequestration is a rare congenital abnormality of the lower respiratory tract. (fetalcaredallas.com)
- Broncho-pulmonary sequestration (BPS) is referred to simply as pulmonary sequestration, is a relatively uncommon congenital anomaly of the lower respiratory tract. (medscicaserep.com)
- Increased expression of this gene is associated with a distinct biologic subset of acute myeloid leukemia (AML) and the occurrence of bronchopulmonary sequestration (BPS) and congenital cystic adenomatoid malformation (CCAM) tissue. (nih.gov)
Pulmonary sequestrations3
- Melfi FM, Viti A, Davini F, Mussi A. Robot-assisted resection of pulmonary sequestrations. (ctsnet.org)
- Small- or moderate-sized pulmonary sequestrations that don't show much change during the pregnancy are generally managed after birth, usually with surgery, to remove the abnormal lung tissue. (fetalcaredallas.com)
- 8. Pitfalls in the sonographic diagnosis of juxtadiaphragmatic pulmonary sequestrations. (nih.gov)
Prenatal5
- Prenatal ultrasound-guided laser coagulation (USLC) for complicated bronchopulmonary sequestrations has been described but a consensus on the procedure and on the following management is still lacking. (bvsalud.org)
- In 80.6% of the cases investigated postnatally, a residual mass was found, 50% of cases who showed prenatal arterial flow cessation had a persistent sequestration postnatally, and 26.3% of cases underwent postnatal sequestrectomy. (bvsalud.org)
- Prenatal USLC seems to be a valid option for bronchopulmonary sequestration complicated by severe hydrothorax and/or fetal hydrops . (bvsalud.org)
- We report a case of a fetus with non-mosaic trisomy 20 in amniotic fluid cultures obtained during the prenatal evaluation of an unusual thoraco-abdominal mass which was found at autopsy to be pulmonary sequestration. (nih.gov)
- 7. Bronchopulmonary sequestration: prenatal diagnosis with clinicopathologic correlation. (nih.gov)
Resection1
- The other three underwent thoracoscopic resection, in two, a viable sequestration was found. (bvsalud.org)
Tumor2
- When a bronchopulmonary sequestration is detected, the high blood flow through the tumor can lead to excess fluid accumulation in the chest cavity (hydrops) and even heart failure. (fetalcaredallas.com)
- Neurogenic tumor arising within the pulmonary sequestration (PS) is rare. (biomedcentral.com)
Postnatal2
- Four cases underwent postnatal computed tomography (CT) scan in one case, there was no evidence of persistent bronchopulmonary sequestration . (bvsalud.org)
- 13. Antenatal and postnatal findings in intra-abdominal pulmonary sequestration. (nih.gov)
Patients1
- Both patients in our series had pathology examination confirming a viable bronchopulmonary sequestration . (bvsalud.org)
Lung tissue3
- Bronchopulmonary sequestration occurs when a mass of abnormal lung tissue forms in the fetal lungs. (fetalcaredallas.com)
- Bronchopulmonary sequestration (BPS), or pulmonary sequestration, is a condition in which one section of lung does not communicate with the normal lung tissue. (losangelesfetalsurgery.org)
- Pulmonary sequestration (PS) is mainly defined as a non-functioning lung tissue that has an unusual feeding artery mostly arising from the aorta, without certain pathological diagnostic criteria [ 3 ]. (biomedcentral.com)
Detection2
- Under these circumstances, bronchopulmonary sequestration may become life-threatening, which is why early detection is so important. (fetalcaredallas.com)
- 16. [A case of detection of pulmonary sequestration during the early stage of pregnancy and subsequent successful therapy]. (nih.gov)
Case1
- 19. A rare case of extra lobar abdominal bronchopulmonary malformation. (nih.gov)
Mass1
- Bronchopulmonary sequestration (BPS) is a mass of nonfunctioning pulmonary tissue that lacks an obvious communication with the tracheobronchial tree and receives all or most of its blood supply from anomalous systemic vessels (Carter, 1959). (fetalcaredallas.com)
Published cases1
- Savic B, Birtel FJ, Tholen W, Funke HD, Knoche R. Lung sequestration: report of seven cases and review of 540 published cases. (ctsnet.org)
Congenital1
- Increased expression of this gene is associated with a distinct biologic subset of acute myeloid leukemia (AML) and the occurrence of bronchopulmonary sequestration (BPS) and congenital cystic adenomatoid malformation (CCAM) tissue. (nih.gov)