Catamenial pneumothorax
Encyclopedia
Catamenial pneumothorax is a condition of collapsed lung
(pneumothorax) occurring in conjunction with menstrual periods (catamenial refers to menstruation
), believed to be caused primarily by endometriosis
of the pleura (the membrane surrounding the lung
).
lung nodule
s, as well as some exceptional presentations.
endometriosis
.
attaches within the thoracic cavity
, forming chocolate-like cyst
s. Generally the parietal pleura
is involved, but the lung itself, the visceral layer, the diaphragm
, and more rarely the tracheobronchial tree
may also be afflicted. How this endometrial tissue
reaches the thorax remains enigmatic. Defects in the diaphragm, which are found often in affected individuals, could provide an entry path, as could microembolization through pelvic veins.
The cysts can release blood
; the endometrial cyst "menstruates" in the lung. Air can move in by an unknown mechanism. The blood and air cause the lung to collapse (i.e. catamenial hemopneumothorax
).
can be hinted by high recurrence rates of lung collapse in a woman of reproductive age with endometriosis. CA-125
is elevated. Nowadays, video-assisted thoracoscopy
is used for confirmation.
can be a medical emergency
, as it can become associated with severe pain and decreased lung function. A chest tube
should be inserted after clinical assessment. This releases the air and menstrual blood, and the lung can re-expand.
Surgery, hormonal treatments and combined approaches have all been proposed, with variable results in terms of short and long term outcome. Surgical removal of the endometrial tissue should be endeavoured during menstruation for optimal visualisation of the cyst. Pleurodesis
may also be helpful. Menstruation and accompanying lung collapse can be suppressed with hormone therapy, like with Lupron Depot
, danazol
or extended cycle combined oral contraceptive pills.
of primary spontaneous pneumothorax (i.e. not due to surgical trauma etc.) in women (about 1/100'000/year), this is a very rare condition. Hence, many basic textbooks don't mention it, and many doctors
have never heard of it. However, catamenial pneumothorax is probably under-recognised.
Pneumothorax
Pneumothorax is a collection of air or gas in the pleural cavity of the chest between the lung and the chest wall. It may occur spontaneously in people without chronic lung conditions as well as in those with lung disease , and many pneumothoraces occur after physical trauma to the chest, blast...
(pneumothorax) occurring in conjunction with menstrual periods (catamenial refers to menstruation
Menstruation
Menstruation is the shedding of the uterine lining . It occurs on a regular basis in sexually reproductive-age females of certain mammal species. This article focuses on human menstruation.-Overview:...
), believed to be caused primarily by endometriosis
Endometriosis
Endometriosis is a gynecological medical condition in which cells from the lining of the uterus appear and flourish outside the uterine cavity, most commonly on the ovaries. The uterine cavity is lined by endometrial cells, which are under the influence of female hormones...
of the pleura (the membrane surrounding the lung
Lung
The lung is the essential respiration organ in many air-breathing animals, including most tetrapods, a few fish and a few snails. In mammals and the more complex life forms, the two lungs are located near the backbone on either side of the heart...
).
Classification
Catamenial pneumothorax is the most common form of thoracic endometriosis syndrome, which also includes catamenial hemothorax, catamenial hemoptysis, catamenial hemopneumothorax and endometriosisEndometriosis
Endometriosis is a gynecological medical condition in which cells from the lining of the uterus appear and flourish outside the uterine cavity, most commonly on the ovaries. The uterine cavity is lined by endometrial cells, which are under the influence of female hormones...
lung nodule
Nodule (medicine)
For use of the term nodule in dermatology, see Nodule In medicine, a nodule refers to a relatively hard, roughly spherical abnormal structure....
s, as well as some exceptional presentations.
Symptoms and signs
Onset of lung collapse is less than 72 hours after menstruation. Typically, it occurs in women aged 30–40 years, but has been diagnosed in young girls as early as 10 years of age and post menopausal women (exclusively in women of menstrual age) most with a history of pelvicPelvis
In human anatomy, the pelvis is the lower part of the trunk, between the abdomen and the lower limbs .The pelvis includes several structures:...
endometriosis
Endometriosis
Endometriosis is a gynecological medical condition in which cells from the lining of the uterus appear and flourish outside the uterine cavity, most commonly on the ovaries. The uterine cavity is lined by endometrial cells, which are under the influence of female hormones...
.
Pathophysiology
Endometrial tissueEndometrium
-Function:The endometrium is the innermost glandular layer and functions as a lining for the uterus, preventing adhesions between the opposed walls of the myometrium, thereby maintaining the patency of the uterine cavity. During the menstrual cycle or estrous cycle, the endometrium grows to a...
attaches within the thoracic cavity
Thoracic cavity
The thoracic cavity is the chamber of the human body that is protected by the thoracic wall ....
, forming chocolate-like cyst
Cyst
A cyst is a closed sac, having a distinct membrane and division on the nearby tissue. It may contain air, fluids, or semi-solid material. A collection of pus is called an abscess, not a cyst. Once formed, a cyst could go away on its own or may have to be removed through surgery.- Locations :* Acne...
s. Generally the parietal pleura
Parietal pleura
The portion of the pleura external to the pulmonary pleura lines the inner surface of the chest wall, covers the diaphragm, and is reflected over the structures occupying the middle of the thorax; this portion is termed the parietal pleura....
is involved, but the lung itself, the visceral layer, the diaphragm
Thoracic diaphragm
In the anatomy of mammals, the thoracic diaphragm, or simply the diaphragm , is a sheet of internal skeletal muscle that extends across the bottom of the rib cage. The diaphragm separates the thoracic cavity from the abdominal cavity and performs an important function in respiration...
, and more rarely the tracheobronchial tree
Tracheobronchial tree
The tracheobronchial tree is the structure from the trachea, bronchi, and bronchioles that forms the airways that supply air to the lungs. It is within the neck and the chest. The structure looks like a tree because the trachea splits into the right and left mainstem bronchi, which "branch" into...
may also be afflicted. How this endometrial tissue
Endometrium
-Function:The endometrium is the innermost glandular layer and functions as a lining for the uterus, preventing adhesions between the opposed walls of the myometrium, thereby maintaining the patency of the uterine cavity. During the menstrual cycle or estrous cycle, the endometrium grows to a...
reaches the thorax remains enigmatic. Defects in the diaphragm, which are found often in affected individuals, could provide an entry path, as could microembolization through pelvic veins.
The cysts can release blood
Blood
Blood is a specialized bodily fluid in animals that delivers necessary substances such as nutrients and oxygen to the cells and transports metabolic waste products away from those same cells....
; the endometrial cyst "menstruates" in the lung. Air can move in by an unknown mechanism. The blood and air cause the lung to collapse (i.e. catamenial hemopneumothorax
Hemopneumothorax
Hemopneumothorax, or haemopneumothorax, is a medical term describing the combination of two conditions: pneumothorax, or air in the chest cavity, and hemothorax , or blood in the chest cavity....
).
Diagnosis
DiagnosisDiagnosis
Diagnosis is the identification of the nature and cause of anything. Diagnosis is used in many different disciplines with variations in the use of logics, analytics, and experience to determine the cause and effect relationships...
can be hinted by high recurrence rates of lung collapse in a woman of reproductive age with endometriosis. CA-125
CA-125
CA-125 also known as mucin 16 or MUC16 is a protein that in humans is encoded by the MUC16 gene. MUC16 is a member of the mucin family glycoproteins...
is elevated. Nowadays, video-assisted thoracoscopy
Thoracoscopy
Thoracoscopy is a medical procedure involving internal examination, biopsy, and/or resection of disease or masses within the pleural cavity and thoracic cavity...
is used for confirmation.
Treatment
PneumothoraxPneumothorax
Pneumothorax is a collection of air or gas in the pleural cavity of the chest between the lung and the chest wall. It may occur spontaneously in people without chronic lung conditions as well as in those with lung disease , and many pneumothoraces occur after physical trauma to the chest, blast...
can be a medical emergency
Medical emergency
A medical emergency is an injury or illness that is acute and poses an immediate risk to a person's life or long term health. These emergencies may require assistance from another person, who should ideally be suitably qualified to do so, although some of these emergencies can be dealt with by the...
, as it can become associated with severe pain and decreased lung function. A chest tube
Chest tube
A chest tube is a flexible plastic tube that is inserted through the side of the chest into the pleural space. It is used to remove air or fluid , or pus from the intrathoracic space...
should be inserted after clinical assessment. This releases the air and menstrual blood, and the lung can re-expand.
Surgery, hormonal treatments and combined approaches have all been proposed, with variable results in terms of short and long term outcome. Surgical removal of the endometrial tissue should be endeavoured during menstruation for optimal visualisation of the cyst. Pleurodesis
Pleurodesis
Pleurodesis is a medical procedure in which the pleural space is artificially obliterated. It involves the adhesion of the two pleura.- Uses :Pleurodesis is done to prevent recurrence of pneumothorax or recurrent pleural effusion...
may also be helpful. Menstruation and accompanying lung collapse can be suppressed with hormone therapy, like with Lupron Depot
Leuprolide
Leuprorelin or leuprolide acetate is a GnRH analog. Proper Sequence: Pyr-His-Trp-Ser-Tyr-D-Leu-Leu-Arg-Pro-NHEt - Mode of action:Leuprolide acts as an agonist at pituitary GnRH receptors...
, danazol
Danazol
Danazol is a derivative of the synthetic steroid ethisterone, a modified testosterone. Also known as 17alpha-ethinyl testosterone. Before becoming available as a generic drug, Danazol was marketed as Danocrine in the United States. It was approved by the U.S. Food and Drug Administration as the...
or extended cycle combined oral contraceptive pills.
Epidemiology
Some sources claim this entity represents 3-6% of pneumothorax in women. In regard of the low incidenceIncidence (epidemiology)
Incidence is a measure of the risk of developing some new condition within a specified period of time. Although sometimes loosely expressed simply as the number of new cases during some time period, it is better expressed as a proportion or a rate with a denominator.Incidence proportion is the...
of primary spontaneous pneumothorax (i.e. not due to surgical trauma etc.) in women (about 1/100'000/year), this is a very rare condition. Hence, many basic textbooks don't mention it, and many doctors
Physician
A physician is a health care provider who practices the profession of medicine, which is concerned with promoting, maintaining or restoring human health through the study, diagnosis, and treatment of disease, injury and other physical and mental impairments...
have never heard of it. However, catamenial pneumothorax is probably under-recognised.