Catamenial pneumothorax is recurrent spontaneous pneumothorax occurring in a fixed temporal relationship to menstruation (typically within 72 hours of onset), almost always right-sided, in women of reproductive age. It is the commonest manifestation of thoracic endometriosis syndrome. The leading pathogenic account is ectopic, hormone-responsive endometrial tissue on the diaphragm and visceral pleura that breaks down cyclically to create air leaks; two further theories — transdiaphragmatic passage of air from the peritoneal cavity through diaphragmatic fenestrations, and prostaglandin-F2-driven alveolar rupture during menses — are also proposed and are not mutually exclusive. Treatment combines hormonal ovarian suppression with surgery (diaphragmatic repair, pleural implant excision, pleurodesis).
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Conditions with similar clinical presentations that must be differentiated from Catamenial Pneumothorax:
name: Catamenial Pneumothorax
creation_date: "2026-10-03T00:00:00Z"
category: Complex
synonyms:
- menstruation-related pneumothorax
- thoracic endometriosis-related pneumothorax
description: >-
Catamenial pneumothorax is recurrent spontaneous pneumothorax occurring in a
fixed temporal relationship to menstruation (typically within 72 hours of
onset), almost always right-sided, in women of reproductive age. It is the
commonest manifestation of thoracic endometriosis syndrome. The leading
pathogenic account is ectopic, hormone-responsive endometrial tissue on the
diaphragm and visceral pleura that breaks down cyclically to create air leaks;
two further theories — transdiaphragmatic passage of air from the peritoneal
cavity through diaphragmatic fenestrations, and prostaglandin-F2-driven
alveolar rupture during menses — are also proposed and are not mutually
exclusive. Treatment combines hormonal ovarian suppression with surgery
(diaphragmatic repair, pleural implant excision, pleurodesis).
disease_term:
preferred_term: catamenial pneumothorax
term:
id: MONDO:0022098
label: catamenial pneumothorax
parents:
- pneumothorax
pathophysiology:
- name: Cyclical Ovarian Hormone Stimulation
biological_scale: ORGANISM
description: >-
Ectopic thoracic endometrial tissue expresses estrogen and progesterone
receptors and responds to the cyclical ovarian hormone environment, so that
the implants proliferate and break down in phase with the menstrual cycle.
That hormone dependence is the rationale for ovarian-suppression therapy.
chemical_entities:
- preferred_term: estradiol
term:
id: CHEBI:16469
label: 17beta-estradiol
- preferred_term: progesterone
term:
id: CHEBI:17026
label: progesterone
evidence:
- reference: PMID:39768909
reference_title: "Catamenial Pneumothorax-Still an Unveiled Disease."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
quote_role: REVIEW_SYNTHESIS
directness: INDIRECT
snippet: "Immunostaining for estrogen and progesterone receptors was postulated to support the diagnosis"
explanation: Estrogen/progesterone-receptor expression on the implants indicates their hormone responsiveness.
downstream:
- target: Thoracic Endometriosis
causal_link_type: DIRECT
- name: Abdominopelvic Endometriosis
biological_scale: TISSUE
description: >-
Co-existing pelvic endometriosis is present in the large majority of women
with thoracic endometriosis and is the reservoir from which endometrial
tissue is proposed to reach the thorax by retrograde menstruation and
transdiaphragmatic migration. It is the upstream origin of the thoracic
implants.
cell_types:
- preferred_term: endometrial glandular epithelial cell
term:
id: CL:0002656
label: glandular endometrial unciliated epithelial cell
- preferred_term: endometrial stromal cell
term:
id: CL:0002255
label: stromal cell of endometrium
evidence:
- reference: PMID:39768909
reference_title: "Catamenial Pneumothorax-Still an Unveiled Disease."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
quote_role: REVIEW_SYNTHESIS
directness: DIRECT
snippet: "Up to 80% of women with thoracic endometriosis have co-existing abdominopelvic endometriosis"
explanation: Most women with thoracic endometriosis have co-existing pelvic endometriosis, the proposed source of the thoracic implants.
downstream:
- target: Thoracic Endometriosis
causal_link_type: INDIRECT_KNOWN_INTERMEDIATES
- name: Thoracic Endometriosis
biological_scale: TISSUE
description: >-
Ectopic endometrial tissue (glandular epithelium and stroma) implants on the
diaphragm, visceral pleura and lung. Retrograde menstruation and
transdiaphragmatic migration of endometrial cells are the proposed routes by
which it reaches the thorax.
cell_types:
- preferred_term: endometrial glandular epithelial cell
term:
id: CL:0002656
label: glandular endometrial unciliated epithelial cell
- preferred_term: endometrial stromal cell
term:
id: CL:0002255
label: stromal cell of endometrium
evidence:
- reference: PMID:20473170
reference_title: "Catamenial pneumothorax."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
quote_role: REVIEW_SYNTHESIS
directness: DIRECT
snippet: "Pathology shows endometriosis in most instances."
explanation: Surgical pathology confirms endometriosis in most catamenial pneumothorax cases.
- reference: PMID:31401265
reference_title: "Diagnosis and Treatment of Catamenial Pneumothorax: A Systematic Review."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
quote_role: PRIMARY_RESULT
directness: DIRECT
snippet: "Diaphragmatic endometriosis and/or nodules were observed in 265 of 297 cases (89%)."
explanation: Diaphragmatic endometriosis/nodules are found in the large majority of assessed cases.
- reference: PMID:27516783
reference_title: "Catamenial pneumothorax - a review of the literature."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
quote_role: REVIEW_SYNTHESIS
directness: DIRECT
snippet: "Histopathological analysis of these nodules (spots) reveals glandular cells, endometrial stroma, and macrophages filled with hemosiderin"
explanation: Histology of the thoracic implants shows endometrial glandular epithelium and stroma.
downstream:
- target: Visceral Pleural and Diaphragmatic Defects
causal_link_type: DIRECT
- target: Hemothorax
causal_link_type: DIRECT
- target: Hemoptysis
causal_link_type: DIRECT
- name: Visceral Pleural and Diaphragmatic Defects
biological_scale: TISSUE
description: >-
Cyclical breakdown of pleural and diaphragmatic endometrial implants, and
diaphragmatic fenestrations, create the defects most frequently found at
thoracoscopy.
locations:
- preferred_term: diaphragm
term:
id: UBERON:0001103
label: diaphragm
- preferred_term: visceral pleura
term:
id: UBERON:0002401
label: visceral pleura
evidence:
- reference: PMID:20473170
reference_title: "Catamenial pneumothorax."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
quote_role: REVIEW_SYNTHESIS
directness: DIRECT
snippet: "At video-assisted thoracic surgery, diaphragmatic defects and nodules are the most frequent findings."
explanation: Diaphragmatic defects and nodules are the predominant operative findings.
- reference: PMID:20473170
reference_title: "Catamenial pneumothorax."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
quote_role: REVIEW_SYNTHESIS
directness: DIRECT
snippet: "Endometrial implants in visceral pleura are also found, although less frequently."
explanation: Visceral pleural endometrial implants are a less frequent but recognised source of defects.
downstream:
- target: Air Accumulation in the Pleural Space
causal_link_type: DIRECT
- target: Shoulder pain
causal_link_type: INDIRECT_KNOWN_INTERMEDIATES
- name: Transdiaphragmatic Air Passage
biological_scale: ORGANISM
description: >-
A proposed, non-mutually-exclusive route in which air passes from the
peritoneal cavity — which communicates with the genital tract during
menstruation when the cervical mucus plug is shed — through diaphragmatic
fenestrations into the pleural space.
evidence:
- reference: PMID:20473170
reference_title: "Catamenial pneumothorax."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
quote_role: REVIEW_SYNTHESIS
directness: DIRECT
snippet: "Findings of surgical explorations support the theory of transabdominal-transdiaphragmatic passage of air to explain the pathogenesis of catamenial pneumothorax."
explanation: Operative findings support transdiaphragmatic air passage as a pathogenic route.
- reference: PMID:39768909
reference_title: "Catamenial Pneumothorax-Still an Unveiled Disease."
supports: SUPPORT
evidence_source: OTHER
quote_role: REVIEW_SYNTHESIS
directness: DIRECT
snippet: "The passage of air from the uterus via the diaphragmatic fenestrations into the pleural cavity results in the development of pneumothorax."
explanation: States the transdiaphragmatic air-passage theory explicitly.
downstream:
- target: Air Accumulation in the Pleural Space
causal_link_type: DIRECT
hypothesis_groups:
- transdiaphragmatic_air_passage
- name: Menstrual Prostaglandin F2alpha Elevation
biological_scale: MOLECULAR
description: >-
First step of a proposed "physiological" theory: prostaglandin F2alpha rises
to high levels during menstruation. This branch does not require endometrial
implants and is offered as a complementary, non-settled account.
chemical_entities:
- preferred_term: prostaglandin F2alpha
modifier: INCREASED
term:
id: CHEBI:15553
label: prostaglandin F2alpha
evidence:
- reference: PMID:39768909
reference_title: "Catamenial Pneumothorax-Still an Unveiled Disease."
supports: SUPPORT
evidence_source: OTHER
quote_role: REVIEW_SYNTHESIS
directness: DIRECT
snippet: "high F2 prostaglandin levels during menstruation induce vasoconstriction and bronchospasm, resulting in alveolar rupture and pneumothorax"
explanation: States that elevated menstrual prostaglandin F2 initiates this proposed chain.
downstream:
- target: Bronchiolar and Vascular Smooth Muscle Contraction
causal_link_type: DIRECT
hypothesis_groups:
- prostaglandin_alveolar_rupture
- name: Bronchiolar and Vascular Smooth Muscle Contraction
biological_scale: CELLULAR
description: >-
Second step of the prostaglandin theory: elevated prostaglandin F2alpha
drives contraction of bronchiolar and vascular smooth muscle during menses.
biological_processes:
- preferred_term: smooth muscle contraction
modifier: INCREASED
term:
id: GO:0006939
label: smooth muscle contraction
evidence:
- reference: PMID:27516783
reference_title: "Catamenial pneumothorax - a review of the literature."
supports: SUPPORT
evidence_source: OTHER
quote_role: REVIEW_SYNTHESIS
directness: DIRECT
snippet: "High concentration of prostaglandin F2 during menses may cause blood vessels and bronchioles to contract, which leads to alveolar rupture and development of pneumothorax."
explanation: States that the elevated prostaglandin causes bronchiolar and vascular contraction in this chain.
downstream:
- target: Alveolar Rupture
causal_link_type: DIRECT
hypothesis_groups:
- prostaglandin_alveolar_rupture
- name: Alveolar Rupture
biological_scale: TISSUE
description: >-
Final step of the prostaglandin theory: the smooth-muscle contraction leads
to alveolar rupture, allowing air into the pleural space.
evidence:
- reference: PMID:27516783
reference_title: "Catamenial pneumothorax - a review of the literature."
supports: SUPPORT
evidence_source: OTHER
quote_role: REVIEW_SYNTHESIS
directness: DIRECT
snippet: "High concentration of prostaglandin F2 during menses may cause blood vessels and bronchioles to contract, which leads to alveolar rupture and development of pneumothorax."
explanation: The same review statement names alveolar rupture as the outcome of this chain.
downstream:
- target: Air Accumulation in the Pleural Space
causal_link_type: DIRECT
hypothesis_groups:
- prostaglandin_alveolar_rupture
- name: Air Accumulation in the Pleural Space
biological_scale: ORGANISM
description: >-
Air entering the pleural space through any of the proposed routes collapses
the lung, producing the pneumothorax and its symptoms.
evidence:
- reference: PMID:27516783
reference_title: "Catamenial pneumothorax - a review of the literature."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
quote_role: REVIEW_SYNTHESIS
directness: DIRECT
snippet: "recurrent accumulation of air in the pleural cavity in reproductive-age women without concomitant respiratory diseases"
explanation: Defines the entity as recurrent accumulation of air in the pleural cavity, which is this node.
downstream:
- target: Spontaneous pneumothorax
causal_link_type: DIRECT
- target: Chest pain
causal_link_type: DIRECT
- target: Dyspnea
causal_link_type: DIRECT
mechanistic_hypotheses:
- hypothesis_group_id: transdiaphragmatic_air_passage
hypothesis_label: Transdiaphragmatic passage of air
status: ALTERNATIVE
description: >-
Air reaches the pleural space from the peritoneal cavity through
diaphragmatic fenestrations during menstruation, rather than from
breakdown of pleural implants. Proposed and not mutually exclusive with the
endometriosis-implant account.
- hypothesis_group_id: prostaglandin_alveolar_rupture
hypothesis_label: Prostaglandin-F2-driven alveolar rupture
status: ALTERNATIVE
description: >-
A "physiological" account in which high menstrual prostaglandin F2alpha
drives bronchiolar and vascular smooth-muscle contraction and alveolar
rupture, requiring no endometrial implants. Proposed and complementary, not
settled.
phenotypes:
- name: Spontaneous pneumothorax
description: >-
Recurrent spontaneous pneumothorax, overwhelmingly right-sided (about 93% of
cases), occurring in a fixed temporal relationship to menstruation.
phenotype_term:
preferred_term: Catamenial (recurrent spontaneous) pneumothorax
term:
id: HP:0002108
label: Spontaneous pneumothorax
temporality: RECURRENT
evidence:
- reference: PMID:31401265
reference_title: "Diagnosis and Treatment of Catamenial Pneumothorax: A Systematic Review."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
quote_role: PRIMARY_RESULT
directness: DIRECT
snippet: "Pneumothorax was found mainly in the right lung (456 of 490 cases, 93%)."
explanation: Catamenial pneumothorax is overwhelmingly right-sided, a hallmark feature.
- reference: PMID:25337402
reference_title: "Catamenial pneumothorax."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
quote_role: REVIEW_SYNTHESIS
directness: DIRECT
snippet: "Usually onset of lung collapse is less than 72 hours after menstruation."
explanation: The pneumothorax is time-locked to menstruation, which defines the entity.
- name: Chest pain
phenotype_term:
preferred_term: Chest pain
term:
id: HP:0100749
label: Chest pain
evidence:
- reference: PMID:31401265
reference_title: "Diagnosis and Treatment of Catamenial Pneumothorax: A Systematic Review."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
quote_role: REVIEW_SYNTHESIS
directness: DIRECT
snippet: "Diagnosis was mostly made clinically, with the patients complaining of the typical symptoms of shortness of breath and recurrent chest pain or shoulder pain a day before to 72 hours after menses."
explanation: Recurrent chest pain perimenstrually is a typical presenting symptom.
- name: Shoulder pain
description: >-
Ipsilateral shoulder (scapular) pain, referred from diaphragmatic irritation,
is a recognised perimenstrual presenting symptom of this disease.
phenotype_term:
preferred_term: Shoulder pain
term:
id: HP:0030834
label: Shoulder pain
evidence:
- reference: PMID:31401265
reference_title: "Diagnosis and Treatment of Catamenial Pneumothorax: A Systematic Review."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
quote_role: REVIEW_SYNTHESIS
directness: DIRECT
snippet: "Diagnosis was mostly made clinically, with the patients complaining of the typical symptoms of shortness of breath and recurrent chest pain or shoulder pain a day before to 72 hours after menses."
explanation: Shoulder pain is listed among the typical perimenstrual presenting symptoms.
- name: Dyspnea
phenotype_term:
preferred_term: Dyspnea
term:
id: HP:0002094
label: Dyspnea
evidence:
- reference: PMID:31401265
reference_title: "Diagnosis and Treatment of Catamenial Pneumothorax: A Systematic Review."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
quote_role: REVIEW_SYNTHESIS
directness: DIRECT
snippet: "Diagnosis was mostly made clinically, with the patients complaining of the typical symptoms of shortness of breath and recurrent chest pain or shoulder pain a day before to 72 hours after menses."
explanation: Shortness of breath is a typical presenting symptom.
- name: Hemothorax
description: >-
Catamenial hemothorax is a separate presentation within the thoracic
endometriosis syndrome spectrum, not a feature of the pneumothorax itself;
reported frequencies are shares of all thoracic endometriosis cases rather
than of catamenial pneumothorax patients.
phenotype_term:
preferred_term: Catamenial hemothorax
term:
id: HP:0012151
label: Hemothorax
evidence:
- reference: PMID:25337402
reference_title: "Catamenial pneumothorax."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
quote_role: REVIEW_SYNTHESIS
directness: DIRECT
snippet: "Catamenial pneumothorax (CP) is the most common form of thoracic endometriosis syndrome, which also includes catamenial hemothorax, catamenial hemoptysis, catamenial hemopneumothorax and endometriosis lung nodules"
explanation: Catamenial hemothorax is part of the thoracic endometriosis syndrome spectrum.
- name: Hemoptysis
description: >-
Catamenial hemoptysis is a separate presentation within the thoracic
endometriosis syndrome spectrum, not a feature of the pneumothorax itself;
reported frequencies are shares of all thoracic endometriosis cases rather
than of catamenial pneumothorax patients.
phenotype_term:
preferred_term: Catamenial hemoptysis
term:
id: HP:0002105
label: Hemoptysis
evidence:
- reference: PMID:25337402
reference_title: "Catamenial pneumothorax."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
quote_role: REVIEW_SYNTHESIS
directness: DIRECT
snippet: "Catamenial pneumothorax (CP) is the most common form of thoracic endometriosis syndrome, which also includes catamenial hemothorax, catamenial hemoptysis, catamenial hemopneumothorax and endometriosis lung nodules"
explanation: Catamenial hemoptysis is part of the thoracic endometriosis syndrome spectrum.
biochemical:
- name: Serum CA-125
presence: INCREASED
notes: >-
CA-125 can be elevated in thoracic endometriosis and has been suggested as a
supportive marker and a guide to recurrence risk; it is not specific or
diagnostic on its own.
evidence:
- reference: PMID:39768909
reference_title: "Catamenial Pneumothorax-Still an Unveiled Disease."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
quote_role: REVIEW_SYNTHESIS
directness: DIRECT
snippet: "An elevated level of CA-125 may accompany the disease."
explanation: Serum CA-125 may be elevated in catamenial pneumothorax.
diagnosis:
- name: Diagnostic videothoracoscopy with histopathologic confirmation
description: >-
Video-assisted thoracoscopy timed to menstruation visualises the ectopic
diaphragmatic and pleural lesions; definitive confirmation is histopathologic
examination of tissue taken at VATS or thoracotomy.
diagnosis_term:
preferred_term: diagnostic videothoracoscopy
term:
id: NCIT:C64949
label: Diagnostic Videothoracoscopy
evidence:
- reference: PMID:39768909
reference_title: "Catamenial Pneumothorax-Still an Unveiled Disease."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
quote_role: REVIEW_SYNTHESIS
directness: DIRECT
snippet: "video-assisted thoracoscopy (VAT) exploration of the thoracic cavity around the menstruation time allows for optimal visualization of ectopic lesions"
explanation: Thoracoscopy timed to menstruation gives optimal visualisation of the ectopic lesions.
- reference: PMID:39768909
reference_title: "Catamenial Pneumothorax-Still an Unveiled Disease."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
quote_role: REVIEW_SYNTHESIS
directness: DIRECT
snippet: "The final confirmation is established by histopathological examination of the tissue material acquired via VATS or open thoracotomy."
explanation: Histopathologic examination of surgically acquired tissue is the confirmatory step.
- name: Contrast-enhanced computed tomography
description: >-
Chest and abdomen CT, preferably contrast-enhanced and performed during
menstruation, can show pneumoperitoneum and diaphragmatic lesions.
diagnosis_term:
preferred_term: computed tomography
term:
id: NCIT:C17204
label: Computed Tomography
evidence:
- reference: PMID:39768909
reference_title: "Catamenial Pneumothorax-Still an Unveiled Disease."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
quote_role: REVIEW_SYNTHESIS
directness: DIRECT
snippet: "Chest and abdomen computed tomography (CT), particularly contrast-enhanced, may additionally show pneumoperitoneum and diaphragmatic lesions"
explanation: Contrast-enhanced CT can demonstrate pneumoperitoneum and diaphragmatic lesions.
- reference: PMID:39768909
reference_title: "Catamenial Pneumothorax-Still an Unveiled Disease."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
quote_role: REVIEW_SYNTHESIS
directness: DIRECT
snippet: "The CT should preferably be performed during menstruation"
explanation: Imaging yield depends on timing the CT to menstruation.
- name: Magnetic resonance imaging
description: >-
MRI has higher specificity than radiography or CT for detecting
endometriosis-related haemorrhage; it is likewise best timed to menstruation.
diagnosis_term:
preferred_term: magnetic resonance imaging
term:
id: NCIT:C16809
label: Magnetic Resonance Imaging
evidence:
- reference: PMID:39768909
reference_title: "Catamenial Pneumothorax-Still an Unveiled Disease."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
quote_role: REVIEW_SYNTHESIS
directness: DIRECT
snippet: "Although commonly used, both RTG and CT have poorer specificity in CP than magnetic resonance imaging (MRI) in detecting endometriosis-related hemorrhage"
explanation: MRI outperforms radiography and CT in specificity for endometriosis-related haemorrhage.
prevalence:
- population: Women of reproductive age presenting with spontaneous pneumothorax
measure_type: UNKNOWN
prevalence_class: NOT_YET_DOCUMENTED
notes: >-
No population rate is defensible; occurrence is reported as a proportion of
spontaneous pneumothorax in reproductive-age women — 7.3% to 36.7% across
series, and about one third of cases referred for surgery.
evidence:
- reference: PMID:31401265
reference_title: "Diagnosis and Treatment of Catamenial Pneumothorax: A Systematic Review."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
quote_role: REVIEW_SYNTHESIS
directness: DIRECT
snippet: "The prevalence of catamenial pneumothorax of all cases of pneumothorax in women of reproductive age ranges from 7.3% to 36.7%."
explanation: Reports the proportion of female reproductive-age pneumothorax that is catamenial.
progression:
- phase: Perimenstrual recurrent episodes
age_range: Reproductive age, most commonly 30-40 years
notes: >-
Episodes recur in a fixed relationship to menstruation, typically within 72
hours of onset, and recurrence is common without treatment of the underlying
mechanism.
evidence:
- reference: PMID:25337402
reference_title: "Catamenial pneumothorax."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
quote_role: REVIEW_SYNTHESIS
directness: DIRECT
snippet: "Usually onset of lung collapse is less than 72 hours after menstruation."
explanation: Establishes the perimenstrual timing that recurs across cycles.
- reference: PMID:31401265
reference_title: "Diagnosis and Treatment of Catamenial Pneumothorax: A Systematic Review."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
quote_role: REVIEW_SYNTHESIS
directness: DIRECT
snippet: "Recurrence rate varied from 14.3% to 55%."
explanation: Quantifies the high recurrence that characterises the course.
treatments:
- name: GnRH agonist therapy
description: >-
Gonadotropin-releasing hormone agonists induce ovarian rest; they are the
hormonal agent most associated with reduced recurrence, often used around
surgery.
therapeutic_modality: PEPTIDE
treatment_term:
preferred_term: Pharmacotherapy
term:
id: NCIT:C15986
label: Pharmacotherapy
therapeutic_agent:
- preferred_term: leuprolide (GnRH agonist)
term:
id: CHEBI:6427
label: leuprolide
target_mechanisms:
- target: Cyclical Ovarian Hormone Stimulation
evidence:
- reference: PMID:39768909
reference_title: "Catamenial Pneumothorax-Still an Unveiled Disease."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
quote_role: REVIEW_SYNTHESIS
directness: DIRECT
snippet: "The use of GnRH analogs has been correlated with a lower incidence of CP recurrence than surgery alone or postoperative estrogen-progesterone therapy."
explanation: GnRH agonists are associated with lower recurrence than surgery or estrogen-progesterone therapy alone.
- name: Hormonal ovarian suppression
description: >-
Danazol, continuous oral contraceptives and progestins suppress ovarian
estrogen production to induce hypoestrogenism and atrophy of the ectopic
endometrium.
therapeutic_modality: SMALL_MOLECULE
treatment_term:
preferred_term: Pharmacotherapy
term:
id: NCIT:C15986
label: Pharmacotherapy
therapeutic_agent:
- preferred_term: danazol
term:
id: CHEBI:4315
label: danazol
target_mechanisms:
- target: Cyclical Ovarian Hormone Stimulation
evidence:
- reference: PMID:39768909
reference_title: "Catamenial Pneumothorax-Still an Unveiled Disease."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
quote_role: REVIEW_SYNTHESIS
directness: DIRECT
snippet: "Pharmacotherapy aims to suppress ovarian estrogen production and atrophy of functional endometrium (including ectopic endometrium within the chest cavity), resulting in a lack of menstruation."
explanation: States the mechanism and aim of hormonal ovarian suppression.
- reference: PMID:27516783
reference_title: "Catamenial pneumothorax - a review of the literature."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
quote_role: REVIEW_SYNTHESIS
directness: DIRECT
snippet: "The primary aim of treatment is to induce hypoestrogenism, resulting in the lack of menses."
explanation: Confirms hypoestrogenism/ovarian rest as the aim of medical therapy.
- name: Thoracoscopic surgery
description: >-
Video-assisted thoracoscopic surgery allows pathologic confirmation and
treatment of the mechanism — partial diaphragmatic resection and excision of
visceral pleural implants.
therapeutic_modality: SURGERY
treatment_term:
preferred_term: thoracoscopic surgery
term:
id: NCIT:C15726
label: Thoracoscopic Surgery
target_mechanisms:
- target: Visceral Pleural and Diaphragmatic Defects
evidence:
- reference: PMID:20473170
reference_title: "Catamenial pneumothorax."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
quote_role: REVIEW_SYNTHESIS
directness: DIRECT
snippet: "Partial diaphragmatic resection and/or exeresis of visceral pleural implants, as well as talc pleurodesis, are nowadays frequently carried out."
explanation: Diaphragmatic resection and visceral pleural implant excision are standard operative treatments.
- name: Pleurodesis
description: >-
Talc or mechanical pleurodesis obliterates the pleural space to prevent
recurrent air accumulation.
therapeutic_modality: SURGERY
treatment_term:
preferred_term: pleurodesis
term:
id: NCIT:C66899
label: Pleurodesis
target_mechanisms:
- target: Air Accumulation in the Pleural Space
evidence:
- reference: PMID:20473170
reference_title: "Catamenial pneumothorax."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
quote_role: REVIEW_SYNTHESIS
directness: DIRECT
snippet: "Partial diaphragmatic resection and/or exeresis of visceral pleural implants, as well as talc pleurodesis, are nowadays frequently carried out."
explanation: Talc pleurodesis is a frequently used procedure to prevent recurrence.
- name: Chest tube drainage (acute management)
description: >-
Acute supportive management of the pneumothorax by tube thoracostomy to
re-expand the collapsed lung, distinct from the causal therapies that prevent
recurrence.
therapeutic_modality: SURGERY
treatment_term:
preferred_term: tube thoracostomy
term:
id: NCIT:C148463
label: Tube Thoracostomy
target_mechanisms:
- target: Air Accumulation in the Pleural Space
evidence:
- reference: PMID:39768909
reference_title: "Catamenial Pneumothorax-Still an Unveiled Disease."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
quote_role: REVIEW_SYNTHESIS
directness: DIRECT
snippet: "in some cases, a chest tube insertion may be necessary to allow for re-expansion of the lung tissue"
explanation: Chest-tube insertion is used acutely to re-expand the lung in the pneumothorax.
differential_diagnoses:
- name: Primary spontaneous pneumothorax
description: >-
Spontaneous pneumothorax in the absence of clinically apparent lung disease,
classically in tall, thin young people and strongly associated with smoking.
It shares the cardinal presentation of recurrent spontaneous pneumothorax but
is not locked to the perimenstrual window and lacks the female, right-sided,
reproductive-age predominance of the catamenial form.
distinguishing_features:
- Episodes are not timed to menstruation, whereas catamenial pneumothorax
recurs within roughly 72 hours of menstrual onset.
- Strongly associated with tobacco smoking and apical subpleural blebs rather
than diaphragmatic fenestrations or pleural endometriotic implants.
- Occurs across both sexes with a male predominance and no reproductive-age or
right-sided skew.
evidence:
- reference: PMID:34774177
reference_title: "Pneumothorax: Classification and Etiology."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
quote_role: REVIEW_SYNTHESIS
directness: DIRECT
snippet: "Tobacco smoking is a major risk factor for primary spontaneous pneumothorax"
explanation: >-
Identifies the smoking association that distinguishes primary spontaneous
pneumothorax from the hormonally driven catamenial form.
- name: Lymphangioleiomyomatosis
disease_term:
preferred_term: Lymphangioleiomyomatosis
term:
id: MONDO:0011705
label: lymphangioleiomyomatosis
description: >-
A rare multisystem disease of women of reproductive age in which progressive
diffuse cystic destruction of the lung parenchyma causes recurrent
pneumothorax. Like catamenial pneumothorax it almost exclusively affects
premenopausal women and is hormone-responsive, but its pneumothoraces arise
from diffuse cystic lung disease rather than from thoracic endometriosis or
diaphragmatic defects.
distinguishing_features:
- Pneumothorax arises from diffuse bilateral cystic lung destruction visible on
high-resolution CT, not from focal diaphragmatic or visceral pleural defects.
- Associated with tuberous sclerosis complex, renal angiomyolipomas, and
chylous effusions.
- Progressive decline in lung function, unlike the self-limited perimenstrual
episodes of catamenial pneumothorax.
evidence:
- reference: PMID:27514586
reference_title: "Lymphangioleiomyomatosis."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
quote_role: REVIEW_SYNTHESIS
directness: DIRECT
snippet: "Lung cysts progressively replace the lung parenchyma, which leads to dyspnea, recurrent pneumothorax, and in some cases respiratory failure"
explanation: >-
Establishes that recurrent pneumothorax in LAM stems from progressive
diffuse cystic lung disease, the feature that separates it from catamenial
pneumothorax.
- name: Birt-Hogg-Dube syndrome
disease_term:
preferred_term: Birt-Hogg-Dube syndrome
term:
id: MONDO:0800444
label: Birt-Hogg-Dube syndrome
description: >-
An autosomal dominant disorder caused by FLCN mutations presenting with
recurrent spontaneous pneumothorax from basally distributed pulmonary cysts,
alongside fibrofolliculomas and renal tumors. It overlaps catamenial
pneumothorax only in the recurrent-pneumothorax presentation and is
distinguished by its hereditary, non-cyclical, multisystem phenotype.
distinguishing_features:
- Autosomal dominant inheritance due to Folliculin (FLCN) mutations, with a
positive family history, rather than a sporadic hormone-dependent disorder.
- Pneumothoraces arise from basally predominant pulmonary cysts seen in the
large majority of adult patients on chest CT.
- Accompanied by cutaneous fibrofolliculomas and early-onset renal cancers that
have no counterpart in catamenial pneumothorax.
evidence:
- reference: PMID:27514594
reference_title: "Birt-Hogg-Dubé Syndrome."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
quote_role: REVIEW_SYNTHESIS
directness: DIRECT
snippet: "characterized by the formation of fibrofolliculomas, early onset renal cancers, pulmonary cysts, and spontaneous pneumothoraces"
explanation: >-
Lists the multisystem hereditary features that differentiate Birt-Hogg-Dube
syndrome from catamenial pneumothorax.
- reference: PMID:27514594
reference_title: "Birt-Hogg-Dubé Syndrome."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
quote_role: REVIEW_SYNTHESIS
directness: DIRECT
snippet: "More than 80% of adult patients with BHD have pulmonary cysts"
explanation: >-
Documents the basal pulmonary cysts underlying pneumothorax in BHD, a
radiologic feature absent in catamenial pneumothorax.
datasets:
Deep research results are used as seeds for research; they do not undergo the same validation as the main records and may contain errors. How we use deep research.
Create: Catamenial Pneumothorax · 2026-10-03T18:49:17Z · View source
Created Catamenial Pneumothorax (MONDO:0022098), an acquired/complex disorder and the commonest manifestation of thoracic endometriosis syndrome. Pathophysiology modeled as the leading account plus two explicitly-proposed competing theories (the reviews frame all three as theories, preserved here): Cyclical Ovarian Hormone Stimulation -> Thoracic Endometriosis (diaphragm/visceral pleura, endometrial glandular+stromal cells CL:0002656/CL:0002255) -> Visceral Pleural and Diaphragmatic Defects -> Air Accumulation in the Pleural Space -> pneumothorax/pleuritic chest pain/dyspnea; plus Transdiaphragmatic Air Passage and Prostaglandin-Mediated Alveolar Rupture (CHEBI:15553 PGF2alpha, GO:0006939) as parallel proposed routes into air accumulation; thoracic endometriosis also wired to catamenial hemothorax and hemoptysis. 5/5 phenotypes causally connected. biochemical: serum CA-125 (INCREASED). prevalence recorded honestly as a proportion of reproductive-age female spontaneous pneumothorax (measure_type UNKNOWN / NOT_YET_DOCUMENTED, 7.3-36.7% in notes). progression: perimenstrual recurrent episodes (<72h of menses, recurrence 14.3-55%). Treatments: GnRH agonist (leuprolide CHEBI:6427, PEPTIDE), hormonal ovarian suppression (danazol CHEBI:4315, SMALL_MOLECULE), thoracoscopic surgery (NCIT:C15726), pleurodesis (NCIT:C66899), all target_mechanisms-linked. Deep research requested falcon, fell back to claude_code (no EDISON_API_KEY), thin, used as a lead only. Evidence anchored on five PubMed reviews/systematic reviews: PMID:31401265, PMID:25337402, PMID:20473170, PMID:39768909, PMID:27516783; 25/25 snippets exact-quote verified. No GeneReviews chapter (acquired/complex). validate-disorders, validate-terms, entity-refs, causal-targets, duplicate-keys, qualifier-terms, coarse-phenotypes pass. Differentials omitted: no precise MONDO term for non-catamenial primary spontaneous pneumothorax to anchor one without a loose parent binding.
Verification status. Only the PMIDs and URLs listed under "Sources actually retrieved" came from searches in this session, and I did not open the full texts or check abstract quotes. Everything else is from background knowledge of the literature and is marked (unverified). Do not cite those items in the KB until they have been fetched with just fetch-reference and quoted exactly. I did not verify any ontology CURIEs. HP, GO, CL, UBERON and NCIT lookups are still needed, and the MONDO ID in the request (MONDO:0022098) is unconfirmed.
Catamenial pneumothorax (CP) is a recurrent spontaneous pneumothorax that occurs within about 72 hours before or after the onset of menstruation. It mostly affects women of reproductive age. Most cases are attributed to thoracic endometriosis syndrome (TES). A recent review describes it as "an uncommon condition, usually associated with thoracic endometriosis syndrome (TES)" and notes that international guidelines are still lacking (PMC11728258).
DISEASE entry, as a subtype of thoracic endometriosis or endometriosis, or as a GROUPING member. Record the decision in the stub notes.Frequencies below are approximate and from memory (unverified). They need to be traced to primary series, such as the Joseph & Sahn and Visouli et al. reviews.
| Phenotype | Notes | Candidate HPO term (look up) |
|---|---|---|
| Recurrent spontaneous pneumothorax | Cyclical, within 72 h of menses. Right-sided in about 85–90%. | Recurrent pneumothorax / Pneumothorax |
| Chest or shoulder pain | Common presenting symptom | Chest pain |
| Dyspnea | Common | Dyspnea |
| Cough | Occasional | Cough |
| Catamenial hemothorax | Part of TES | Hemothorax |
| Catamenial hemoptysis | Part of TES | Hemoptysis |
| Pelvic endometriosis | Often coexisting | Endometriosis |
| Pelvic pain, dysmenorrhea | Often coexisting | Dysmenorrhea |
No causal genes or pathogenic variants are established. I found no evidence for a Mendelian cause, so do not create genetic: causal rows without a source. Endometriosis itself has polygenic susceptibility (GWAS loci), but I did not verify any specific locus for thoracic disease. There is no chromosomal or epigenetic data in what I retrieved.
There are no established environmental, lifestyle or infectious factors. The relevant trigger is endogenous: the menstrual cycle.
Proposed causal chains (the search results name four theories: physiological, migrational, microembolic-metastatic, and the diaphragmatic theory of air passage):
Suggested ontology terms (all need lookup before use):
No molecular profiling, single-cell or CRISPR data were found.
NCIT:C15986, with therapeutic_agent for leuprolide or other agents. Look up each agent in CHEBI/NCIT.NCIT:C15329. A thoracoscopic-surgery term may exist and needs checking.No naturally occurring animal disease was identified. This section is not applicable on the evidence I retrieved. I did not query OMIA.
I found no established animal model of catamenial pneumothorax. Endometriosis models exist in mouse and primate, but I did not verify any that reproduce thoracic disease. Treat this section as a gap, and consider a KNOWLEDGE_GAP discussion rather than an animal_models: entry.
just fetch-reference PMID:<id> and quote only exact substrings.just check-causal-targets and wire the phenotypes. Unconnected phenotypes would be a likely weakness in this entry.Checked with linkml-reference-validator 0.3.0rc3.
| Outcome | Count |
|---|---|
| References checked | 8 |
| Resolved | 3 |
| Unresolved (possible confabulation) | 0 |
| Unverifiable | 5 |
| References weighed for topical relevance | 3 |
| On topic | 3 |
| Off topic | 0 |
3 of 8 references resolved; the rest could not be looked up either way.
Checked with linkml-term-validator 0.4.5, through the ols: adapter.
| Outcome | Count |
|---|---|
| Terms checked | 3 |
| Resolved | 3 |
| Unresolved (possible confabulation) | 0 |
| Obsolete | 0 |
| Unverifiable | 0 |
Every term resolved, and every label the report gave matched.