Catamenial Pneumothorax

Complex MONDO:0022098 Pathograph 20 Show in embeddings browser pneumothorax

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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9
Pathophys.
6
Phenotypes
2
Hypotheses
20
Pathograph
5
Medical Actions
3
Differentials
1
Deep Research
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Mechanistic Hypotheses

2
Transdiaphragmatic passage of air
transdiaphragmatic_air_passage ALTERNATIVE
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.
Prostaglandin-F2-driven alveolar rupture
prostaglandin_alveolar_rupture ALTERNATIVE
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.
⚙

Pathophysiology

9
Cyclical Ovarian Hormone Stimulation
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.
Show evidence (1 reference)
PMID:39768909 SUPPORT INDIRECT REVIEW SYNTHESIS Human Clinical
"Immunostaining for estrogen and progesterone receptors was postulated to support the diagnosis"
Estrogen/progesterone-receptor expression on the implants indicates their hormone responsiveness.
Abdominopelvic Endometriosis
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.
endometrial glandular epithelial cell CL:0002656 Cell Ontology (CL) Relation: this pathophysiological event involves this cell type This pathophysiological event involves endometrial glandular epithelial cell, annotated with glandular endometrial unciliated epithelial cell (CL:0002656). CL:0002656 is a cell type from the Cell Ontology. endometrial stromal cell CL:0002255 Cell Ontology (CL) Relation: this pathophysiological event involves this cell type This pathophysiological event involves endometrial stromal cell, annotated with stromal cell of endometrium (CL:0002255). CL:0002255 is a cell type from the Cell Ontology.
Show evidence (1 reference)
PMID:39768909 SUPPORT DIRECT REVIEW SYNTHESIS Human Clinical
"Up to 80% of women with thoracic endometriosis have co-existing abdominopelvic endometriosis"
Most women with thoracic endometriosis have co-existing pelvic endometriosis, the proposed source of the thoracic implants.
Thoracic Endometriosis
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.
endometrial glandular epithelial cell CL:0002656 Cell Ontology (CL) Relation: this pathophysiological event involves this cell type This pathophysiological event involves endometrial glandular epithelial cell, annotated with glandular endometrial unciliated epithelial cell (CL:0002656). CL:0002656 is a cell type from the Cell Ontology. endometrial stromal cell CL:0002255 Cell Ontology (CL) Relation: this pathophysiological event involves this cell type This pathophysiological event involves endometrial stromal cell, annotated with stromal cell of endometrium (CL:0002255). CL:0002255 is a cell type from the Cell Ontology.
Show evidence (3 references)
PMID:20473170 SUPPORT DIRECT REVIEW SYNTHESIS Human Clinical
"Pathology shows endometriosis in most instances."
Surgical pathology confirms endometriosis in most catamenial pneumothorax cases.
PMID:31401265 SUPPORT DIRECT PRIMARY RESULT Human Clinical
"Diaphragmatic endometriosis and/or nodules were observed in 265 of 297 cases (89%)."
Diaphragmatic endometriosis/nodules are found in the large majority of assessed cases.
PMID:27516783 SUPPORT DIRECT REVIEW SYNTHESIS Human Clinical
"Histopathological analysis of these nodules (spots) reveals glandular cells, endometrial stroma, and macrophages filled with hemosiderin"
Histology of the thoracic implants shows endometrial glandular epithelium and stroma.
Visceral Pleural and Diaphragmatic Defects
Cyclical breakdown of pleural and diaphragmatic endometrial implants, and diaphragmatic fenestrations, create the defects most frequently found at thoracoscopy.
diaphragm UBERON:0001103 Uberon multi-species anatomy ontology (UBERON) Relation: this pathophysiological event occurs in this anatomical location This pathophysiological event occurs in diaphragm (UBERON:0001103). UBERON:0001103 is an anatomical location from the Uberon multi-species anatomy ontology. visceral pleura UBERON:0002401 Uberon multi-species anatomy ontology (UBERON) Relation: this pathophysiological event occurs in this anatomical location This pathophysiological event occurs in visceral pleura (UBERON:0002401). UBERON:0002401 is an anatomical location from the Uberon multi-species anatomy ontology.
Show evidence (2 references)
PMID:20473170 SUPPORT DIRECT REVIEW SYNTHESIS Human Clinical
"At video-assisted thoracic surgery, diaphragmatic defects and nodules are the most frequent findings."
Diaphragmatic defects and nodules are the predominant operative findings.
PMID:20473170 SUPPORT DIRECT REVIEW SYNTHESIS Human Clinical
"Endometrial implants in visceral pleura are also found, although less frequently."
Visceral pleural endometrial implants are a less frequent but recognised source of defects.
Transdiaphragmatic Air Passage
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.
Show evidence (2 references)
PMID:20473170 SUPPORT DIRECT REVIEW SYNTHESIS Human Clinical
"Findings of surgical explorations support the theory of transabdominal-transdiaphragmatic passage of air to explain the pathogenesis of catamenial pneumothorax."
Operative findings support transdiaphragmatic air passage as a pathogenic route.
PMID:39768909 SUPPORT DIRECT REVIEW SYNTHESIS Other
"The passage of air from the uterus via the diaphragmatic fenestrations into the pleural cavity results in the development of pneumothorax."
States the transdiaphragmatic air-passage theory explicitly.
Menstrual Prostaglandin F2alpha Elevation
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.
Show evidence (1 reference)
PMID:39768909 SUPPORT DIRECT REVIEW SYNTHESIS Other
"high F2 prostaglandin levels during menstruation induce vasoconstriction and bronchospasm, resulting in alveolar rupture and pneumothorax"
States that elevated menstrual prostaglandin F2 initiates this proposed chain.
Bronchiolar and Vascular Smooth Muscle Contraction
Second step of the prostaglandin theory: elevated prostaglandin F2alpha drives contraction of bronchiolar and vascular smooth muscle during menses.
smooth muscle contraction GO:0006939 Gene Ontology (GO) Relation: this pathophysiological event involves this biological process This pathophysiological event involves increased smooth muscle contraction (GO:0006939). GO:0006939 is a biological process from the Gene Ontology. ↑ INCREASED
Show evidence (1 reference)
PMID:27516783 SUPPORT DIRECT REVIEW SYNTHESIS Other
"High concentration of prostaglandin F2 during menses may cause blood vessels and bronchioles to contract, which leads to alveolar rupture and development of pneumothorax."
States that the elevated prostaglandin causes bronchiolar and vascular contraction in this chain.
Alveolar Rupture
Final step of the prostaglandin theory: the smooth-muscle contraction leads to alveolar rupture, allowing air into the pleural space.
Show evidence (1 reference)
PMID:27516783 SUPPORT DIRECT REVIEW SYNTHESIS Other
"High concentration of prostaglandin F2 during menses may cause blood vessels and bronchioles to contract, which leads to alveolar rupture and development of pneumothorax."
The same review statement names alveolar rupture as the outcome of this chain.
Air Accumulation in the Pleural Space
Air entering the pleural space through any of the proposed routes collapses the lung, producing the pneumothorax and its symptoms.
Show evidence (1 reference)
PMID:27516783 SUPPORT DIRECT REVIEW SYNTHESIS Human Clinical
"recurrent accumulation of air in the pleural cavity in reproductive-age women without concomitant respiratory diseases"
Defines the entity as recurrent accumulation of air in the pleural cavity, which is this node.
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Pathograph

Use the checkboxes to hide or show graph categories. Hover nodes for evidence and cross-linked metadata.
Pathograph: causal mechanism network for Catamenial Pneumothorax Interactive directed graph showing how pathophysiology mechanisms, phenotypes, genetic factors and variants, experimental models, environmental triggers, and treatments relate through causal and linked edges.
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Phenotypes

6
Respiratory 4
Spontaneous pneumothorax HP:0002108 Human Phenotype Ontology (HP) Relation: this clinical feature is this phenotype This clinical feature is Catamenial (recurrent spontaneous) pneumothorax, annotated with Spontaneous pneumothorax (HP:0002108), qualified as temporality recurrent. HP:0002108 is a phenotype from the Human Phenotype Ontology.
Temporal: RECURRENT
Show evidence (2 references)
PMID:31401265 SUPPORT DIRECT PRIMARY RESULT Human Clinical
"Pneumothorax was found mainly in the right lung (456 of 490 cases, 93%)."
Catamenial pneumothorax is overwhelmingly right-sided, a hallmark feature.
PMID:25337402 SUPPORT DIRECT REVIEW SYNTHESIS Human Clinical
"Usually onset of lung collapse is less than 72 hours after menstruation."
The pneumothorax is time-locked to menstruation, which defines the entity.
Dyspnea HP:0002094 Human Phenotype Ontology (HP) Relation: this clinical feature is this phenotype This clinical feature is Dyspnea (HP:0002094). HP:0002094 is a phenotype from the Human Phenotype Ontology.
Show evidence (1 reference)
PMID:31401265 SUPPORT DIRECT REVIEW SYNTHESIS Human Clinical
"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."
Shortness of breath is a typical presenting symptom.
Hemothorax HP:0012151 Human Phenotype Ontology (HP) Relation: this clinical feature is this phenotype This clinical feature is Catamenial hemothorax, annotated with Hemothorax (HP:0012151). HP:0012151 is a phenotype from the Human Phenotype Ontology.
Show evidence (1 reference)
PMID:25337402 SUPPORT DIRECT REVIEW SYNTHESIS Human Clinical
"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"
Catamenial hemothorax is part of the thoracic endometriosis syndrome spectrum.
Hemoptysis HP:0002105 Human Phenotype Ontology (HP) Relation: this clinical feature is this phenotype This clinical feature is Catamenial hemoptysis, annotated with Hemoptysis (HP:0002105). HP:0002105 is a phenotype from the Human Phenotype Ontology.
Show evidence (1 reference)
PMID:25337402 SUPPORT DIRECT REVIEW SYNTHESIS Human Clinical
"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"
Catamenial hemoptysis is part of the thoracic endometriosis syndrome spectrum.
Constitutional 2
Chest pain HP:0100749 Human Phenotype Ontology (HP) Relation: this clinical feature is this phenotype This clinical feature is Chest pain (HP:0100749). HP:0100749 is a phenotype from the Human Phenotype Ontology.
Show evidence (1 reference)
PMID:31401265 SUPPORT DIRECT REVIEW SYNTHESIS Human Clinical
"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."
Recurrent chest pain perimenstrually is a typical presenting symptom.
Shoulder pain HP:0030834 Human Phenotype Ontology (HP) Relation: this clinical feature is this phenotype This clinical feature is Shoulder pain (HP:0030834). HP:0030834 is a phenotype from the Human Phenotype Ontology.
Show evidence (1 reference)
PMID:31401265 SUPPORT DIRECT REVIEW SYNTHESIS Human Clinical
"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."
Shoulder pain is listed among the typical perimenstrual presenting symptoms.
💊

Medical Actions

5
GnRH agonist therapy
Action: PharmacotherapyNCI Thesaurus (NCIT) Relation: this treatment is this clinical intervention This treatment is Pharmacotherapy (NCIT:C15986). NCIT:C15986 is a clinical intervention from the NCI Thesaurus. NCIT:C15986
Agent: leuprolide (GnRH agonist) CHEBI:6427 Chemical Entities of Biological Interest (CHEBI) Relation: this treatment uses this therapeutic agent This treatment uses leuprolide (GnRH agonist), annotated with leuprolide (CHEBI:6427). CHEBI:6427 is a therapeutic agent from Chemical Entities of Biological Interest.
Platform: Peptide
Gonadotropin-releasing hormone agonists induce ovarian rest; they are the hormonal agent most associated with reduced recurrence, often used around surgery.
Mechanism Target:
Cyclical Ovarian Hormone Stimulation
Show evidence (1 reference)
PMID:39768909 SUPPORT DIRECT REVIEW SYNTHESIS Human Clinical
"The use of GnRH analogs has been correlated with a lower incidence of CP recurrence than surgery alone or postoperative estrogen-progesterone therapy."
GnRH agonists are associated with lower recurrence than surgery or estrogen-progesterone therapy alone.
Hormonal ovarian suppression
Action: PharmacotherapyNCI Thesaurus (NCIT) Relation: this treatment is this clinical intervention This treatment is Pharmacotherapy (NCIT:C15986). NCIT:C15986 is a clinical intervention from the NCI Thesaurus. NCIT:C15986
Agent: danazol CHEBI:4315 Chemical Entities of Biological Interest (CHEBI) Relation: this treatment uses this therapeutic agent This treatment uses danazol (CHEBI:4315). CHEBI:4315 is a therapeutic agent from Chemical Entities of Biological Interest.
Platform: Small molecule
Danazol, continuous oral contraceptives and progestins suppress ovarian estrogen production to induce hypoestrogenism and atrophy of the ectopic endometrium.
Mechanism Target:
Cyclical Ovarian Hormone Stimulation
Show evidence (2 references)
PMID:39768909 SUPPORT DIRECT REVIEW SYNTHESIS Human Clinical
"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."
States the mechanism and aim of hormonal ovarian suppression.
PMID:27516783 SUPPORT DIRECT REVIEW SYNTHESIS Human Clinical
"The primary aim of treatment is to induce hypoestrogenism, resulting in the lack of menses."
Confirms hypoestrogenism/ovarian rest as the aim of medical therapy.
Thoracoscopic surgery
Action: thoracoscopic surgeryNCI Thesaurus (NCIT) Relation: this treatment is this clinical intervention This treatment is thoracoscopic surgery (NCIT:C15726). NCIT:C15726 is a clinical intervention from the NCI Thesaurus. Ontology label: Thoracoscopic Surgery NCIT:C15726
Platform: Surgery
Video-assisted thoracoscopic surgery allows pathologic confirmation and treatment of the mechanism — partial diaphragmatic resection and excision of visceral pleural implants.
Mechanism Target:
Visceral Pleural and Diaphragmatic Defects
Show evidence (1 reference)
PMID:20473170 SUPPORT DIRECT REVIEW SYNTHESIS Human Clinical
"Partial diaphragmatic resection and/or exeresis of visceral pleural implants, as well as talc pleurodesis, are nowadays frequently carried out."
Diaphragmatic resection and visceral pleural implant excision are standard operative treatments.
Pleurodesis
Action: pleurodesisNCI Thesaurus (NCIT) Relation: this treatment is this clinical intervention This treatment is pleurodesis (NCIT:C66899). NCIT:C66899 is a clinical intervention from the NCI Thesaurus. Ontology label: Pleurodesis NCIT:C66899
Platform: Surgery
Talc or mechanical pleurodesis obliterates the pleural space to prevent recurrent air accumulation.
Mechanism Target:
Air Accumulation in the Pleural Space
Show evidence (1 reference)
PMID:20473170 SUPPORT DIRECT REVIEW SYNTHESIS Human Clinical
"Partial diaphragmatic resection and/or exeresis of visceral pleural implants, as well as talc pleurodesis, are nowadays frequently carried out."
Talc pleurodesis is a frequently used procedure to prevent recurrence.
Chest tube drainage (acute management)
Action: tube thoracostomyNCI Thesaurus (NCIT) Relation: this treatment is this clinical intervention This treatment is tube thoracostomy (NCIT:C148463). NCIT:C148463 is a clinical intervention from the NCI Thesaurus. Ontology label: Tube Thoracostomy NCIT:C148463
Platform: Surgery
Acute supportive management of the pneumothorax by tube thoracostomy to re-expand the collapsed lung, distinct from the causal therapies that prevent recurrence.
Mechanism Target:
Air Accumulation in the Pleural Space
Show evidence (1 reference)
PMID:39768909 SUPPORT DIRECT REVIEW SYNTHESIS Human Clinical
"in some cases, a chest tube insertion may be necessary to allow for re-expansion of the lung tissue"
Chest-tube insertion is used acutely to re-expand the lung in the pneumothorax.
🔬

Biochemical Markers

1
Serum CA-125 (INCREASED)
Show evidence (1 reference)
PMID:39768909 SUPPORT DIRECT REVIEW SYNTHESIS Human Clinical
"An elevated level of CA-125 may accompany the disease."
Serum CA-125 may be elevated in catamenial pneumothorax.
🔬

Diagnosis

3
Diagnostic videothoracoscopy with histopathologic confirmation
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.
diagnostic videothoracoscopy NCIT:C64949 NCI Thesaurus (NCIT)
Show evidence (2 references)
PMID:39768909 SUPPORT DIRECT REVIEW SYNTHESIS Human Clinical
"video-assisted thoracoscopy (VAT) exploration of the thoracic cavity around the menstruation time allows for optimal visualization of ectopic lesions"
Thoracoscopy timed to menstruation gives optimal visualisation of the ectopic lesions.
PMID:39768909 SUPPORT DIRECT REVIEW SYNTHESIS Human Clinical
"The final confirmation is established by histopathological examination of the tissue material acquired via VATS or open thoracotomy."
Histopathologic examination of surgically acquired tissue is the confirmatory step.
Contrast-enhanced computed tomography
Chest and abdomen CT, preferably contrast-enhanced and performed during menstruation, can show pneumoperitoneum and diaphragmatic lesions.
computed tomography NCIT:C17204 NCI Thesaurus (NCIT)
Show evidence (2 references)
PMID:39768909 SUPPORT DIRECT REVIEW SYNTHESIS Human Clinical
"Chest and abdomen computed tomography (CT), particularly contrast-enhanced, may additionally show pneumoperitoneum and diaphragmatic lesions"
Contrast-enhanced CT can demonstrate pneumoperitoneum and diaphragmatic lesions.
PMID:39768909 SUPPORT DIRECT REVIEW SYNTHESIS Human Clinical
"The CT should preferably be performed during menstruation"
Imaging yield depends on timing the CT to menstruation.
Magnetic resonance imaging
MRI has higher specificity than radiography or CT for detecting endometriosis-related haemorrhage; it is likewise best timed to menstruation.
magnetic resonance imaging NCIT:C16809 NCI Thesaurus (NCIT)
Show evidence (1 reference)
PMID:39768909 SUPPORT DIRECT REVIEW SYNTHESIS Human Clinical
"Although commonly used, both RTG and CT have poorer specificity in CP than magnetic resonance imaging (MRI) in detecting endometriosis-related hemorrhage"
MRI outperforms radiography and CT in specificity for endometriosis-related haemorrhage.
📈

Progression

1
Perimenstrual recurrent episodes
Age: Reproductive age, most commonly 30-40 years
Episodes recur in a fixed relationship to menstruation, typically within 72 hours of onset, and recurrence is common without treatment of the underlying mechanism.
Show evidence (2 references)
PMID:25337402 SUPPORT DIRECT REVIEW SYNTHESIS Human Clinical
"Usually onset of lung collapse is less than 72 hours after menstruation."
Establishes the perimenstrual timing that recurs across cycles.
PMID:31401265 SUPPORT DIRECT REVIEW SYNTHESIS Human Clinical
"Recurrence rate varied from 14.3% to 55%."
Quantifies the high recurrence that characterises the course.
📊

Prevalence

1
Women of reproductive age presenting with spontaneous pneumothorax
Unknown Not yet documented
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.
Show evidence (1 reference)
PMID:31401265 SUPPORT DIRECT REVIEW SYNTHESIS Human Clinical
"The prevalence of catamenial pneumothorax of all cases of pneumothorax in women of reproductive age ranges from 7.3% to 36.7%."
Reports the proportion of female reproductive-age pneumothorax that is catamenial.
🔀

Differential Diagnoses

3

Conditions with similar clinical presentations that must be differentiated from Catamenial Pneumothorax:

Primary spontaneous pneumothorax
Overlapping Features 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.
Show evidence (1 reference)
PMID:34774177 SUPPORT DIRECT REVIEW SYNTHESIS Human Clinical
"Tobacco smoking is a major risk factor for primary spontaneous pneumothorax"
Identifies the smoking association that distinguishes primary spontaneous pneumothorax from the hormonally driven catamenial form.
Overlapping Features 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.
Show evidence (1 reference)
PMID:27514586 SUPPORT DIRECT REVIEW SYNTHESIS Human Clinical
"Lung cysts progressively replace the lung parenchyma, which leads to dyspnea, recurrent pneumothorax, and in some cases respiratory failure"
Establishes that recurrent pneumothorax in LAM stems from progressive diffuse cystic lung disease, the feature that separates it from catamenial pneumothorax.
Overlapping Features 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.
Show evidence (2 references)
PMID:27514594 SUPPORT DIRECT REVIEW SYNTHESIS Human Clinical
"characterized by the formation of fibrofolliculomas, early onset renal cancers, pulmonary cysts, and spontaneous pneumothoraces"
Lists the multisystem hereditary features that differentiate Birt-Hogg-Dube syndrome from catamenial pneumothorax.
PMID:27514594 SUPPORT DIRECT REVIEW SYNTHESIS Human Clinical
"More than 80% of adult patients with BHD have pulmonary cysts"
Documents the basal pulmonary cysts underlying pneumothorax in BHD, a radiologic feature absent in catamenial pneumothorax.
{ }

Source YAML

click to show
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:
📚

References & Deep Research

Deep Research

1

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.

Evaluations and curation notes (1)

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.

Claude Code ▸
Catamenial Pneumothorax: Research Report
claude-haiku-4-5-20251001, claude-sonnet-5-5 10 citations 2026-10-03T18:39:35.545926

Catamenial Pneumothorax: Research Report

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.

1. Disease information

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).

  • Synonyms: menstrual pneumothorax, endometriosis-related pneumothorax. It is the commonest manifestation of TES. The others are catamenial hemothorax, catamenial hemoptysis, and pulmonary endometriosis nodules.
  • Identifiers: I did not confirm OMIM, Orphanet, ICD-10/11 or MeSH entries. ICD-10 N80.8 (other endometriosis) is a plausible fit (unverified). The search tool only reached the NCBI MedGen page (C0340007) and did not retrieve its contents.
  • Lump/split note for dismech: CP is a clinical presentation of TES, not a separate pathogen-defined disease. Consider whether it belongs as a DISEASE entry, as a subtype of thoracic endometriosis or endometriosis, or as a GROUPING member. Record the decision in the stub notes.
  • Data source: The evidence is aggregated case series and case reports. It does not come from EHR-derived cohorts.

2. Etiology

  • Causal factors: There is no single causal gene or exposure. The leading mechanisms are thoracic endometriosis (diaphragmatic, pleural or parenchymal) and passage of air through diaphragmatic defects. Lung blebs or bullae and hormonal effects are other proposed contributors.
  • Risk factors:
  • Pelvic endometriosis. CP can be the first presentation of both thoracic and pelvic disease (Ciriaco et al., PMID 35268286).
  • Reproductive age.
  • Prior uterine or pelvic surgery (unverified).
  • Genetic susceptibility is not established.
  • Protective factors: Ovarian suppression by GnRH analogues or hormonal contraception reduces recurrence once the disease is present. These are treatments, not primary protective factors.
  • Gene-environment interactions: None are established.

3. Phenotypes

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
  • Onset: Typically the 30s to 40s (unverified). Onset is acute and episodic, and episodes recur with the cycle.
  • Quality of life: Repeated hospitalizations and drainage procedures, and diagnostic delay. The sources I found did not give formal QoL instrument data.

4. Genetic and molecular information

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.

5. Environmental information

There are no established environmental, lifestyle or infectious factors. The relevant trigger is endogenous: the menstrual cycle.

6. Mechanism / pathophysiology

Proposed causal chains (the search results name four theories: physiological, migrational, microembolic-metastatic, and the diaphragmatic theory of air passage):

  1. Diaphragmatic air-passage branch (anatomical).
  2. Diaphragmatic fenestrations are present, usually right-sided.
  3. During menses the cervical mucus plug is absent.
  4. Air passes from the genital tract into the peritoneal cavity.
  5. Air crosses the diaphragmatic fenestrations into the right pleural space.
  6. The result is pneumothorax at menses.
  7. This is a proposed pathway, not demonstrated in all patients. It is the usual explanation when no thoracic endometriosis is found.
  8. Thoracic endometriosis branch.
  9. Endometrial tissue reaches the diaphragm or pleura. The route is proposed to be retrograde menstruation with transperitoneal passage, or microembolic or lymphovascular spread. This is inferred, not shown.
  10. Ectopic endometrial implants respond to cyclic hormones.
  11. Menstrual shedding at the implants causes the diaphragmatic perforation or visceral pleural/parenchymal disruption.
  12. Air enters the pleural space, and pneumothorax follows.
  13. Hormonal/prostaglandin branch.
  14. Prostaglandin F2α rises at menses.
  15. It is proposed to cause bronchiolar constriction, especially in expiration.
  16. Alveolar rupture follows and traps air in the pleural space.
  17. This is a hypothesis. The search summary describes it as a theory only.
  18. Bleb rupture branch. Cyclic hormonal changes are proposed to cause spontaneous bleb rupture. This is weakly supported.

Suggested ontology terms (all need lookup before use):

  • GO: response to estradiol, response to progesterone, prostaglandin biosynthetic process.
  • CL: endometrial stromal cell, endometrial epithelial cell.
  • UBERON: diaphragm, parietal pleura, visceral pleura, pleural cavity, right lung.
  • CHEBI: prostaglandin F2α.

No molecular profiling, single-cell or CRISPR data were found.

7. Anatomical structures affected

  • Primary: Right hemidiaphragm, pleura and pleural space, and lung. Right-sided predominance is widely reported (unverified figure).
  • Secondary: Pelvic organs, because endometriosis often coexists.
  • Tissue and cell level: Ectopic endometrial glands and stroma on the diaphragm or pleura.
  • Lateralization: Predominantly right-sided and usually unilateral.

8. Temporal development

  • The course is recurrent and cyclical, with episodes clustering around menses.
  • Without definitive treatment, recurrence is frequent. Chest tube drainage alone carries a recurrence rate of up to 50% according to the search summary.
  • Remission occurs during ovarian suppression, pregnancy, or after menopause (unverified).

9. Inheritance and population

  • It is not a Mendelian disease, and no inheritance pattern is established.
  • It is rare. I could not source prevalence or incidence figures.
  • It affects only people with a menstrual cycle, with onset mostly in the reproductive years.
  • Diagnostic delay is likely, because the cyclical pattern is missed. Reviews describe it as an "unveiled disease" (PMC11728258).

10. Diagnostics

  • Clinical criterion: Pneumothorax recurring within 72 hours of menses, with a cyclical history (unverified criterion).
  • Imaging: Chest X-ray, and CT, which can show diaphragmatic defects or nodules. Pelvic and thoracic MRI can help detect endometriosis. Timing the imaging to menses improves the yield (unverified).
  • Thoracoscopy: VATS allows direct inspection of the diaphragm for perforations or implants. It is also the route for biopsy, with histology showing endometrial glands and stroma. Immunohistochemistry (ER, PR, CD10) is supportive (unverified).
  • Differential diagnosis: Primary spontaneous pneumothorax, lymphangioleiomyomatosis, Birt-Hogg-Dubé syndrome, and other causes of cystic lung disease.
  • Genetic testing: Not applicable for typical CP. FLCN testing is relevant only where cystic lung disease or Birt-Hogg-Dubé syndrome is a differential.

11. Outcome and prognosis

  • Recurrence depends on the treatment. Surgery followed by prompt hormonal therapy gave no recurrence in the series summarized in the search. One patient had a recurrence when hormone therapy was delayed by 6 weeks (from search summaries; I did not read the underlying papers).
  • Mortality is very low, and complications relate to repeated pneumothorax and procedures. I found no sourced survival data.
  • Prognostic factors: completeness of surgical treatment and adherence to hormonal suppression.

12. Treatment

  • Acute episode: Chest tube drainage. Recurrence after drainage alone is high, up to 50% per the search summary.
  • Hormonal therapy:
  • GnRH analogues for 6–12 months are described as the broad consensus for preventing recurrence. They induce hypogonadotropic hypogonadism and amenorrhea.
  • Oral contraceptives, progestins and danazol have been used (unverified).
  • Candidate NCIT terms: Pharmacotherapy NCIT:C15986, with therapeutic_agent for leuprolide or other agents. Look up each agent in CHEBI/NCIT.
  • Surgery:
  • VATS inspection of the diaphragm.
  • Mechanical pleurodesis.
  • Repair of diaphragmatic defects, with or without mesh.
  • Resection of implants or blebs.
  • Candidate NCIT: Surgical Procedure NCIT:C15329. A thoracoscopic-surgery term may exist and needs checking.
  • Combination of VATS plus postoperative hormonal therapy gives the best outcomes, per the search summary.
  • Multidisciplinary care: Thoracic surgery, gynecology and pulmonology (PMC11728258).
  • Pharmacogenomics, gene/cell/RNA therapies and clinical trials: None found. I did not search ClinicalTrials.gov.

13. Prevention

  • There is no primary prevention.
  • Tertiary prevention is hormonal suppression after surgery, started promptly.
  • Early recognition of the menstrual pattern could shorten diagnostic delay.

14. Other species / natural disease

No naturally occurring animal disease was identified. This section is not applicable on the evidence I retrieved. I did not query OMIA.

15. Model organisms

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.

Curation recommendations

  1. Fetch the references below with just fetch-reference PMID:<id> and quote only exact substrings.
  2. Locate the primary large-series sources for laterality, age, frequency and recurrence figures, and for the TES component percentages. Do not rely on my recalled numbers.
  3. Verify the MONDO ID, and decide the lump/split question first.
  4. Run just check-causal-targets and wire the phenotypes. Unconnected phenotypes would be a likely weakness in this entry.

Sources actually retrieved

Reference Validation

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.

Term Validation

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.