A pneumoperitoneum: or the importance of the infradiafragmatic region for pulmonologists' Ulrike Him

1
A pneumo-peritoneum or the importance of the
infra-diafragmatic region for pulmonologists.
Ulrike Himpe, Eric Verbeken, Johny Verschakelen,
Philippe Nafteux, Wim WuytsU.Z. Leuven
Gasthuisberg dienst pneumologie
Case report
Abstract
The first case of thoracic endometriosis syndrome
(TES) was published in 1938 by Schwarz but only
34 years later the link with the menstrual cycle
was made by Maurer. Since then over 140 articles
were described in literature. Endometriosis
affects 5 to15 of all menstruating women. TES
is the term used to refer to varying clinical and
radiological manifestations associated with the
growth of endometrial glands and stroma in the
lungs or on the pleural surface. Catamenial
pneumothorax represents the most common
manifestation besides haemoptysis, pulmonary
nodules and haemothorax. We present a case of a
35 year old female with recurrent pneumothorax in
the history, now presenting with concomitant
catamenial pneumothorax with asymptomatic
pneumoperitoneum and hemoptysis, which was
successfully treated with hormonal replacement
and surgery.
1. History A 34 year old female patient
underwent surgery in 1997 for pelvic
endometriosis AFS IV. In 2001 a bilateral
pleurectomy was performed for spontaneous
bilateral pneumothorax. 2. Presentation
intermittent hemoptysis and progressive dyspnea.
3. Medication Ovysmen (ethinylestradiol 0.035
mg norethisteron 1 mg), Naproxen and
Paracetamol. 4. Clinical examination negative.
5. Pulmonary function testing restrictive
pattern with diminished diffusion.

• 6. X-ray thorax bilateral pneumothorax
• 7. Chest CT bilateral pneumothorax, pleural
  fluid right, some ground glass opacities
  posterior in the right lung (compatible with
  pulmonary bleeding) and a substantially amount of
  intra-abdominal air and intraperitoneal fluid
  without evidence of an intestinal perforation.
• 8. Blood analysis elevated sedimentation rate
  and C-reactive protein. Hemoglobin was 10.9 g/dl.
  Arterial blood gas analysis was normal.
• 9. Bronchoscopy a little purple lesion of 3 mm
  on the ventral side of the distal trachea was
  seen. Proximal in the main right bronchus there
  was some old blood visible. Cultures, central en
  peripheral biopsies were negative. BAL analysis
  showed a few hemosiderin-loaded macrophages,
  immunological analysis was normal.
• 10. Gynaecological ultrasound recurrence of deep
  endometriosis in the rectosigmoid part of the
  colon with important adhesions. Secondary to a
  peritoneal pseudocyst there was a
  hematoperitoneum.
• 11. Treatment
• - a bilateral arterial embolisation with PVA
  -microparticles.
• a chest tube was placed in the left hemi-thorax
• a right thoracotomy with decortication was
  performed with closure of a proven fenestration
  of the diaphragm.

Pathogenesis
TES has been classified as either pleural or
parenchymal disease. The pathogenesis of TES
remains unclear. It is thought to be a
consequence of sloughing of thoracic endometrial
tissue. Endometrial tissue may be found in the
lung parenchyma, visceral and parietal pleura,
diaphragm and rarely in the tracheobronchial
tree. 4 theories have been proposed to explain
TES. 1.Metaplasia theory by coelomic metaplasia
with spontaneous rupture of blebs. 2.Embolisation
theory by lymphatic or hematogenous embolisation
from the uterus and pelvis. 3.Metastatic/Transplan
tation theory retrograde menstruation with
subsequent transperitoneal-transdiaphragmatic
migration of endometrial tissue. 4.Menses
synchronous increase in prostaglandins PGF2?
which may cause vaso- and bronchospasm with
possible rupture of alveoli. None of these
theories can per se explain all the clinical
manifestation of TES, and the disease has
probably a multifactorial etiology. Pleural
endometriosis is believed to be caused by
regurgitation and migration of tissue to the
pleura trough diafragmatic fenestrations,
parenchymal endometriosis is believed to arise
from embolism of tissue via the pulmonary
arteries.
Background
The diagnosis of TES is challenging and should be
made primarily on clinical grounds. It should be
considered in women presenting with spontaneous
pneumothorax, usually right-sided and coincident
with menses. Several case reports have been
published showing the improved recognition of
the disease and an increased interest by the
medical community. Despite this, the exact
incidence, the pathologic mechanisms as well as
the optimal management of TES remain unclear.
Four theories have been proposed to explain TES,
none of these can explain every presentation.
Management may be very difficult, from both
surgical and a medical point of view.

12. Biopsies diaphragm chronically inflammatory
infiltrate and collections of hemosiderin-loaded
macrophages. Pleura focally some gland
structures with endometrial type epithelium
besides hemosiderin-loaded macrophages.
Immunochemically there was a slight positive test
for estrogen receptors. 13 Follow-up - 1
month later subjective much better, but
re-evaluation with X-ray and CT-scan showed a
recurrent pneumothorax on the right side with
diminished ground glass opacities on the left
side. New chest tube and talcage were
performed. Finally the patient agreed to start
hormonal therapy with nomegestrolacetaat
(Lutenyl). - 2 months after this procedure
there was still a small pneumothorax visible on
the right side, besides a residual pneumothorax
on the left side which eventually recuperated
spontaneously.
Treatment

• In TES we are confronted with the localized
  complication of asystemic disease. 2 goals
• closure of the holes by surgery
• prevent recurrence by medical castration
  (hormonal treatment or hysterectomy with
  bilateral salpingo-oophorectomy in case of
  failure of other treatment)
• It is necessary to individualize treatment based
  on each case. If hormonal replacement therapy is
  administered, recurrence is possible. Nowadays
  there is agreement that the association of VATS
  surgery with hormonal treatment represents the
  standard therapy of catamenial pneumothorax.

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