Ann Thorac Surg 2006;82:e24-e25
© 2006 The Society of Thoracic Surgeons
Case report
Catamenial Bernard-Horner's Syndrome Related to Thoracic Endometriosis
Edoardo Mercadante, MD*,
Cristiano Giovannini, MD,
Fabio Castaldi, MD,
Umberto Passaro, MD,
Roberto Dell'Avanzato, MD,
Elisabetta Abruzzese, MD,
Massimo Carlini, MD
Department of General Thoracic and Abdominal Surgery, S. Eugenio Hospital, Rome, Italy
Accepted for publication May 15, 2006.
* Address correspondence to Dr Mercadante, Department of General Thoracic and Abdominal Surgery, S. Eugenio Hospital, Viale dell'Umanesimo 10, Rome, 00144 Italy (Email: e.mercadante{at}tiscali.it).
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Abstract
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We describe the case of a young woman with a recurrent right Bernard-Horner's syndrome associated with the onset of intermittent supraclavicular mass. Surgical excision of the mass revealed the endometrial pattern of the tumor and the close relationship between Bernard-Horner's syndrome and thoracic endometriosis. No other case of this syndrome has been reported in the literature.
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Introduction
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Thoracic endometriosis is a rare disorder characterized by the presence of functioning endometrial tissue within the pleural cavity, the airways, or the lung parenchyma, with a varying clinical presentation. It affects women, during their reproductive years or women receiving estrogen replacement therapy, presenting with hemoptysis, hemothorax, pneumothorax, pulmonary nodules, pleural implants, or thoracic pain.
A 33-year-old woman was referred to our department for a right sided Bernard-Horner's syndrome (BHS) (miosis, enophthalmos, and palpebral ptosis) associated with right superior arm pain and a supraclavicular mass. A chest roentgenogram showed an enlargement of the right upper mediastinum and a 8-cm cystic tumor of the right apex was evident on thoracic computed tomographic scan (Fig 1). A fine needle aspiration biopsy was performed and chocolate-like fluid was aspirated; however a cytologic examination was nondiagnostic. During the recovery time we observed a regression of the supraclavicular mass and amelioration of the arm pain and BHS symptoms. Accurate anamnestic examination revealed the intermittent onset of the symptoms, but the relationship with the menses was not suspected until the postoperative finding. A preoperative neurologic examination, pelvic ultrasonography, and electromyography of the right superior arm were normal.
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Fig 1. (A) Preoperative chest roentgenogram with the mass in the right apex. (B) Preoperative computed tomographic scan. (C) Postoperative finding.
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During the operation, due to the suspected involvement of the nervous structures of the pleural apex, a right thoracotomy at the fourth intercostal space was performed. A cystic tumor of about 6 cm in diameter with a thick wall and thin adhesions to the apical segment of the superior lobe was found. The tumor was strongly adherent to the thoracic wall, to the vessels of the apex, and to the brachial plexus root. Careful surgical excision was successfully performed and the postoperative course was uneventful. Pathologic specimen examination revealed the endometrial pattern of the tumor cells. Ten months after the procedure the patient was symptom free, with a complete restoration of right miosis, enophthalmos, and palpebral ptosis.
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Comment
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Thoracic endometriosis is a rare disorder with a varying clinical presentation. The mean age at diagnosis is 35 years. Pneumothorax occurs in 73% of the cases, hemothorax in 14%, hemoptysis in 7%, and pulmonary nodules in 6% [1]. In 90% of the patients the symptoms have a temporal relationship with the onset of menstruation, and they usually occur 2 days later. Three different pathogenetic theories have been proposed to explain thoracic endometriosis [2]: (1) migration of air through the uterus and fallopian tubes into the abdomen and then into the thorax through pre-existing diaphragmatic fenestrations; (2) high levels of serum prostaglandins causing vasospasm and bronchospasm, thus resulting in alveolar rupture and pneumothorax; and (3) endometrial tissue microembolization. The latter seems real in our patient.
Treatment of thoracic endometriosis remains controversial and must be tailored to the patient. Although hormonal therapy with progesterone and danazole are successfully used to suppress the endometrial tissue function, recurrence rates greater than 50% have been reported [1]. In addition, hormonal manipulation could be unaccepted by some women because it precludes pregnancy. Pleurodesis, either with talc or by mechanical abrasion, has been used and has shown to be effective in the treatment of the cases complicated by hemothorax or pneumothorax. Video-assisted thoracic surgery pulmonary wedge resections, or surgical excision of any pleural endometrial implant can be curative [3]., and if possible, it must be timed around the beginning of menstrual flow to allow the best visibility of endometriotic implants.
In this case report, the patient was strongly symptomatic for a right sided BHS associated to right superior arm pain and a supraclavicular mass.
Bernard-Horner's syndrome is actually a well-known clinical syndrome due to a deficiency of sympathetic activity and related to a moltitude of conditions as described in Table 1. It was first described in animal experiments as early as in 1727 by François Pourfour du Petit [4]. Nevertheless, the syndrome was more clearly described by Claude Bernard [5] in 1858 and by Horner in 1869 [6].
Our patient complained of unusual presentation of the BHS, characterized by the intermittent onset of symptoms with a closer relationship to the menses.
In conclusion, with reference to the catamenial pneumothorax, defined as recurrent spontaneous pneumothorax occurring within 72 hours before or after the onset of mestruation, we suggest that this rare case of BHS could be defined as catamenial BHS. Even if it is only an anecdotal case, thoracic surgeons and gynecologists must be cognizant of this pathological entity.
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References
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- Josef J, Sahn SA. Thoracic endometriosis syndromenew observations from an analysis of 110 cases. Am J Med 1996;100:164-170.[Medline]
- Fonseca P. Catamenial pneumothoraxa multifactorial etiology. J Thorac Cardiovasc Surg 1998;116:872-873.[Free Full Text]
- Weber F. Catamenial hemoptysis Ann Thorac Surg 2001;72:1750-1751.[Abstract/Free Full Text]
- Pourfour du Petit F. Mémoire dans lequel il est démontré que les nerfs intercostaux fournissent des rameaux que portent des esprits dans les yeux Hist Acad Roy Sci, Paris, (Mém) 1727:1-19.
- Bernard C. Leçons sur la physiologie et la pathologie du système nerveux 1858;2:473-474.
- Horner JF. Über eine Form von Ptosis Klinische Monatsblätter für Augenheilkunde, Stuttgart 1869;7:193-198.
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Abstract
Introduction
