A Case Report of MEN Syndrome Type 1 with Boerhaave’s Syndrome Presenting As Tension Hydropneumothorax

Hüseyin Döngelli^1, , Sevgi Kara¹, Betül Kurumluoğlugil¹ and Mesut Akarsu²

¹Department of Internal Medicine, Dokuz Eylul University Hospital, Izmir, Turkiye, 35330

²Department of Gastroenterology, Dokuz Eylul University Hospital, Izmir, Turkiye, 35330

Cite this paper:
Hüseyin Döngelli, Sevgi Kara, Betül Kurumluoğlugil and Mesut Akarsu. A Case Report of MEN Syndrome Type 1 with Boerhaave’s Syndrome Presenting As Tension Hydropneumothorax. American Journal of Medical Case Reports. 2024; 12(9):134-137. doi: 10.12691/ajmcr-12-9-1

Abstract

Boerhaave Syndrome is a spontaneous rupture of the esophagus due to repeated vomiting and has a high mortality rate. Multiple endocrine neoplasia(MEN) syndromes are autosomal dominant disorders that can develop tumors in endocrine glands. A male patient in his 40s was admitted to the emergency room with complaints of chronic diarrhea and nausea. The patient was hospitalized in the gastroenterology department with a preliminary diagnosis of acute pancreatitis which was excluded later. On the first day of hospitalization, the patient developed dyspnea with desaturation after repeated retching. Thoracoabdominal CT revealed massive pleural effusion on the right side. Therefore, a chest tube was inserted. Six-hour later, he became hypotensive and a chest X-ray revealed a left-sided pneumothorax. A second chest tube was inserted. He was diagnosed with Boerhaave Syndrome by esophagography. Metallic stent was placed in the ruptured site.He eventually died of sepsis due to mediastinitis due to Boerhaave’s syndrome.

Keywords:
Boerhaave’s Syndrome Dieulafoy's lesion MEN Syndrome pneumothorax Zollinger-Ellison Syndrome

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References:

[1]	DERBES, V. J., & MITCHELL, R. E., Jr (1955). Hermann Boerhaave's Atrocis, nec descripti prius, morbi historia, the first translation of the classic case report of rupture of the esophagus, with annotations. Bulletin of the Medical Library Association, 43(2), 217–240.
[2]	Lee, W., Siau, K., & Singh, G. (2013). Boerhaave's syndrome presenting as an upper gastrointestinal bleed. BMJ case reports, 2013, bcr2013201267.
[3]	Venø, S., & Eckardt, J. (2013). Boerhaave's syndrome and tension pneumothorax secondary to Norovirus induced forceful emesis. Journal of thoracic disease, 5(2), E38–E40.
[4]	De Schipper, J. P., Pull ter Gunne, A. F., Oostvogel, H. J., & van Laarhoven, C. J. (2009). Spontaneous rupture of the oesophagus: Boerhaave's syndrome in 2008. Literature review and treatment algorithm. Digestive surgery, 26(1), 1–6.
[5]	Roberts, D. J., Leigh-Smith, S., Faris, P. D., Blackmore, C., Ball, C. G., Robertson, H. L., Dixon, E., James, M. T., Kirkpatrick, A. W., Kortbeek, J. B., & Stelfox, H. T. (2015). Clinical Presentation of Patients with Tension Pneumothorax: A Systematic Review. Annals of surgery, 261(6), 1068–1078.
[6]	Callender, G. G., Rich, T. A., & Perrier, N. D. (2008). Multiple endocrine neoplasia syndromes. The Surgical clinics of North America, 88(4), 863–viii.
[7]	Pisegna J. (1999). Zollinger-Ellison Syndrome. Current treatment options in gastroenterology, 2(3), 195–204.
[8]	Engel, J., & Albert, J. G. (2013). Bleeding Dieulafoy's Lesion of the Duodenum as Treated by Endoscopic Metal Clip Placement. Video Journal and Encyclopedia of GI Endoscopy, 1(1), 215-216.
[9]	Schweigert, M., Beattie, R., Solymosi, N., Booth, K., Dubecz, A., Muir, A., Moskorz, K., Stadlhuber, R. J., Ofner, D., McGuigan, J., & Stein, H. J. (2013). Endoscopic stent insertion versus primary operative management for spontaneous rupture of the esophagus (Boerhaave syndrome): an international study comparing the outcome. The American surgeon, 79(6), 634–640.
[10]	Baxter, M., & Aly, E. H. (2010). Dieulafoy's lesion: current trends in diagnosis and management. Annals of the Royal College of Surgeons of England, 92(7), 548–554.
[11]	Vallabhajosyula, S., Sundaragiri, P. R., & Berim, I. G. (2016). Boerhaave Syndrome Presenting as Tension Pneumothorax: First Reported North American Case. Journal of intensive care medicine, 31(5), 349–352.
[12]	Lieu, M. T., Layoun, M. E., Dai, D., Soo Hoo, G. W., & Betancourt, J. (2018). Tension hydropneumothorax as the initial presentation of Boerhaave syndrome. Respiratory medicine case reports, 25, 100–103.
[13]	Nguyen Ho, L., Tran Van, N., & Le, T. V. (2016). Boerhaave's syndrome - tension hydropneumothorax and rapidly developing hydropneumothorax: two radiographic clues in one case. Respirology case reports, 4(4), e00160.
[14]	Onyeka, W. O., & Booth, S. J. (1999). Boerhaave's syndrome presenting as tension pneumothorax. Journal of accident & emergency medicine, 16(3), 235–236.