Case Report

An unusual case of acute dyspnea

Abstract

Emergency physicians sometimes have to face rare causes of acute dyspnea. We report a case of laryngopharyngeal compression in a patient with Launois-Bensaude syndrome. This patient had predictive criteria of difficult intubation and failed to respond to noninvasive ventilation (NIV) treatment. The relatively well-supported situation sustained intubation decision until ventilation ceased a few minutes after in- hospital arrival. In this case, in-hospital intubation was better than prehospital’s.

Launois-Bensaude syndrome consists of an accumulation of multiple painless unencapsulated lipomata in the body which usually only causes physical disfigurement [1-3]. To date, around 200 cases have been reported, and some intrathoracic lipomata causing progressive dyspnea due to tracheal compression have been described [2,4,5]. We report a case of acute dyspnea from laryngopharyngeal compres- sion in a patient with Launois-Bensaude syndrome.

The emergency unit Call Center was contacted by a woman for her 70-year-old husband who had been feeling breathless and had not been able to speak for 1 hour. The dyspnea had deteriorated during the morning, but with no signs of infection or chest pain. The Emergency Unit Call Center immediately sent an ambulance.

Physical examination result revealed a Glasgow Coma Score of 15/15, cardiac frequency of around 100 beats per minute, blood pressure of 170/110 mm Hg in both arms, respiratory rate of around 40 per minute, and saturation of 70%. Auscultation revealed crackles, wheezing, and general- ized rhonchi and stridor. The patient had a sternocleidomas- toid retraction and cyanosis. The rest of the clinical examination was not contributive.

His medical history included hypertension, smoking, chronic obstructive pulmonary disease, and cervical masses surgically removed 3 years previously. He also had Sleep apnea and was treated with a breathing machine at night. His usual treatment was NIV at night, an association of 50 mg of captopril and 25 mg of hydrochlorothiazide, an association

of 500 ug of fluticasone and 25 mg of salmeterol twice a day, and salbutamol on demand. Twelve-lead electrocardiogram only revealed atrial fibrillation.

Treatment consisted of oxygenotherapy with a high concentration mask reaching 90% saturation, peripheral venous catheter, 1 mg/kg of intravenous methylprednisolone and salbutamol aerosol. Facing the lack of improvement, NIV was tried and led to an improvement with 95% saturation in a few minutes. Unfortunately, the patient could not tolerate NIV, which was thus stopped and high concentration mask was reintroduced.

Fifteen minutes later, the patient’s ventilation ceased, which required orotracheal intubation and assisted ventila- tion. This was followed by extreme bradycardia, treated with 1 mg of epinephrine. Blood gas analysis showed pH of 7.27, PO2 of 136 mm Hg, PCO2 of 59 mm Hg. He was transferred to the medical intensive care unit. The intensive care unit staff knew this patient had Launois-Bensaude disease. Computed tomography scan showed a considerable accumulation of lipomata throughout the neck, mediastin, glottis, and vocal cords with fibrosis compounds. There was an unusual difference between the laryngeal and tracheal area owing to this acute aspect of the chronic disease. The final treatment was tracheotomy.

In front of criteria predictive of difficult intubation (cervical masses, short neck, overweight), the relatively well-supported situation, and the lack of benefit (chronic obstructive pulmonary disease), in-hospital intubation is better than prehospital intubation. In this case, the only indication would have been ventilation arrest.

Laurent Boidron MD SAMU 21, CHU de Dijon, 3 rue du Faubourg Raines 21000 Dijon, France

E-mail address: [email protected]

Anne-Laure Mosca MD

Departement de Genetique CHU de Dijon, 21000 Dijon, France

doi:10.1016/j.ajem.2007.06.023

0735-6757/$ – see front matter (C) 2008

111.e2 Case Report

References

1. Launois PE, Bensaude R. De l’adeno-lipomatose symmetrique. Bulletin Memoire Societe Medicale Hopitaux Paris 1898;1:298-318.
2. Harsh A, et al. insulin resistance, hyperleptinemia, and obstructive sleep apnea in Launois-Bensaude syndrome. Obes Res 2002;10:625-32.
3. Donhauser G, Vieluf D, Ruzicka T, et al. Benigne symmetrische lipomatose Launois-Bensaude typ III und Bureau-Barriere-Syndrom. Der Hautarzt 1991;42:311-4.
4. Enzi G. Multiple symmetric lipomatosis: an updated clinical report. Medicine 1984;63:56-64.
5. Brackenbury ET, Morgan WE. Surgical management of Launois- Bensaude syndrome. Thorax 1996;52:834-5.