Esotracheal fistulas in adults are rare (fewer than 30 cases in the literature) and few cases have been reported in recent years [1]. Hodgkin’s disease with any sort of fistula formation is extremely rare. Esophageal fistulas have been reported infrequently in the English literature since Vieta and Craver described three patients with advanced Hodgkin’s disease seen at the Memorial Hospital from 1917 to 1940 with this finding [2]. We report the case of a young patient followed for Hodgkin’s lymphoma associated to an esotracheal fistula.

A 27-year-old patient, followed for stage IV Hodgkin’s lymphoma, consulted in the emergency room for dyspnea, having as antecedent a Hodgkin’s lymphoma diagnosed when he was 25 years old and having received two chemotherapy sessions, the last one dating back to one month before his symptomatology.

The physical examination showed the following:

• Respiratory rate: 36 cycle/min
• Pulsed oximetry: 80% under 6 L of oxygen
• Heart rate: 133 bpm
• Blood pressure: 64/34 mmHg
• Glasgow score: 6
• Fever: 39.5°C

His arterial blood gas test showed a severe metabolic acidosis pH: 7.1 with HCO₃-: 14 mmol/L, hypoxemia PaO₂ at 50 mmHg with hyperlactatemia 3.8 mmol/L and anemia at 6 g/dL.

The patient was admitted from the emergency room to the intensive care unit, where his ventilation was first ensured via an oxygen mask at high concentration. Then we took an arterial line for continuous monitoring of blood pressure and put in place a femoral vein cannulation to administer vasoactive drugs. The patient also received a blood transfusion and sodium bicarbonate in order to stabilize his blood pressure.

A nasogastric tube was put in place with aspiration of gastric content, then put in siphoning mode. We noticed the bag quickly inflating with air. An orotracheal intubation after stabilization of the blood pressure was performed with mechanical ventilation in controlled mode. The patient then received a thoracic CT scan which revealed a massive left pleural fluid effusion and a stomach full of air (Figure 1).

Then the patient had a chest drainage, which brought back 5 L of purulent fluid, with a control X-ray showing the drain in place and the lung returning to the chest wall, which was then confirmed by ultrasound (Figure 2).

Due to the persistence of desaturation, the need for increased noradrenaline doses, a bag of siphonage connected to the nasogastric tube full of air confirmed by imagery, it was decided to perform a bronchial fibroscopy, a nasogastric fibroscopy and to review the CT scan reconstruction sections (Figure 3).

The bronchial fibroscopy showed a left bronchus full of inflammatory granuloma while the right bronchus was unexplorable, probably because of the fistula confirmed by the CT scan which showed a right esobronchial fistula. Unfortunately, the patient died before we could conduct an esogastric fibroscopy. We could not achieve any selective intubation because of the right esobronchial fistula and the destroyed left lung. One of the possible alternatives was the ECMO, which we did not have at our disposal.

Esotracheal fistulas and Hodgkin’s lymphoma

• Hodgkin’s disease with any sort of fistula formation is extremely rare. Esophageal fistulas have been reported infrequently in the English literature since Vieta and Craver described three patients with advanced Hodgkin’s disease seen at the Memorial Hospital from 1917 to 1940 with this finding [2].
• A total of 22 patients with Hodgkin’s disease causing esophageal fistula have been described previously in the English literature [2],[3].

Most patients had an advanced lymphoma with recurrence (eight patients) or a stage IIIB/IV lymphoma (five patients) when the fistula was diagnosed.

• Fistulas formed most often in the upper twothirds of the esophagus, resulting most commonly in tracheoesophageal communications [4].
• Fistula formation as a complication of either RT or chemotherapy for Hodgkin’s disease is an extremely rare event. Only two patients described in the literature had such an occurrence [5],[6].

In our case, the fistula was probably formed as a result of RT, it was responsible for the death of our patient since the other decompensation was managed, namely drainage of the pyothorax, disappearance of the fever under antibiotherapy, improvement of its hemodynamic state by noradrenaline and blood transfusion.

• Malignant esotracheal fistulas are rare, they are commonly seen with esophageal or lung cancer, which are associated with a high mortality rate [7],[8].
• A literature review reported a total of 22 patients with Hodgkin’s lymphoma and a tracheoesophageal fistula. It showed that the fistula could be seen with any of the classical Hodgkin’s lymphoma subtypes. Usually, it is also associated with an active disease at the site of fistula [4].

However, esotracheal fistulas could be developed from rapid tumor necrosis during RT or chemotherapy. It could also be seen as a late complication of RT because of the erosion of scar tissue [9].

• Two-thirds of fistulas occurred in the upper two-thirds of the esophagus, and 67% of fistulas closed without surgical intervention, just with chemotherapy and/or RT [4],[10].

Management and treatment

• The most important feature of management of tracheoesophageal fistulas is early identification and initiation of therapy. The recommended initial therapy is placement of a stent, either in the trachea or esophagus [11].
• Stenting prevents further damage to the respiratory tract and thus decreases the risk of pneumonia or respiratory compromise to allow for malignancy specific therapy. In addition, feeding via a gastrostomy tube further reduces the risk of pulmonary soilage, aspiration pneumonia, and mediastinitis.
• Extracorporeal membrane oxygenation can give the patient more chances, especially refractive hypoxemia. This technique uses a membrane gas exchanger. By extension, the ECMO has become a respiratory and cardiorespiratory assistance technique used in case of respiratory failure and/or cardiac failure while waiting for the restoration of a normal respiratory state or a possible transplantation.

Its use is easy, quick, and can be initiated in the bed of the patient.

Some thoracic surgery centers use it routinely as assistance to the realization of lung transplantation and also some intensive care unit teams use it for the treatment of acute respiratory distress syndrome [12].

In our case, the patient died because of the refractive hypoxemia even with 100% of FiO₂ in the mechanic ventilation, the ECMO would give us more time to treat the fistula and save our patient.

Prognosis

Tracheal fistulas complicating Hodgkin’s lymphoma are rare and have a much better prognosis than fistulas of other malignancies [8].

Esotracheal or esobronchial fistula could be seen with any of the classical Hodgkin’s lymphoma subtypes. It could also be a complication of either RT or chemotherapy for Hodgkin’s lymphoma which is an extremely rare event. The majority of fistulas occurred in the upper two-thirds of the esophagus, and 67% treated solely with chemotherapy and/or RT closed without surgical intervention. In the case of our patient, the rapidly deteriorating evolution of his respiratory state did not give us the time to perform an esogastric endoscopy and manage the fistula. If we had ECMO we could have a better evolution.