Recurrent Inactive Hydatid Cyst of the Liver Causing Restrictive Pulmonary Physiology.

Hydatid and alveolar cysts are formed by the helminths Echinococcus granulosus and Echinococcus multilocularis, respectively, which are endemic to pastoral areas, and are more commonly found in South America, the Mediterranean, Russia, and China. Hydatid cysts can cause bacteremia, form abscesses, or cause mass effect by compressing surrounding organs. Strategies to prevent such complications include benzimidazoles, surgical resection, and Puncture, Aspiration, Injection and Re-aspiration (PAIR) procedure. A 71-year-old Egyptian man with remote history of Echinococcus infection one year status post PAIR procedure, presented with dyspnea on exertion. On exam, the patient had a palpable right upper quadrant mass. The patient had a known small hydatid liver cyst on prior ultrasound, however repeat imaging showed growth to 15×19×14cm, with right hemidiaphragm elevation, compressive atelectasis, and compression of the right atrium. He had no peripheral eosinophilia and negative echinococcal serology, consistent with remote infection. The patient underwent repeat PAIR procedure and 3L of serous fluid was drained from the cyst. Fluid analysis was negative for scolices, cysts or hooklets. His symptoms improved; however the cyst re-accumulated 1 month later. Total cystectomy was performed surgically by hepatic wedge resection, with permanent improvement in symptoms. This case is a rare example of Echinococcus infection causing significant respiratory morbidity requiring repeated invasive procedures and surgery, in the setting of inactive disease.

Combined-modality therapy for pulmonary alveolar proteinosis in a remote setting: a case report.

BACKGROUND: Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by accumulation of phospholipoproteinaceous material in the alveoli. The presentation is nonspecific but typically includes dyspnea; the spectrum of disease includes rapidly progressive hypoxic respiratory failure. Whole lung lavage (WLL) is the treatment of choice in symptomatic PAP, but transient worsening of oxygenation sometimes requires salvage modalities of support such as extracorporeal membrane oxygenation (ECMO). Granulocyte macrophage colony-stimulating factor (GM-CSF) plays a role in the pathophysiology of PAP. We highlight a case of severe PAP treated with exogenous GM-CSF and sequential lobar lavage due to the unavailability of salvage methods of oxygenation. CASE PRESENTATION: A 36 year old female was admitted with fevers, chills, and progressive dyspnea. On presentation she was tachypneic, tachycardic, and hypoxemic; labs revealed leukocytosis and lactic acidosis. Chest CT identified diffuse ground glass opacities in a 'crazy-paving' pattern. Following intubation due to impending respiratory failure, bronchoscopy with bronchoalveolar lavage was performed. The lavage return stained positive with Periodic Acid Schiff, confirming the diagnosis of PAP. Continued deterioration necessitated treatment; however, at this geographically remote center without ECMO services WLL was judged to carry significant risk. Nebulized GM-CSF was administered without significant improvement. Subcutaneous GM-CSF was administered and isolated subsegmental lavages of the bilateral upper lobes were performed, with rapid improvement in oxygenation. Additional sequential lobar lavage and continued GM-CSF therapy as an outpatient resulted in complete resolution of oxygen requirement and return to normal pulmonary physiology. CONCLUSIONS: The autoimmune form of PAP is the most common, indicating that therapy with GM-CSF may play an important role for many patients. Treatment with WLL may be impractical in some clinical settings due to the expertise and salvage modalities required. Sequential lobar lavage requires less specialized expertise and may incur less risk of refractory hypoxemia. We posit that this combined-modality therapy is ideally suited to geographically-remote centers such as our own.