Year : 1996 | Volume
: 7 | Issue : 2 | Page : 189--193
Single Lung Transplantation for Alveolar Micro-Lithiasis: The First Clinical Report
Hassan Raffa, Medhat El-Dakhakhny, Khalid Al-Ibrahim, Mohammed S Mansour
Department of Cardiopulmonary Transplantation, King Fahd Heart Center, Jeddah, Saudi Arabia
Department of Cardiopulmonary Transplantation, King Fahd Heart Center, P.O. Box 8488, Jeddah
Pulmonary alveolar microlithiasis (P.A.M.) is a rare pulmonary disorder that pursues usually an asymptomatic course and can culminate in severe respiratory failure. We report a 48 year old Saudi female patient with P.A.M. who deteriorated rather steadily after the initial 18 years of asymptomatic course until a frank type I respiratory failure is established. Single lung transplantation (S.L.T.) was performed successfully and the patient returned to full daily activity and has now survived 12 months post S.L.T. The immunosuppression consisted of Cyclosporine-A 10 mg/kg/day, azathioprine (immuran) 2 mg/kg/day and prednisolone 10 mg daily. The bronchial anastomosis was done by telescoping the recipient and donor main bronchus without omental wrap. A significant bronchial stricture of the anastomotic site occurred 4 months post S.L.T. which was dilated endoscopically with good clinical and bronchoscopic result. No episodes of rejection or infection were encountered so far.
|How to cite this article:|
Raffa H, El-Dakhakhny M, Al-Ibrahim K, Mansour MS. Single Lung Transplantation for Alveolar Micro-Lithiasis: The First Clinical Report.Saudi J Kidney Dis Transpl 1996;7:189-193
|How to cite this URL:|
Raffa H, El-Dakhakhny M, Al-Ibrahim K, Mansour MS. Single Lung Transplantation for Alveolar Micro-Lithiasis: The First Clinical Report. Saudi J Kidney Dis Transpl [serial online] 1996 [cited 2020 Sep 20 ];7:189-193
Available from: http://www.sjkdt.org/text.asp?1996/7/2/189/39526
A variety of degenerative, metabolic, enzymatic and inflammatory changes of the lungs can end in either a restrictive or obstructive pattern of pulmonary disease that can culminate in irreversible respiratory failure  . Pulmonary alveolar microlithiasis (P.A.M.) is a rare pulmonary disorder of yet unknown etiology. It is characterized by diffuse deposition of calcium containing bodies (calcipherytes) in the alveoli and can lead also to severe and irreversible respiratory failure. The first unilateral (single) human lung transplantation was performed in 1963  , and more than 40 such attempts have been made without long-term clinical success. Most of these patients died in the early postoperative period from infection, bronchial dehiscence or from hyperacute rejection  . Successful single lung transplantation (S-L.T) was carried out in Toronto in 1983  . Since then increasing number of S.L.T. have been performed in the world for end-stage respiratory failure due to lung fibrosis  , chronic obstructive airway disease (C.O.P.D.)  , primary pulmonary hypertension  , and secondary pulmonary hypertension (Eisenmenger Syndrome) with repair of an intracardiac communication (A.S.D)  . Our report represent the first successful clinical application of S.L.T. for irreversible respiratory failure due to alveolar rnicrolithiasis.
A 47 year old Saudi female presented to our unit in a severe respiratory embarrassment and hypoxaemia with hypocapnea (Type I respiratory failure). She was on continuous oxygen therapy for the last two years. Her illness dates back for twenty years when accidental chest radiography was interpreted as miliary tuberculosis and she received a full one and half year course of antituberculosis chemotherapy despite the fact she was persistently negative for T.B. on direct smear and culture, and her PPD was nonreactive all through. She had been chest symptom-free for seventeen years. Then she started to notice exertional dyspnea, which progressed over the last three years to reach severe shortness of breath even at rest, she become bed bound and socially isolated. Her physical examination was surprisingly unremarkable comparative to the gross radiological changes. Her pulmonary function tests are shown in [Table 1].
Arterial blood gasses analysis showed type 1 respiratory failure which was only correctable by inhaling 4 liters of Oxygen/ minutes. The chest X-ray revealed diffuse calcification of both lungs in a fine mottled pattern with normal size heart [Figure 1].
She underwent right and left cardiac catheterization which revealed severe pulmonary hypertension with high pulmonary vascular resistance [Table 2].
Lung scintigraphy Technetium Pyrophosphate revealed and verified the uptake by the alveolar calcipherytes [Figure 2].
Perfusion lung scan showed that the right lung was receiving 60% of the cardiac output. Lung biopsy showed that almost all alveoli were filled with rounded psammoma like calcipherytes which were completely devoid of nuclear material. Significantly alveolar wall fibrosis was present. Computerized axial tomography C.T. scan of the chest was performed and confirmed the presence of alveolar microlithiasis with diffuse deposition in both lungs, however, more marked inferiorly. The left lung was more affected than the right lung. Small bleps were also noted [Figure 3].
The patient was accepted by the transplantation committee for single lung transplantation. Her blood group was A+. A compatible 55 year old brain-dead male donor who sustained intracerebral hemorrhage and was available with excellent oxygenation capacity yielding a PO2 of > 280 mmHg on FiO2 of 100% and PEEP of 5 cm H2O for 5 minutes. The recipient thoracic dimensions (submammary diameter) and radiographic vertical and horizontal diameters were compatible. The lymphocytotoxicity test was negative. Both the recipient and donor were C.M.V. positive. The decision was made to perform a left S.L.T. as both recipient lungs were equally involved and the donor left lung was radiographically clearer. A posterolateral thoracotomy was performed in the recipient in the 4th intercostal space with resection of that rib. Then the hilar structures were dissected and exposed. Meanwhile, the donor underwent a median sternotomy with consequent establishment of total cardiopulmonary bypass and extracorporeal circulation. Deep hypothermia was induced on CP-Bypass, while the renal team was dissecting both kidneys for harvesting.
When the core temperature reached 18 degrees centigrade, the donor heart was arrested with cardioplegic solution, the lungs left in mild inflation and the left lung resected in block with a wide cuft of the left atrium. The left main bronchus (L.M.B.) was divided at the carina. The most proximal part of the L.M.B. was clamped and the donor lung immersed in iced saline.
Trial clamping of the left pulmonary artery of the recipient induced a significant rise in the pulmonary artery pressure, measured by the Swan Gauz catheter and induced also cardiac distension and systemic hypotension which necessitated establishment of partial femoral vein-femoral artery cardiopulmonary bypass. The left pneumonectomy was then well tolerated. Donor main bronchi were tailored. The recipient's own L.M.B. was left as long as possible and the donor L.M.B. as short as possible. The donor atrium was tailored and anastomosed to the recipient remaining atrial cuft with a continuous 4x0 prolene suture ensuring a wide and non-restrictive atrial stoma. The recipient L.M.B. was then telescoped into the donor's L.M.B. for 1 cartilage and bronchial anastomosis done with 4x0 prolene continuous suture posteriorly and interrupted sutures anteriorly. No omental wrap, intercostal muscle flap or pericardial flap were used. Left pulmonary arteries were both anastomosed ensuring proper orientation and axis. By using the divided first branch of the pulmonary artery, the left atrial clamp was gradually released and back-bleeding through the untied pulmonary artery suture line was assured to deair the pulmonary vasculature system. When hemostasis was assured, partial cardiopulmonary bypass was discontinued. Arterial blood gases revealed excellent oxygenation capacity of the normally ventilated new lung.
The patient was kept on artificial ventilation for 48 hours. No PEEP was used. All arterial blood gases remained excellent after extubation.
The postoperative chest x-ray revealed normal appearance of the transplanted left lung [Figure 4]. The immune suppression consisted of oral Cyclosporine 10 mg/kg body weight in two divided doses, azathioprine (Immuran) 2 mg/kg body weight as a single dose. Steroids were not used because of their known deleterious effect on the bronchial healing with potential bronchial dehiscence. Anti-thymocytic globuline (ATG) was used from the next day in a dose of 5 mg/kg intravenously, however due to the development of severe leucopenia, it was discontinued after the initial two doses. On the 22nd day, she was noticed to look ill and febrile. Arterial blood gases revealed significant hypoxemia. Chest x-ray was suggestive of a mild perihilar infiltrate and peripheral blood smear revealed leucocytosis. Transbronchial biopsy did not reveal any evidence of rejection or infection. Rejection was however presumed and marked clinical and oxymetric improvement occurred 8 hours after pulse therapy with 500 mg intravenous methylprednisolone (Solumedrol) /day which was continued for 5 days. The patient was discharged at the 29th day in an excellent clinical and respiratory status. She was started on oral prednisolone at the 21st postoperative day. At serial follow-up, she was found after 4 months of S.L.T. to have significant exercise intolerance with hypoxemia and stridor like sounds at auscultation of the transplanted left lung. Bronchoscopy at another hospital revealed bronchial stricture and stenosis at the anastomotic site with left upper lobe collapse most probably due to retained secretions distal to that stricture.
Bronchoscopic dilation and stening was attempted and the left upper lobe was reinflated. There was slight improvement; however the patient presented to us within 2 weeks with severe dyspnea and hypoxemia. The chest x-ray revealed no signs of rejection or infection. Bronchoscopy confirmed a severe stenosis and stricture at the anastomosis site not permitting a 5 mm rigid bronchoscope to pass through it. Successful gradual dialation using larger diameter bronchoscopes was performed. Excellent clinical, radiographic and oxymetric improvement occurred since then and the patient is doing very well with excellent exercise tolerance, normal radiographic appearance of the transplanted lung, normal pulse oxymetry data and no episodes of rejection or infection. The present immunosuppression is carried on with oral daily maintenance dose ' of Cyclosporine of 8mg/kg, with a serum trough level of 600 nanogram %, azathioprine (Imuran) 1 mg/kg/day and prednisolone 5 mg twice daily, 1 tab. of trimethoprime-sulfa is given on alternating days in addition to nystatin (oral and mouthwash). The serial follow-up of hepatic and renal functions revealed no abnormality.
Though P.A.M. runs an asymptomatic or rather mild course in the majority of patients, there were few reports of severe or fatal outcome of the disorder , . Brown et al pointed out that the clinical presentation would depend on the extent of the replacement of the alveolar air by concretions as well as the presence or absence of interstitial fibrosis or hemodynamic changes  . They reported a patient with normal haemodynamics at rest who developed mild to moderate pulmonary hypertension with supine leg exercise, possibly reflecting reduced capacity of the vascular bed evident only with increased pulmonary blood flow and tachycardia. Our patient showed severe pulmonary hypertension, which was related to the marked encroachment on her vital capacity. Five cases were reported from Saudi Arabia but none had such advanced course , . No effective therapy is available for P.A.M., symptomatic patients should be followed by serial pulmonary function testing. Hypoxemic patients should receive supplemental oxygen, while the advanced cases should not be denied the benefit of lung transplantation. Single lung transplantation therefore, as our clinical report demonstrates, is an excellent pollination for patients with end-stage respiratory failure due to j P.A.M. Excellent exercise tolerance can be achieved with S.L.T. alone without the need 2. for bilateral lung transplantation  . This would widen the donor pool for other patients waiting for lung transplantation, reduce the operative morbidity and mortality and preserve some cough reflex. Our lung donor was about 55 years of age with excellent oxygenation capacity.
Yacoub states that lung donors should not be above the age of 40 years  however we believe that donors up to the age of 55 years can be utilized provided there is a good lung function and oxygenation capacity and no history or evidence of smoking so that the donor pool can be extended. Omental, intercostal or pericardial wrap can be omitted if telescoping technique is used in the bronchial anastomosis. C.M.V. match between the donor and recipient should be 8 aimed at (either positive or negative) in order to reduce the probability of severe C.M.V. infections.
P.A.M. will stay for a while as a mysterious disease in its etiology, clinical course and ultimate outcome. Though many cases were followed long enough to monitor the progress or cessation of the disease, small reports described the ultimate impact of the disease on the morbidity and/or mortality of individual patients.
Family screening for patients with P.A.M. should be done. Only serial follow-up and time would tell whether P.A.M. would appear in the transplanted lung or not!
Triebel reported in 1987 a patient with P.A.M. and right ventricular failure, and stated that a combined heart and lung transplantation might be the only therapy that could prolong the patient's life  . Single lung transplantation was never done before for P.A.M. and this first clinical report documents the excellent pollination that can be achieved with it for such patients with terminal alveolar microlithiasis.
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