A YOUNG LADY WITH VANISHING LUNG SHADOWS

Johnson Samuel MD, FRCP

Balamugesh Thangakunam, MD, DM 

Basildon University Hospital, United Kingdom 

Presented at the Great Cases Session at the American Thoracic Society International Conference, May 16, 2010 in New Orleans, LA.

Reference as: Samuel J, Thangakunam B. A young lady with vanishing lung shadows. Southwest J Pulm Crit Care 2011;2:40-4. (Click here for PDF version)

Abstract

   A previously well 20-year-old young lady who presented with nonspecific right-sided chest pain was found to have a rounded shadow on chest X-ray. Investigations to rule out malignancy revealed multiple lung masses. Initial blood tests and percutaneous image guided biopsy were inconclusive. Surgical lung biopsy revealed features suggestive of Bronchocentric Granulomatosis. Her lung shadows spontaneously resolved and there was no evidence of symptomatic or radiological recurrence on follow up for five years. Bronchocentric Granulomatosis is a rare condition particularly in non-asthmatic individuals and should be considered in the lesser-known differential diagnosis of benign lung shadows. 

Case Presentation 

History and Examination

   A 20-year-old lady presented to the Emergency department with right-sided pleuritic chest pain after watching a football match in the local pub.  A Chest radiograph showed a 1cm oval opacity in the periphery of the left upper zone. She was treated with a course of antibiotics and a repeat chest radiograph two weeks later showed an increase in the size of the opacity (Fig 1).

 

Figure 1.  Chest radiograph showing a rounded opacity in left upper zone.

   She was then referred to the respiratory clinic for evaluation. She was a non-smoker with no previous medical history of note. There was no history of recent respiratory infections. Clinical examination including breast examination was entirely normal.

Investigations

    Haemoglobin was 11.2 gm/dl, white cell count 7.0 x10⁹/L, platelet count 255x10⁹/L and erythrocyte sedimentation rate 22mm/hr. Differential white cell count was normal with absolute eosinophil count 0.1x10⁹/L.  Serum angiotensin converting enzyme was 36 IU/L. Serum compliment levels were normal and tumour markers were negative. Human chorionic gonadotrophin level was not elevated. Rheumatoid factor was 38 IU/ml, autoantibodies and Human Immunodeficiency Virus tests were negative. Mantoux test was non-reactive

   Computed Tomography (CT) showed three opacities, two of which were on the right side (Fig 2).

 Figure 2.  Computerised Tomography scan of chest showing bilateral lung opacities

   A CT guided biopsy showed necrotizing chronic granulomatous inflammation with no evidence of malignancy. There were many eosinophils seen in the biopsy sample (Fig 3).

 

Figure 3.  Computerised Tomography guided biopsy of the lesion showing necrotizing granulomatous inflammation. The arrowheads indicate necrosis centre of the picture with many eosinophils. The area circled shows a granuloma including a multi-nucleated giant cell indicated by the short arrow. (High power view- formalin fixed, haemotoxylin and eosin staining).

   As the diagnosis was still uncertain and as there was the suspicion of metastatic malignancy of uncertain origin, she underwent a video assisted thoracoscopic biopsy (VATS biopsy). Histopathology of the nodule revealed abundant necrosis, surrounded by a rim of chronically inflamed fibrous tissue with focal areas of discontinuous elastic lamina (Fig 4).

Figure 4.  Video assisted thoracoscopic biopsy showing focal necrotizing granulomatous inflammation. Arrowhead indicated the central necrosis. Short arrow indicated the multi-nucleated giant cell (High power view – formalin fixed, haemotoxylin and eosin staining).

   Mycobacterial and fungal cultures were negative. There were no features of vasculitis or malignancy.  Based on the histopathology and the clinical features, a diagnosis of Bronchocentric Granulomatosis was made.

Follow up

   The patient was asymptomatic throughout she did not receive treatment. On follow up, after initial increase in size, the nodules started regressing and serial chest X-ray’s showed complete resolution of the lung lesions in four months. She has since been followed up in the respiratory clinic for five years and there was no radiological evidence of recurrence. Her serial spirometry during follow up was normal. She remains asymptomatic and has joined the police force leading an active lifestyle.

Discussion

   Bronchocentric granulomatosis is a necrotizing granulomatous inflammation of the small to medium sized bronchi and bronchioles (1). Approximately half the patients are asthmatics and among these patients, the disease appears to be a hypersensitivity reaction to inhaled allergens particularly Aspergillus fumigatus. (2,3). In the non-asthmatic group, the causative agent is usually not identified. Similar appearances have been described in patients with Pulmonary Echinococcosis, Wegener’s Granulomatosis and Rheumatoid Arthritis (4). Occasionally, it may show histological features indistinguishable from tuberculosis.

    Asthmatic patients, who are usually in the age group 20-40 years, present with worsening wheeze, dyspnoea, cough, fever and occasionally haemoptysis. Peripheral blood may show eosinophilia and Aspergillus fumigatus is commonly grown in sputum culture. Non-asthmatic patients are often asymptomatic or may present with an acute febrile illness. Radiologically, Bronchocentric Granulomatosis can present as mass lesions, alveolar infiltrates, pneumonic consolidation or reticulo-nodular opacities (5). As this can mimic malignancy as in our case, a surgical lung biopsy may be required to rule out vasculitis or malignancy.  There are no definite diagnostic criteria and diagnosis is based on clinical, radiological and pathological correlation. Some patients may need corticosteroids for rapid clinical and radiological resolution while others recover without treatment. Surgical resections have been done to firmly establish the diagnosis given the concern about malignancy. However, when there are no alarming symptoms or signs, it may be appropriate to monitor progress or consider medical treatment.

    Bronchocentric Granulomatosis though rare is a reassuringly benign and usually self-limiting condition, which can present with alarming radiological signs. It should be considered in the asthmatic and asymptomatic patient when other sinister causes are ruled out by definitive investigations, which may include a surgical biopsy.

Acknowledgement:

   Dr. Nazar Alsanjari, Pathology department, Basildon University Hospital, Basildon. For kindly providing the photomicrographs of the biopsy specimens.

References

1. Liebow AA. Pulmonary angiitis and granulomatosis. American Review of Respiratory Disease 1973;108:1-18.

2.  Sulavik SB. Bronchocentric granulomatosis and allergic bronchopulmonary aspergillosis. Clin Chest Med 1988;9:609-21.

3.  Koss MN, Robinson RG, Hochholzer L. Bronchocentric granulomatosis. Hum  Pathol 1981;12:632-8.

4. Bonafede RP, Benatar SR. Bronchocentric granulomatosis and rheumatoid arthritis. Br J Dis Chest 1987;81:197-201.

5. Ward S, Heyneman LE, Flint JD, Leung AN, Kazerooni EA, Miller NL. Bronchocentric granulomatosis: computed tomographic findings in five patients. Clin Radiol 2000;55:296-300.

Corresponding author:

Balamugesh Thangakunam

Speciality Doctor

Respiratory services

Basildon University Hospital

Basildon, Essex, United Kingdom

SS16 5NL

E mail: drbalamugesh@yahoo.com

This case was presented at the Great Cases session at the American Thoracic society meeting in New Orleans, LA on May 16, 2009. A link to the slides used in that presentation is below.

Slide Presentation

A 61 Year Old Immunosuppressed Man With A Symptomatic Pulmonary Infiltrate

Carmen Luraschi-Monjagatta¹

Amber Noon¹

Neil Ampel¹

Suzette Chavez¹

Mitch Goldman²

Kenneth S  Knox¹

University of Arizona, Department of Medicine. Southern Arizona VA Healthcare System. Tucson, Arizona¹

Indiana University School of Medicine, Indianapolis, IN²

Reference as: Luraschi-Monjagatta C, Noon A, Ampel N, Chavez S, Goldman M, Knox KS. A 61 year old immunosuppressed man with a symptomatic pulmonary infiltrate. Southwest J Pulm Crit Care 2011;2:17-24. (Click here for PDF version)

Abstract

   Coccidioidomycosis is a common cause of community acquired pneumonia in Arizona.  Although self-limited in the majority of patients, immunosuppression often causes a blunting of symptoms and a poor antibody response.  We present a patient with coccidioidomycosis while receiving adalimumab for psoriasis.  The diagnosis was challenging due to negative serology and constellation of symptoms on immunosuppression.

Case Presentation

History of Present Illness

   A 61 year-old man with a history of psoriatic arthritis was admitted to the hospital after 5 days of fever, night sweats, dry cough, dyspnea, fatigue and inspiratory chest pain. He was treated for a bacterial community-acquired pneumonia and discharged but was readmitted 2 days later because of persistent symptoms. 

   On readmission he noted that the fevers had resolved, but stated his other symptoms remained unchanged. Twelve years previously, he had been diagnosed with psoriatic arthritis and was initially treated with methotrexate and prednisone. Due to poorly controlled disease, adalimumab was initiated 6 weeks prior to his illness. Coccidioides serology and tuberculin skin testing were negative at that time.

Physical examination

   On re-admission vital signs were within normal limits. Oxygen saturation was 95% on ambient air. He was a well developed man in no acute distress. There was a faint maculopapular rash on his trunk and erythematous plaques with dry silver scale on his limbs. Inspiratory crackles were auscultated over the left lung field anteriorly. Cardiac, abdominal and neurological examinations were normal.

Laboratory findings

   The peripheral blood white cell count was 12,200 cell/mm³ with 12.2 % eosinophils. Other laboratory tests, including hematocrit and hemoglobin, platelets, basic metabolic panel and hepatic panel, were normal. Coccidioides IgM and IgG serology performed by immunodiffusion were negative. The re-admission chest radiography (Figure 1) and computed tomography of the chest (figure 2) are shown.

 

Figure 1: A:  Pulmonary radiograph two months before admission. B: Pulmonary radiograph at admission.

Figure 2: Computerized tomography of the chest showing left lower lobe lesions, left hilar adenopathy, and a right pulmonary nodule (arrow).

   A left-sided pulmonary parenchymal infiltrate, already observed during the first admission, with left hilar adenopathy, were the predominant findings. Indistinct right sided densities were newly appreciated. Bronchoscopy with bronchoalveolar lavage (BAL) was performed which showed a normal airway and a cell differential of 18% macrophages, 14% neutrophils, 15% lymphocytes and 53% eosinophils. A Papanicolau stain was negative for fungal elements. Culture of the BAL fluid was obtained.

Hospital course

   Our patient remained stable with persistent symptoms during his brief hospital stay. Antibacterial treatment for community acquired pneumonia was discontinued and the patient was sent home. Three days later cultures grew Coccidioides. The patient was referred to the Valley Fever Center and started on fluconazole with near complete resolution of his symptoms. Therapy for psoriasis was not restarted.  After 4 weeks, the immunodiffusion (IDTP) turned positive at a titer of 1:4. 

Discussion

   Coccidioidomycosis is a soil-borne fungal infection encountered predominantly in the southwestern United Stated and northern Mexico. In the United States, 60% of symptomatic cases are currently reported from Arizona. Two species, Coccidioides immitis and C. posadasii, are the causative agents. Three important points are illustrated by this case. First, coccidioidomycosis can often be mistaken for a bacterial community acquired pneumonia. Second, the diagnosis of coccidioidomycosis can be challenging, with serology sometimes negative in patients with underlying immunosuppression. Third, therapy with inhibitors of TNF-α may increase the risk of symptomatic coccidioidomycosis.

   Among populations living in the endemic coccidioidal region, approximately 25% of patients presenting to urgent care clinics with community-acquired pneumonia have coccidioidomycosis.(1) The diagnosis can be difficult, but the presence of erythema nodosum, hilar or mediastinal lymphadenopathy on chest radiograph, and peripheral blood eosinophilia are suggestive of coccidioidomycosis.

   Serologic tests for coccidioidomycosis may take up to six weeks in immunocompetent individuals to become positive. Several different assays for coccidioidal antibodies are currently available (2). Acute IgM reactions can be detected using the tube precipitin (TP) assay, immunodiffusion (IDTP), or enzyme immunoassay (EIA). For detection of IgG, complement fixation (CF), immunodiffusion (IDCF), or EIA can be employed. The CF and IDCF can be titrated and elevated titers are an indication of more severe disease.

   Serologic assays for coccidioidomycosis are very specific. Serology testing for coccidioidomycosis is routinely and repeatedly performed during an acute infection but can take up to six weeks in immunocompetent individuals to produce reliably detectable antibodies. In acute disease, IgM can be detected with serologic tests in up to 53% of patients in the first week, which increases to 75% in the presence of erythema nodosum and further increases to 91% at 3-4 weeks of infection (3). However, in the immunosuppressed patient these tests are less likely to be positive than among immunocompetent patients.  Coccidioides antigen testing may be useful in patients with large fungal burden (4).   In our patient, serology was initially negative, but eventually became positive.

   When suspecting pulmonary coccidioidomycosis, a bronchoscopy with bronchoalveolar lavage (BAL) is often performed. Cell differentials frequently show a high percentage of eosinophils. Blood and BAL eosinophilia is common in Coccidioidomycosis. In several recent cases, we have seen intense eosinophilic pneumonia (>50% eosinophils in BAL) due to Coccidioides infection. Although it is tempting to diagnose acute eosinophilic pneumonia in these instances, it is important to keep Coccidioides infection in the differential diagnosis, especially in endemic areas. Cytology examination of BAL secretions can give a rapid diagnosis, but the sensitivity is low, even in severely immunocompromised patients. The Papanicolaou stain is superior to other stains in identifying Coccidioides spherules when compared with 10% potassium hydroxide and to calcofluor white staining. Culture of bronchial specimens increases the yield. The gold standard for the diagnosis of coccidiodomycosis is culture or visualization of spherules in tissue.   Coccidioides grows on routine bacterial or fungal media in three to seven days at 35°C. Growth of Coccidioides on solid culture media is a major hazard for infection and microbiology laboratories should always be alerted when coccidioidomycosis is suspected. In our case, Coccidioides grew from BAL fluid, confirming the diagnosis.

   In humans, an effective response to coccidioidal infection depends on an intact cellular immune response. In this regard, an increased risk of symptomatic coccidioidomycosis (both reactivation and newly acquired infection) in patients with inflammatory arthritis receiving TNF-α antagonists has been documented (5,6). Primary anti-fungal prophylaxis for patients in the endemic area starting anti-TNF therapy is not recommended, but adequate data regarding this subject is lacking. In our practice we check coccidioidomycosis serology and a CXR prior to initiation of anti-TNF therapy and then annually. Although the timing of discontinuing adalimumab in our patient might raise suspicion for immune reconstitution inflammatory syndrome (IRIS), to date Coccidioides related IRIS has not been definitively reported upon withdrawal of anti-TNF therapy.

Summary

   Coccidioidomycosis is a common cause of community acquired pneumonia in the endemic area.  The diagnosis should be included in the differential diagnosis of pulmonary infiltrates with eosinophilia. Immunosuppression with anti TNF- α therapy increases the risk for a negative serologic response in coccidioidomycosis.

References

1. Valdivia L, Nix D, Wright M, Lindberg E, Fagan T, Lieberman D, Stoffer T, Ampel NM, Galgiani JN. Coccidioidomycosis as a common cause of community-acquired pneumonia. Emerg Infect Dis 2006;12:958-62.
2. Blair JE, Coakley B, Santelli AC, Hentz JG, Wengenack NL. Serologic testing for symptomatic coccidioidomycosis in immunocompetent and immunosuppressed hosts. Mycopathologia 2006; 162:317-24.
3. Smith CE, Saito MT, Simons SA. Pattern of 39,500 serologic tests in coccidioidomycosis. JAMA. 1956;160:546-52
4. Durkin M, Estok L, Hospenthal D, Crum-Cianflone N, Swartzentruber S, Hackett E, Wheat LJ. Detection of Coccidioides antigenemia following dissociation of immune complexes. Clin Vaccine Immunol. 2009;16(10):1453-6.
5. Bergstrom L, Yocum DE, Ampel NM, Villanueva I, Lisse J, Gluck O, Tesser J, Posever J, Miller M, Araujo J, Kageyama DM, Berry M, Karl L, Yung CM. Increased risk of coccidioidomycosis in patients treated with tumor necrosis factor alpha antagonists. Arthritis Rheum 2004; 50:1959-66.
6. Ampel NM. New perspectives in coccidioidomycosis. Proc Am Thorac Soc 2010: 7: 181-5.

Corresponding author:

Kenneth S Knox, M.D.

Associate Professor of Medicine, University of Arizona

Southern Arizona VA Health Care System

3601 S 6^th Ave (1-11C)

Tucson, AZ 85723

Tel: 520-629-1848 Fax: (520) 629-4976

Acknowledgment:

We would like to thank the Valley Fever Center for Excellence http://www.vfce.arizona.edu/  for its support

Author Contributions:

- Drs. Luraschi-Monjagatta and Knox had full access to data in the report and take full   responsibility for the integrity and the accuracy of the report.

- Report concept and design: Drs. Luraschi-Monjagatta and Knox.

- Acquisition of data: Drs. Luraschi-Monjagatta, Noon, Knox and Ms Chavez.

- Drafting of the manuscript: Dr. Luraschi-Monjagatta, Ampel and Knox.

- Critical revision of the manuscript for important intellectual content: Drs. Goldman, Ampel, and Knox.

Abbreviations:

tube precipitin (TP), immunodiffusion tube precipitin (IDTP),  enzyme immunoassay (EIA), complement fixation (CF), immunodiffusion compliment fixation (IDCF), Bronchoalveolar lavage (BAL), Immune reconstitution inflammatory syndrome (IRIS) 

PET Positive Pleural Plaques Decades after Pleurodesis: Mesolthelioma?

Ellen A. Middleton¹

Jonathan C. Daniel²

Kenneth S. Knox¹

Kathleen Williams¹

Departments of Medicine¹ and Surgery², University of Arizona College of Medicine, Tucson, AZ

Reference as: Middleton EA, Daniel JC, Know KS, Williams K.  PET positive pleural plaques decades after pleurodesis: mesolthelioma? Southwest J Pulm Crit Care 2011;2:9-16. (Click here for PDF version)

Abstract 

   A 59-year-old patient was evaluated for abnormal chest CT and hypermetabolic pleural foci on FDG-PET scan. The scans were obtained as routine surveillance for resection of an in situ pancreatic tumor.  The patient had a remote history of automobile manufacturing and the abnormalities were suggestive of asbestos exposure.  Because hypermetabolic areas were concerning for pleural malignancy, a VATS lung biopsy was performed and revealed chronic talc-induced pleuritis. The patient had a history of pnemothoraces with bilateral talc pleurodesis at the age of 16. As cancer screening and surveillance increasingly relies on extensive imaging modalities, physicians should be aware of the chronic complications of talc pleurodesis and the possibility of false positive imaging tests.

Background 

   The treatment of primary spontaneous pneumothorax (PSP) varies greatly among physicians. Treatment options include observation, chest tube decompression, simple catheter aspiration, medical thoracoscopy with pleurodesis, and surgical intervention. Talc pleurodesis is often performed as part of the intervention and although both safe and effective, the long term complications of talc exposure are not well defined. Specifically, no documented cases of pleural malignancy after instillation of talc exist. We report a patient with increased pleural activity on 18-fluorodeoxyglucose positron-emission tomography (FDG-PET) mimicking mesothelioma which ultimately proved to be chronic talc-induced pleuritis decades after pleurodesis for PSP. Biopsy and definitive diagnosis via video assisted thoracoscopic surgery (VATS) changed the course of care for this patient.

Case Report

   The patient is a 59-year-old man with past medical history significant for pancreatic adenocarcinoma in situ status post Whipple procedure in January 2009. His follow up radiologic examination revealed calcified pleural-based nodules on his CT scan. The concern for malignant mesothelioma arose, particularly as he had a history of asbestos exposure while working with automobile manufacturing in his youth.

   The patient was referred to the thoracic surgery service for evaluation. FDG-PET scan was performed and pleural nodules with a maximum SUV of 13.1 were noted (Fig 1).

Figure 1. FDG-PET and CT showing bilateral pleural nodules.

    Pulmonary function testing revealed a mild restrictive ventilatory defect.  VATS was performed and the patient underwent left thoracoscopic lysis of adhesions, pleural biopsy, and wedge resections of pleural plaques adhesed to the lung. Frozen section intra-operatively was consistent with talc crystals and fibrosis. Pathologic examination later confirmed chronic pleuritis due to talc extending into lung parenchyma without evidence of granuloma, malignancy, or presence of ferruginous bodies (Fig. 2).

 

Figure 2. The patient’s biopsy specimen showing inflammation and polarizable birefringent crystals consistent with talc.

Discussion 

   Treatment of PSP with talc pleurodesis is an effective and relatively safe means of preventing recurrence. Both acute and long-term adverse effects of talc pleurodesis are few. In our patient, the FDG-PET scan was highly suspicious for malignancy and mesothelioma. A study following patients from 14 to 40 years after pleurodesis for PSP did not shown evidence of mesothelioma or increased incidence of lung cancer (1). Lange et al. (2) demonstrated that greater than two decades post pleurodesis a large percentage of patients have pleural thickening demonstrated by plain chest x-ray and this may lead to mild restrictive impairment. In the era of the FDG-PET scan, a diagnostic dilemma is emerging as it appears many of these changes can manifest as chronic inflammatory lesions with increased metabolic activity.

    Recent case reports have documented talc related pulmonary processes that appeared malignant by FDG-PET. Tenconi et al. (3) documented a case in which a patient was experiencing cough, chest pain, and weight loss associated with hypermetabolic (SUV 4.8) pleural plaques by FDG-PET scan. The patient underwent thoracotomy and total pleurectomy revealing talc pleural granulomas from a pleurodesis 42 years prior. The authors concluded that the treatment of choice for PSP should be VATS blebectomy and pleural scarring. Similarly, Ahmadzadehfar and colleagues (4) presented a patient with pleural, mediastinal, and intrapulmonary lesions demonstrating high glucose uptake on FDG-PET imaging five years following talc pleurodesis. The pathology was consistent with talc granulomatosis.

   FDG-PET is commonly used in the diagnosis of pulmonary nodules and staging of lung cancer. It is emerging as an imaging modality in the diagnosis and management of malignant mesothelioma. The International Mesothelioma Interest Group’s consensus statement states FDG-PET is an accurate and reliable tool to differentiate malignant and benign pleural lesions. The group made the recommendation that one must exercise caution when interpreting surveillance images from patients with talc pleurodesis in the setting of mesothelioma as those patients demonstrate higher average SUV (5). No mention is made of the patient that has the radiographic appearance of mesothelioma, but histologically has talc related lesions.

    Two case series have examined the progression of pleural involvement in cancer patients following pleurodesis for malignant effusion or postthoracotomy air leak.  Nguyen and colleagues (6) performed serial FDG-PET scans between 1 month and 58 months. SUV values ranged from 1.9 to 12.6 and it was concluded that FDG uptake may persist or increase over time in chronic inflammatory plaques. Kwek et al. (7) similarly evaluated patients between 10 days and 71 months after pleurodesis and found an SUV range from 2.0 to 16.3. One patient was found to have a new area of contralateral increased FDG uptake representing pleural metastasis. The authors concluded that talc deposits can lead to false positive FDG-PET scan but it is essential to monitor stability of hypermetabolic foci.  

Conclusion

   As the population ages, the medical community can expect to see an increased number of patients who underwent talc pleurodesis decades previously. The utility of FDG-PET imaging to define malignant disease is likely limited in patients whose history includes talc pleurodesis. Physicians must be aware of the  pitfalls of emerging radiologic modalities Documented reports of FDG-PET false positives for pleural malignancy include asbestos reaction, pleurisy, recent surgery, radiotherapy, and pleural effusion secondary to inflammatory processes (7). Review of prior CT scans to evaluate the progression or stability of pleural plaques is essential. False-positive results can be devastating to patients, and often lead to invasive procedures.

References

1. Chappell AG, Johnson A, Charles J. A survey of the long-term effects of talc and kaolin pleurodesis. British journal of diseases of the chest. 1979; 73:285-288.
2. Lange P, Mortensen J, Groth S. Lung function 22-35 years after treatment of idiopathic spontaneous pneumothorax with talc poudrage or simple drainage. Thorax. 1988 Jul; 43:559-61.
3. Tenconi S, Luzzi L, Paladini P, Voltolini L, Gallazzi MS, Granato F, Gotti G. Pleural granuloma mimicking malignancy 42 years after slurry talc injection for primary spontaneous pneumothorax. Eur Surg Res. 2010; 44:201-3.
4. Ahmadzadehfar H, Palmedo H, Strunk H, Biersack HJ, Habibi E, Ezziddin S. False positive 18F-FDG-PET/CT in a patient after talc pleurodesis. Lung Cancer. 2007; 58:418-21.
5.  Nowak AK, Armato SG 3rd, Ceresoli GL, Yildirim H, Francis RJ. Imaging in pleural mesothelioma: A review of imaging research presented at the 9th international meeting of the International Mesothelioma Interest Group. Lung Cancer. 2010;70:1-6.
6.  Nguyen NC, Tran I, Hueser CN, Oliver D, Farghaly HR, Osman MM. F-18 FDG PET/CT characterization of talc pleurodesis-induced pleural changes over time:a retrospective study. Clin Nucl Med. 2009 Dec; 34 (12) :886-90. 
7. Kwek BH, AquinoSL, Fischman AJ. Fluorodeoxyglucose positron emission tomography and CT after talc pleurodesis. Chest. 2004;125:2356-60.

Corresponding author:

      Kathleen Williams, DO

      Assistant Professor of Medicine

      Pulmonary Critical Care Division

      1501 N Campbell Ave

      Tucson, AZ 85724-5030

      Tel: (520) 626-6114 Fax: (520) 626-6970

Author Contributions:

      - Acquisition of data: Dr. Daniel

      - Drafting of the manuscript: Dr. Middleton 

      - Critical revision of the manuscript for important intellectual content: Drs. Middleton, Knox, and Williams.

Abbreviation List:

      CT- Computed tomography

      FDG-PET- Positron-emission tomography with 18-fluorodeoxyglucose

      PSP- Primary spontaneous pneumothorax

      VATS - Video-assisted thoracoscopic surgery

The Development of Glossopharyngeal Breathing and Palatal Myoclonus in a 29 Year Old after Scuba Diving

Cristian Jivcu, MD

Manoj Mathew, MD

David M Baratz, MD

Allen R Thomas, MD 

Banner Good Samaritan and Phoenix VA Medical Centers

Phoenix, AZ

Reference as: Jivcu C, Mathew M, Baratz DM, Thomas AR. The development of glossopharyngeal breathing and palatal myoclonus in a 29 year old after scuba diving. Southwest J Pulm Crit Care 2011;2:3-6. (Click here for PDF version)

Introduction

   Palatal myoclonus is a rare movement disorder characterized by brief, rhythmic involuntary movements of the soft palate.  Palatal myoclonus is further subdivided into “essential palatal tremor” (EPT) and “symptomatic palatal tremor” (SPT).  EPT is characterized by involvement of the tensor veli palatini, myoclonus that might persist during sleep, as well as ear clicks, usually the patient’s presenting complaint.  The MRI and neurological exam are normal in EPT.  SPT is characterized by involvement of the levator veli palatini and myoclonus which consistently perseveres during sleep.  The MRI shows olivary hypertrophy and clinical features may include ataxia, dysarthria and nystagmus, depending on the size of the lesion¹.  Glossopharyngeal breathing is a technique used by deep-sea divers to increase lung vital capacity, which is also useful in patients with ventilator dependence from poliomyelitis and Duchenne muscular dystrophy.  To date there have been no reported cases of palatal myoclonus and glossopharyngeal breathing occurring simultaneously.  We present the case of a 29 year-old female with palatal myoclonus and glossopharyngeal breathing after scuba-diving.

Case Presentation

   A 29 year-old female presented to her physician with complaints of fevers, chills, rapid shallow breathing, ear clicks and inspiratory “spasms of the neck.” The symptoms started three days after a week-long scuba-diving trip.  She reported a total of four consecutive and uneventful dives no deeper than 50 feet.  Her descents and ascents were well-controlled.  On initial presentation she was ruled-out for pulmonary embolism and was discharged home.  Two months after first presentation she was referred to our office with persistent symptoms of ear clicks, intermittent headaches and neck spasms.  The headaches were bilateral, starting in the occipital region, radiated frontally to the eyes and felt “like a hang-over.”  The neck spasms which initially occurred with a frequency of one with every breath had decreased to one spasm every 2-3 breaths (click here for video-requires Quiktime).  Symptoms of dyspnea, wheezing, cough were absent and the rest of her medical history, including a psychiatric history, was unremarkable. Her vital signs showed a blood pressure of 110/76; Heart rate of 85 beats per minute; Temperature of 99F; Respiratory Rate of 16, and oxygen saturation of 100% while breathing room air.  On exam palatal tremor was noted with every breath, and glossopharyngeal breathing occurred every 1-2 breaths. Cranial nerves II – XII were intact and no difficulty was noted with swallowing or speaking.  Her strength was 5/5 in bilateral upper and lower extremities, and her biceps, brachioradialis and patellar reflexes were normal (+2).  Her gait was normal.  The rest of the physical exam was normal.  Her workup included a normal chest X-ray and a normal MRI of the brain. Notably absent in the brain MRI was olivary hypertrophy.  She was started on clonazepam for palatal myoclonus and neck spasms which were consistent with glossopharyngeal breathing.   After one week of administration the patient reported symptomatic improvement.  By week 2 the palatal myoclonus and glossopharyngeal breathing had completely resolved.

Discussion

   Glossopharyngeal breathing was first noted in 1951 when a respirator-dependent poliomyelitis patient with a paralyzed diaphragm was noted to be “gulping air” and increased his vital capacity from 250cc to 600cc ².  In our case the glossopharyngeal breathing was unintentional, and occurred after a scuba-diving trip.  There are no reported cases of this entity in the medical literature.  Furthermore there have been no reported cases of symptomatic palatal tremor in the setting of glossopharyngeal breathing.  A possible explanation of this co-existent entity can be found through anatomic relationships.  The glossopharyngeal nerve is involved in the movements used in glossopharyngeal breathing.  This nerve also sends a branch to the pharyngeal plexus, which in turn innervates the levator veli palatini, the muscles involved in SPT.  An overstimulation of the glossopharyngeal nerve could theoretically result in glossopharyngeal breathing as well as palatal myoclonus.

Summary

   Palatal myoclonus and glossopharyngeal breathing are usually two unrelated clinical findings.  Our conclusion is that an overstimulation of the glossopharyngeal nerve through inadvertent air-gulping during scuba-diving resulted in symptomatic palatal tremor and unintentional glossopharyngeal breathing.  Treatment with benzodiazepines resulted in complete symptom resolution.

References

1. Zadikoff C, Lang AE. Kelin C.  The ‘essentials’ of essential palatal tremor: a reappraisal of the nosology.  Brain. 2006;129:832-840.
2. Dail CW. "Glossopharyngeal breathing" by paralyzed patients: a preliminary report. Cal Med 1951;75:15-25.
3. Bach JR. Bianchi C. Vidigal-Lopes M. Turi S. Felisari G. Lung inflation by glossopharyngeal breathing and "air stacking" in Duchenne muscular dystrophy. American Journal of Physical Medicine & Rehabilitation. 86(4):295-300, 2007 Apr.
4. Bianchi C. Grandi M. Felisari G. Efficacy of glossopharyngeal breathing for a ventilator-dependent, high-level tetraplegic patient after cervical cord tumor resection and tracheotomy.  American Journal of Physical Medicine & Rehabilitation 2004;83:216-9.