Bradshaw, Murray, Amundson

m.

Computed tomographic scan of the chest from (A) 1991 showing a giant bulla in the right lung with significant shift ofthe mediastinum to the left and (B) in 1993 showing a dramatic reduction in the size of the bulla. Note that the lung isalmost completely re-expanded and the mediastinal shift is resolved.

inflammation may further obstruct already com-promised bronchial communications with thebullae resulting in a closed space. Eventuallyfluid and then air resorption leads to regressionofthe bulla. If this mechanism is valid, one mightspeculate that other known causes of airwayobstruction such as tumour, mucous plugging,or blood clot could also lead to shrinkage of thebulla. A retrospective review from Japan reportedthree cases of lung bulla regression associatedwith bronchogenic cancer, although no detailson individual cases were provided.'0Our case is of interest not only because of

the rarity with which spontaneous regressionhas been reported in the literature, but alsobecause it was associated with such dramaticimprovements in the radiological picture andpulmonary function. Unlike earlier reports, thisoccurred in the absence of overt infection ortumour. Re-expansion of compressed lung andreversal of mediastinal shift was accompaniedby improved pulmonary blood flow. Substantialincreases in FEV, as documented in thispatient, are sometimes seen following surgicalbullectomy in well selected patients. Ourpatient was not thought to be a good candidatefor bullectomy because of diffuse emphysema,

which underlines the difficulty in selectingpatients for surgery.

The Chief, Bureau ofMedicine and Surgery, Navy Department,Washington, DC, Clinical Investigation Program sponsoredthis report #84-16-1968-495, as required by HSETCINST6000.41A. The views expressed in this article are those of theauthors and do not reflect the official policy or position of theDepartment of the Navy, Department of Defense, or the UnitedStates Government.

1 Thurlbeck WM. Chronic airflow obstruction. In: Pathologyof the lung. Stuttgart, New York: Thieme Medical Pub-lishers, 1988:549-50.

2 Boushy SF, Kohen R, Billig DM, Heiman MJ. Bullousemphysema: clinical, roentgenologic and physiologic studyof 49 patients. Dis Chest 1968;54:327-34.

3 Stone DJ, Schwartz A, Feltman JA. Bullous emphysema. Along-term study of the natural history and the effects oftherapy. Am Rev Respir Dis 1960;94:493-507.

4 CIBA Guest Symposium. Terminology, definitions, andclassification of chronic pulmonary emphysema and re-lated conditions. Thorax 1959;14:286-99.

5 Murphy DM, Fishman AP. Bullous disease of the lung. In:Fishman AP, ed. Pulmonary diseases and disorders. NewYork: McGraw-Hill, 1988:1219-22.

6 Morgan MD, Edwards CW, Morris J, Matthews HR. Originand behaviour of emphysematous bullae. Thorax 1989;44:533-8.

7 Rubin EH, Buchberg AS. Capricious behavior ofpulmonarybullae developing fluid. Dis Chest 1968;54:546-9.

8 Rothstein E. Infected emphysematous bullae. Report of fivecases. Am Rev Tuberc 1954;69:287-96.

9 Douglas AC, Grant IW. Spontaneous closure of large pul-monary bullae. A report on three cases. Br Tuberc DisChest 1958;33:335-8.

10 Tsutsui M, Araki Y, Shirakusa T, Inutsuka S. Characteristicradiographic features of pulmonary carcinoma associatedwith large bulla. Ann Thorac Surg 1988;46:679-83.

Thorax 1996;51:550-552

CardiothoracicCentre,Liverpool L14 3PE,UKG E WilsonC C Evans

Correspondence to:Dr C C Evans.Received 9 September 1994Retumed to authors30 November 1994Revised version received20 February 1995Accepted for publication24 February 1995

Sternocostoclavicularhyperostosis presentingwith thoracic sinusformation

G E Wilson, C C Evans

AbstractSternocostoclavicular hyperostosis (SCCH)is a condition which is welil described in theJapanese literature but is rare in WesternEurope. It is characterised by pain and

swelling in the upper anterior part of thechest, which tends to be progressive. Apatient is described with bilateral chronicdischarging sinuses over the anterior endsof the clavicles in whom the diagnosis ap-peared to be one of SCCH.(Thorax 1996;51:550-552)

Keywords: stemocostoclavicular hyperostosis, thoracicsinus, pustular psoriasis.

Case reportA 75 year old woman of Ashkenazi Jewishextraction was referred because of increasingshortness of breath. For five years she hadsuffered from recurrent clavicular problems.Initially this had been swelling, pain, and stiff-ness around the medial ends of the clavicles

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Sternocostoclavicular hyperostosis presenting with thoracic sinus formation

Figure 1 Discharging sinuses over the upper chest walland night venous congestion.

that had progressed to chronic dischargingsinuses on the upper anterior chest wall. Shehad developed pustular psoriasis affecting thesoles of the feet and the palms of the hands.On examination she weighed 67 kg and was

145 cm tall. She was not clubbed or cyanosed.The jugular venous pressure on the right sidewas elevated and non-pulsatile. The veins over

the right breast and upper arm were engorged.On the left side of the neck there were dis-charging sinuses with deformity of the area (fig1). The right side had a large fluctuant massat the head of the sternoclavicular joint and a

pocketed firmer mass over the right stem-omastoid. A surgical scar from her hemi-colectomy for diverticulosis still had a dressingon it two and a half years following surgery.

Investigations at the clinic were as follows:ECG revealed sinus rhythm with evidence ofright atrial enlargement ('P' pulmonale). Lungfunction tests showed a mixed and severe ob-structive and restrictive pattern with forcedexpiratory volume in one second (FEVy) 0-57 1(36%), forced vital capacity (FVC) 0 9 1 (45%),and FEV,% 64. Chest radiography showedapparent thickening of the anterior end of theleft clavicle with some bone destruction (fig 2)The right clavicle was not clearly seen but a

possible opacity was noted in the underlyinglung.Ten ml of pus was aspirated from one of the

lesions and sent for urgent staining. This failedto reveal the presence of any acid and alcoholfast bacilli (AAFB) and subsequent culturewas negative. Her full blood count revealed

Figure 2 Chest radiograph showing bony destruction of clavicles.

haemoglobin of 10-9 g/dl, white cell count of7-2 x 109/1 (lymphocytes 26-2%, monocytes10-3%, granulocytes 63-5%). Her erythrocytesedimentation rate (ESR) was 46 mm in thefirst hour. Serum calcium was normal and thealkaline phosphatase was raised at 171 U/I (nor-mal 10-110). Rheumatoid factor and anti-nuclear factor were negative. The sinuses wereswabbed repeatedly and specimens were sentfor microbiological examination and culture.The swabs failed to grow any organisms in-cluding bacteria, fungi, AAFB, or actinomyces.A computed tomographic (CT) scan showedbilateral destruction of the clavicles and con-firmed the presence of a mass in the right upperchest which extended to the right lobe of thethyroid gland. This appeared to be compressingthe superior vena cava but not the trachea. Inview of the sterile discharging sinuses, anti-tuberculous treatment with rifampicin andisoniazid was started. After eight weeks of treat-ment there had been no resolution ofsymptomsnor of the rate of discharge from the sinusesand the medication was therefore stopped.Nebulised salbutamol, 5 mg four hourly, wasgiven for her airways obstruction with an im-provement in her spirometric parameters. Afibreoptic bronchoscopic examination was car-ried out which showed a normal endobronchialtree. Two years later her physical signs remainunchanged.

DiscussionStemocostoclavicular hyperostosis (SCCH) isa condition with an equal sex distributionwhose peak incidence occurs in middle age.The original reports were from Japan in 1967,although since then cases have been describedin America and Europe (there does not appearto be an increased incidence in Japanese mi-grants to these areas). The disorder was firstreported by Sasaki' who described a case ofbilateral hyperostosis of the clavicles associatedwith pustulosis palmaris and plantaris. Nosingle aetiological agent has been defined andculture of bone biopsy specimens or resectedmaterial has failed to demonstrate an infectivecause.2 There is debate over the pathogenesisof the disease. Kholer et al3 proposed that it isan ossifying periostitis which begins withinbone and progresses to a generalised hy-perostosis, but Fritz et al4 have argued that itis primarily a rheumatological rather than anorthopaedic condition. The diagnosis is prim-arily clinical and radiological. The most fre-quently abnormal laboratory investigationsinclude increased ESR, C-reactive protein andalkaline phosphatase. Radionuclide studiesshow uptake in the sternoclavicular region andmay show extrasternal involvement.

Radiological features of SCCH are said tobe characteristic and have been classified intothree stages by Resnick.5 In the case describedhere the changes corresponded with stage II,with marked destruction of the sternoclavicularjoints, the clavicle, and formation of an ossificmass. Extrinsic compression of the subclavianvein and brachial plexus neuropathies due tothe effect of local pressure have been reported

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Wilson, Evans

and may require decompressive surgery.67 CTscanning or MRI may reveal the characteristicproliferation ofrestrosternal tissue, but a biopsyspecimen may be required to exclude malig-nancy. A biopsy specimen was not taken in thiscase due to the overall poor health of thepatient.As far as we are aware, there are no previous

reports of sinus formation occurring in SCCH,either over the affected area or peripherally.The differential diagnosis of SCCH includes

malignancies such as plasmacytoma involvingthe sternum and metastatic thyroid or oralcavity carcinomas. Non-malignant causes in-clude diffuse idiopathic skeletal hyperostosis,Paget's disease condensing osteitis, and osteo-myelitis.Treatment is usually conservative as no spe-

cific therapy has been shown to alter the courseof the disease. Following stage II, which maylast for many years, the final stage III is char-acterised by ankylosis of the affected joints and

ossification of the cartilage of the ribs. Limitedsymptomatic relief may be gained with the useof non-steroidal anti-inflammatory agents andcorticosteroids.

The authors wish to thank Mr A Jones, Dr J Verbov, Dr HCarty.

1 Sasaki T. A case with osteomyelitis of the bilateral claviclesassociated with pustulosis palmaris et plantaris. RinshoSeikeigeka 1967;2:333-7 (in Japanese).

2 Chigira M, Maehara S, Nagase M, Ogimi T, Udagawa E.Sternoclavicular hyperostosis. A report of nineteen caseswith special reference to etiology and treatment. J BoneJ7oint Surg [Am] 1986;68:103-12.

3 Kholer H, Uehlinger E, Kutzner J, et al. Stemocostoclavicularhyperostosis: painful swelling of the sternum, clavicles andupper ribs. Report of two new cases. Ann Intern Med 1977;87:192-4.

4 Fritz P, Baldauf G, Wilke HJ, Reitter I. Sternocostoclavicularhyperostosis: its progression and radiological features. Astudy of 12 cases. Ann Rheum Dis 1992;51:658-64.

5 Resnick D. Sternocostoclavicular hyperostosis. AJR 1980;135:1278-80.

6 Jirik FR, Stein HB, Chalmers A. Clavicular hyperostosis withenthesopathy, hypergammaglobulinaemia and thoracic out-let syndrome. Ann Intern Med 1982;97:48-50.

7 Haenel LC, Bradway WR, Constantini PJ. Thrombophlebitiscomplicating sternocostoclavicular hyperostosis. PostgradMedJ3 1980;68:113-8.

Thorax 1996;51:552-553

Lymphangitiscarcinomatosacomplicating primary

malignant peritonealmesothelioma

Paul S Craft, Martin S Reading,Sanjiv Jain, Ross A O'Neil

Medical OncologyUnitP S CraftM S Reading

HistopathologyDepartmentS Jain

Radiology DepartmentR A O'Neil

Woden ValleyHospital, Canberra,ACT 2605, Australia

Correspondence to:Dr P S Craft.

Received 5 January 1995Accepted for publication4 April 1995

AbstractA patient with malignant peritonealmesothelioma and a diffuse pulmonaryinfiltrate is described. Computed tomo-graphic scanning suggested lymphangitiscarcinomatosa. This was confirmed on

transbronchial biopsy to be due to meta-static mesothelioma.(Thorax 1996;51:552-553)

Keywords: peritoneal mesothelioma, lymphangitis car-cinomatosa, metastasis.

Malignant mesothelioma is a rare tumour withan annual incidence of between 0-7 and 1-5per million."2 Increasing incidence has beenreported in many countries including theUnited States and, in particular, Australia.' 3The disease is strongly linked to asbestos ex-

posure, particularly crocidolite. Approximately10% of mesotheliomas arise from the peri-

toneum.4 Lymphangitis carcinomatosa is char-acterised histologically by diffuse permeationof tumour cells within pulmonary lymphatics.We are not aware of any previously reportedexamples of lymphangitis carcinomatosa dueto malignant mesothelioma.

Case reportA 52 year old man presented with a two monthhistory of weight loss and upper abdominalpain. There was a history of asbestos exposure20 years earlier. A chest radiograph, taken at theonset ofsymptoms, was normal. An abdominalcomputed tomographic (CT) scan showed adiffuse omental mass. Needle biopsy yieldedcytologically abnormal mesothelial cells con-sistent with mesothelioma.One month later the patient developed a

non-productive cough and exertional dys-pnoea. Auscultation of the lung fields revealedbilateral fine basal crackles. The chest radio-graph on this occasion showed a widespreadreticulonodular pattern with septal thickening.A high resolution CT scan of the thorax re-vealed diffuse nodular thickening with polygonformation (fig 1). Bronchoscopic examinationwas normal. Transbronchial biopsy specimensshowed abnormal epithelioid malignant cellswith mild nuclear pleomorphism and prom-inent nucleoli. These cells were identical tothose obtained from the previous omentalneedle biopsy. Ultrastructurally they showedlong branching microvilli consistent with meso-thelioma (fig 2). Cytotoxic chemotherapy withcisplatin and doxorubicin was associated withstable disease for three months. The patientdied eight months after presentation from res-piratory failure due to progressive disease.

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