Intrathoracic meningocele

Intrathoracic Meningocele * A Case Report RICHARD R . MOYER, M .D ., HARRY R . CRAMER, M .D . and GARFIELD G . DUNCAN, M .D . Philadelphia, Pennsylvania CASE REPORT

x intrathoracic meningocele associated with neurofibromatosis (von Recklinghausen's disease) was first reported by Pohl in 1933 .' Since that time some twenty cases have been

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T . D . (Unit History No . 11821), a thirty-nine year old white woman, had cutaneous manifestations of neurofibromatosis apparent since birth . There was no family history of the disease . The course was uneventful until the patient was nineteen years of age when, because of progressive kyphoscoliosis, she was admitted to a Philadelphia hospital . A diagnosis of Pott's disease of the spine was made . The patient was asymptomatic following a spinal fusion procedure (in spite of prompt recurrence of her kyphoscoliosis) until the age of thirty-nine, when she complained of headaches of moderate degree . It is significant that the headache was precipitated by coughing . She also suffered from dyspnea on exertion, a symptom which slowly increased in severity and eventually accounted for her admission to the Pennsylvania Hospital six weeks after the onset of these symptoms . Upon physical examination she was found to have multiple "cafe au lait" spots and cutaneous neurofibromas, a right kyphoscoliosis (Fig . 1) a hydrothorax on the right side (Fig . 2A) and generalized hyperreflexia . A thoracentesis yielded 2,000 cc . of a sterile, straw-colored fluid . A residual radiopaque shadow in the posterosuperior aspect of the right side of the chest was interpreted as due to loculated pleural fluid . (Fig. 2B .) Further attempts at aspiration were unsuccessful . Because of the diagnosis of probable tuberculosis, antituberculous therapy was begun and the patient was discharged to be followed in the outpatient clinic . Six weeks following her discharge Miss T. D . was readmitted to the hospital because of persistence of the density in the right hemithorax. On the day of her admission it was reported that acid-fast bacilli had been obtained from gastric washings cultured at the time of her previous admission . These organisms were subsequently found to be virulent via guinea pig inoculation . Since it was still believed that the radiopaque area in the right lung field was due to loculated fluid, a needle was inserted in the fourth intercostal space posteriorly approximately 4 cm . from the convexity of the thoracic spine (Fig . 1), and crystal-clear fluid with the appearance and chemical properties of spinal fluid was withdrawn . It was then

FIC . 1 . Note caf6 au lait spots, neurofibromas, right kyphoscoliosis (X) and site of second thoracentesis

J.

reported . According to Laitinen et al . 2 only five of these were diagnosed preoperatively . It is our purpose in this paper to add one more case of intrathoracic meningocele associated with neurofibromatosis and complicated by pulmonary tuberculosis ; to discuss the diagnosis and methods of treatment ; and briefly to discuss the probable pathogenesis of these lesions .

From the Divisions of Internal Medicine, Pennsylvania Hospital, and the Benjamin Franklin Clinic, Philadelphia, Pa . 334

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Intrathoracic Meningocele--Mover

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2 .A 2B 2C . : 2 . (A) the roentgenngram obtained at time of initial admission showing right pleural effusion . (B), to( ntgenogram Fu taken after the thoracentesis was done shows residual opacity in the posterosuperiur region nn the right side, (G) . ntyclugraphy, with air as contrast medium, reveals a fluid level within thr mcningneele . considered that the roentgen shadow under consideration was due to a meningoccle . Various methods have been used in an attempt to delineate these thoracicc lesions : thoracic puncture, alone and with a simultaneous lumbar puncture by which means comparisons in pressure can be observed ; thoracoscopy, and myclography with contrast liquid media or air. Transthoracic puncture of these cysts involves the danger of meningitis and, in this instance, the added hazard of tuberculous meningitis . These cysts usually have large patent communications with the subarachnoid space and fill readily with air, making it a simple matter to establish a definite diagnosis . The most efficacious and benign procedure is that of performing air myclography . This was first described in this connection by Cross' and subsequently by Laitinen .s With the patient in the left lateral decubitus position a total of 80 cc . of spinal fluid was removed via lumbar puncture and replaced by air . Films as .shown in Fig. 2C were obtained . The choice between conservative therapy or operative interference was considered . Sears' reported fifteen cases of this type, nine of which were treated surgically . There were four deaths in this group . Surgical correction of these lesions is difficult and is frequently complicated by recurrence of the lesion, leakage of the spinal fluid into the pleural space or meningitis . Baker and Curtis-- report successful treatment by resection of the lesion in six patients with nteningoceles of this type . They believe improved results from surgery in recent years are due to preoperative diagnosis, modern surgical technic and suitable use of antibiotics . They believe that when the lesion is diagnosed preoperatively and the patient is asymptomatic, a conservative course should be followed . FEBRUARY,1t57

The following factors should influence the selection of therapy for the individual patient : (1) progressive enlargement of the lesion . (2) ncurologiru symptoms, (3) encroachment upon surrounding structuresvascular, pulmonary or osseous", (4) operative risk, and (5) associated ptrlmonic disease . Because this patient was considered a poor operative risk, and in view of the mildness of symptoms and the presence of active tuberculous pulmonary disease, conservative therapy was instituted, consisting of antitubercutous treatment, close clinical and roentgenologic follow-up, and symptomatic relief . Several months have elapsed during which this patient has been observed periodically in the out patient department . She continues to be free from symptoms except for intermittent nuchal headaches of moderate degree and low back pain, both of which respond to mild analgesics . No roentgenologic change in the lesion has been detected . COMMENTS

The controversy over the origin of bone lesions in neurofibromatosis has many facets, Are these lesions part of multiple congenital mesodermal anomalies or are neurofibromas etodermal in origin initiating bony change by trophic or pressure change? If these lesions are due to expanding neurofibromas, arc the neurofibromas intrinsic to bone or in juxtaposition to bone? This raises the controversial question of intrinsic . nerve supply to bone . Is there an associated defect in reabsorption of phosphorus by the renal tubules, as suggested by S\vann?s Holts has presented an excellent discussion on

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Intrathoracic Meningocele-Moyer

this subject . Suffice it to say here that kvphoscoliosis is the most frequent osseous abnormality seen in conjunction with neurofibromatosis ; that associated meningoceles are almost always discovered in middle life ; that they occur on the convex side of the scoliosis ; and that they are associated with vertebral defects at the site of communication with the spinal canal . This would make it appear that they are simply meningeal hernias occurring because of preexisting osseous lesions. While these lesions are rare, their presence should be suspected in all cases of posterior thoracic densities associated with vertebral anomalies, especially in the presence of neurofibromatosis .

Acknowledgment. We wish to thank Paul A . Bishop, M .D ., Director of Roentgenology Department, Pennsylvania Hospital, and Mrs . Sophie .I . Kocielski for their counsel and assistance in this undertaking . REFERENCES

Meningokele in Brustraum unter dens Bilde eines Intrathorakelen Rundschattens . Rontgen,praxis,

1 . POHL, R .

5 : 747, 1933 . 2 . LAITINEN, H . and TuTuNEN, M, Diagnosis of intrathoracic meningocele . Di.r. of Chest, 27 : 547, 1955 . 3 . CROSS, G . 0., REAVIS, J . R . and SAUNDERS, W . E . Lateral intrathoracic meningocele . J. .Neurosurg ., 6 : 423, 1949 . 4 . SEARS, A . D., CLAYTON, R . S . and SEEREL, E . Intra-

5. SUMMARY

A case of intrathoracic meningocele associated with neurofibromatosis and active pulmonary tuberculosis is presented . The diagnostic measures, probable pathogenesis and therapeutic approaches in this disorder are briefly discussed .

et al .

thoracic meningocele not associated with neurofibromata . J. Thoracic Surg ., 26 : 101, 1953 . CECLIA, P . Intrathoracic meningocele-excision with three and one-half year follow-up . J. Thoracic .Sung .,

23 : 283, 1952 . 6 . HOLT, J . F. and WRtOIIT, E . M . The radiologic features of neurofibromatosis . Radiology, 51 : 647, 1948 . 7 . BAKER, I M. and CURTIS, M . Intrathoracic meningocele . West . J. Surg ., 61 : 209, 1953 . 8 . SWANN, G . F . Pathogenesis of bone lesions in neurofibromatosis. Brit . J. Radiol., 27, 623, 1954 .

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