CASE REPORTS REPORT OF A CASE OF NEURENTERIC CYST WITH ASSOCIATED CHRONIC MENINGITIS AND HYDROCEPHALUS*

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CASE REPORTS NEURENTERIC CYSTS REPORT OF A CASE OF NEURENTERIC CYST WITH ASSOCIATED CHRONIC MENINGITIS AND HYDROCEPHALUS* LCDR. FREDERICK E. JACKSON (MC), U.S.N. Department of Neurological Surgery, U.

1 CASE REPORTS NEURENTERIC CYSTS REPORT OF A CASE OF NEURENTERIC CYST WITH ASSOCIATED CHRONIC MENINGITIS AND HYDROCEPHALUS* LCDR. FREDERICK E. JACKSON (MC), U.S.N. Department of Neurological Surgery, U. S. Naval Hospital, San Diego, California Mediastinal cysts evidencing gastric epithelium were first described by Staehelin-Burckhardt. 6 By 1947 ~5 case reports had been collected by Davis and Salkin. 1 A total of only 3~ cases had been reported by 195~ when Williams and Johnson 7 added their case report. Since that time at least 6 more cases have been reported. 2-~ The following case is noteworthy because of the preoperative delineation of this cyst by radioopaque material (Hypaque) and because of the associated conditions of chronic meningitis and hydrocephalus. CASE REPORT A 7-month-old white male infant was first admitted on April 17, 1959 with a history of recurrent cystic swelling in the anterior part of the neck on the left side, associated with fever, meningismus and irritability. The mother had enjoyed good health during her pregnancy, and there were no complications incident to the delivery. The child appeared normal at birth. He developed normally until the age of 5 months, at which time a cystic mass was first discovered in the left anterior cervical area. At that time it was also noted that the child had large supraclavicular and inguinal lymph nodes. The adenopathy, and also the cyst, quickly decreased in size after a course of antibiotics, but ~ weeks later the cervical cyst reappeared. Once again he was treated with antibiotics, this time with slow resolution of the cervical adenopathy and of the swelling in the neck. Three weeks prior to admission cervical adenopathy was noted again, but on this occasion the child did not respond to antibiotics and there commenced a period of fever, diarrhea, nausea and vomiting which required hospitalization. Examination. Nuchal rigidity was noted, and lumbar puncture revealed 85 white blood cells per ram., all polymorphonuclear. On culture, the organism proved to be Streptococcusfecalis. Course. Treatment was initiated with Chloromycetin, penicillin and streptomycin with slow resolution of the fever and meningeal signs. The patient was discharged from the hospital, but within a week, the meningeal signs recurred, and he was re-admitted. * The opinions expressed in this article are those of the author and not necessarily those of the United States Navy. (Received for publication September ~27, 1960) 678 2nd Admission. Examination revealed the child to be undernourished and irritable. The fontanelles were tense and bulging, the veins of the scalp were distended, and the neck was stiff. There was no papilledema. The head was disproportionately large in comparison with the rest of tile body, the circumference of the head being at the 80 percentile for his age, although his body weight was at only the 8 percentile. Motor power, sensation and reflexes were normal for his age. There was a fluctuant, cystic mass, 3 by 3 cm. in size, presenting in the left anterior cervical region. Lumbar puncture yielded clear fluid with a cell count of 99, of which 74 were polymorphonuclear cells and ~5 were lymphocytes. The cerebrospinal fluid protein was 38 rag. per cent. Culture of the cerebrospinal fluid again produced Streptococcus fecalis. Plain roentgenograms of the chest revealed segmental abnormalities of the cervical spine from C3 through C7, and of the thoracic spine from T1 through T5, involving primarily the bodies of these vertebrae, and, to a lesser extent, the posterior arches as well. The trachea and bronchi were displaced markedly from left to right (Fig. 1). On April ~3, 1959, the fluctuant cyst in the neck was aspirated, and 14 ml. of "coffee-ground" material were obtained. Two ml. of Hypaque were instilled which delineated nicely the outline of the cervical cyst (Figs. and 3). A delayed roentgenogram was taken 6 hours after the instillation of Hypaque into the cyst in the neck, and it was seen that the contrast material had descended from the left anterior cervical cyst into a more inferior cyst lying on the anterior portion of the bodies of the 1st to the 4th thoracic vertebra. Residual Hypaque was still apparent in the upper cyst, into which the Hypaque had been instilled originally. Figs. 4 and 5 show the descent of the Hypaque from the upper lobule of the cyst to the main portion of the cyst, lying within the mediastinum. A small amount of Hypaque had been absorbed, and excreted by the kidneys, and is visible in the bladder (Fig. 4). Fig. 5 is a lateral view demonstrating the position of the mediastinal cyst in relation to the anterior portion of the centrum of the upper thoracic vertebrae. Operation. On May 1, 1959, under general endotracheal anesthesia, through a low cervical incision, the cystic mass in the left side of the neck was exposed, and by sharp dissection its connection with the mediastinal cyst was established. The cyst in the neck was then excised completely and the mediastinal cyst was renmved by a combination of blunt and sharp dissection. Its removal was effeeted quite easily. The mediastinal cyst

T1 to TS, with dysraphia of the centra of involved vertebrae.

2 NEURENTERIC CYSTS 679 FIG. 1. Roentgenogram of chest revealing segmental abnormalities of cervical spine from C3 through C7 and of thoracic spine from T1 to TS, with dysraphia of the centra of involved vertebrae. The tracheal air shadow is displaced markedly from left to right. A soft-tissue density may be seen in the left side of the neck. FIGs. ~ and 3. Roentgenograms revealing Hypaque outlining cyst in upper part of neck on left side. Anteroposterior (left) and lateral (right) views.

A small amount of Hypaque evidently has been absorbed, and is visible in the urinary bladder.

3 680 FREDERICK E. JACKSON FIGS. 4 and 5. (Left) Delayed roentgenogram revealing filling of mediastinal cyst by Hypaque that has descended from superior lobule of cyst in neck. A small amount of Hypaque evidently has been absorbed, and is visible in the urinary bladder. (Right) Lateral view of mediastinal cyst, demonstrating its relation to anterior surface of upper thoracic vertebrae. Fro. 6. Cross section of wall of superior lobule of cyst demonstrating nevus-like cells with abundant pigment lining the wall of the cyst. Hematoxylin and eosin, X40.

4 NEURENTERIC CYSTS 681 FIG. 7. Epithelial lining of mediastinal portion of cyst. Hematoxylin and eosin, )<40. was attached by a pedicle to the centrum of the ~nd thoracic vertebra, and through this pedicle there ran a small sinus tract which was explored with a very fine probe. The sinus was seen to extend to the malformed centrum of the ~nd thoracic vertebra. Spinal fluid was not encountered within the sinus. Pathological Report. The superior cyst which had presented in the neck was composed histologically of a matting of nevus-like cells in which a great amount of pigment was present. Several strands of nerves were seen, and in one portion of the specimen there was a ganglionic body in which ganglion cells and other nerve elements were present (Fig. 6). The mediastinal portion of the cyst is shown in Fig. 7. Postoperative Course. The patient did very well, although his hydrocephalus continued to remain manifest. Therefore, on May 8, 1959, a Pudenz-Heyer type of ventriculo-atrial shunt was performed for correction of the hydrocephalus. This functioned well for several weeks, but then hecame occluded and was revised on June ~, Since that time the patient has continued to do very well, showing a gratifying decrease in circumference of the head and with no further attacks of meningitis. DISCUSSION Evidence is accumulating u-5 that intrathoracic mediastinal gastric cysts are often associated with defects in the centra of the lower cervical or upper thoracic vertebrae. Neuhauser's 4 second case revealed a pedicle which passed from the cyst through a defect in the vertebral bodies and extended to merge with the cervical cord. The stalk contained elements of neural tissue as well as smooth muscle. In Case 3 of Neuhauser's series there was actually a communication from the subarachnoid space to the mediastinal cyst. In our case there was not only a defect in the centra of the lower cervical and upper thoracic vertebrae, but also a sinus tract between the cyst and the ~nd thoracic vertebra, with an associated chronic recurrent Streptococcus fecalis meningitis. Staehelin-Burckhardt ~ pointed out that at an early stage in embryogenesis the notochord lies in juxtaposition to the primitive entoderm. The notochordal plate invaginates and subsequently forms the neural canal. It is quite conceivable that small portions of the surrounding entoderm may retain their apposition to the developing neural ectoderm. Later in the course of embryogenesis, the interposition of the mesoderm forms the covering of the spinal cord, including the bony spinal column. It seems logical, therefore, to explain the mesodermal separation, as manifested by the defects in the anterior portion of the centra of the involved vertebrae, as being caused by a persistent neuroectodermal-entodermal communication or juxta-

5 68~ FREDERICK E. JACKSON position. To support this concept of pathogenesis, the presence of an obvious communication between the mediastinal cyst and the spinal column or spinal subarachnoid space has been demonstrated in other cases and in this case. The anterior defects in the centra of the involved vertebrae are not just "incidental findings," but reflect the dysraphia caused by the persistent entodermal-ectodermal communication. SUMMARY A case of a mediastinal cyst containing gastric epithelium, accompanied by chronic recurrent Streptococcus fecalis meningitis and hydrocephalus, is presented. At operation, a sinus tract was seen to communicate from the cyst to the ~nd thoracic vertebra. The cyst and tract were removed successfully and the hydrocephalus subsequently was treated successfully by a Pudenztteyer vcntriculo-auricu]ostomy. REFERENCES 1. DAws, E. W., and SALKIN, D. Intrathoracic gastric cysts. J. Amer. reed. Ass., 194~7, 135: ~18-~o~1. ~. EHLER, A. A., and ATWELL, S. Gastric cyst of the mediastiuum. J. thoracic. Surg., 1948, 17: 809-8~0. S. MoosE, J. A., and JAHNKE, E. J., JR. Mediastinal gastric cyst. Report of an unusual case. J. Dis. Child., 1957, 94: 19~ NEUHAUSER, E. B. D., HARRIS, G. B. C., and BERRETT, A. Roentgenographic features of neurenteric cysts. Amer. J. Roentgenol., 1958, 79: ~$5-~ OVERTON, R. C., JR., and OVERSTREET, J.W. Peptic bronchitis: case report of mediastinal gastrogenic cyst with bronchial communication. Amer. Surg., 1958, 24: STAEHELIN-BURCKHARDT, A. Ueber eine mit Magenschleimhaut versehene Cyste des Oesophagus. Arch. Verdau]cr., 1909, 15: ~VILLIAMS, M. H., and Jo~'soN, J.F. Mediastinal gastric cyst. Successful excision in an eight-week-old infant. Arch. Surg., Chicago, 195~, 64:

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