Paraesophageal ciliated cysts of the mediastinum

Paraesophageal Ciliated the Mediastinum REPORT

OF FOUR

CASES

AND

REVIEW

OF THE

Cysts of ENGLISH

LITERATURE

LT. COL. G. F. RUMER, M.c.,U.S.A.,COL. S. W. FRENCH, III,M.c.,U.S.A.AND CAPT. RICHARD

C. FROEDE, M.c.,u.s.A.F.. Sun Francisco, California

From tbe Tboracic and CardiovascuZaT Surgical Service and Patbology Service, Letterman Army Hospital, San Francisco, Calijornia.

they shouId be IaheIIed as bronchogenic or paraesophageal wouId appear to depend on their Iocation at operation or postmortem examination rather than on their histoIogica1 appearance which can be identica1. Another factor which supports this theory is that at the time when the trachea and esophagus separate (4 to 5 mm. stage of the embryo), they are both Iined with ciIiated coIumnar epitheIium. If at this stage tissue fragments are sequestrated this wouId account for the common components of these cysts. Moreover, the eIongation of the esophagus which occurs as the heart descends accounts for the frequent Iocation of paraesophageal cysts in the Iower third of the esophagus.

ciliated cysts of the mediascomprise an increasingIy encountered group of congenita1 abnormaIities. In 1940, Heuer and Andrus [r] were abIe to collect onIy twenty-five cases of this entity, to which they added one case of their own. Creech and DeBakey [23in 1954 were abIe to coIIect fortynine cases from the Iiterature incIuding those reported by Heuer and Andrus, to which they added two cases of their own. A further review of the EngIish Iiterature since Creech and DeBakey’s articIe reveaIs two more cases. The first was reported by Minor [3]who treated the cyst by marsupiaIization to the esophagus with an exceIIent recovery. The second case was reported by Desforges, Madison, and Starkey [4], and the diagnosis of paraesophagea1 cyst was estabIished as the cause of a bIoody pIeura1 effusion. The cyst was a Iarge 20 cm. Iesion attached to the Iower esophagus and heId 700 cc. of typical contents of this type of cyst. Recovery foIIowed operative removal. It is the purpose of this paper to report four more cases of this congenita1 abnormaIity and to discuss the present concepts of this interesting cIinica1 entity. ARAESOPHAGEAL

P tinum

HISTOLOGY

Bronchogenic cysts are commonIy Iocated at or just beIow the bifurcation of the trachea deep within the mediastinum. They may occur in chains or may be singIe and muItiIocuIated. These cysts usuaIIy do not communicate with any other organ, are thin waIIed and of various sizes and contain either a cIear, geIatinous or thick miIky materia1. MicroscopicaIIy, the cysts are Iined by pseudostratified ciIiated coIumnar epitheIium and in some cases isIands of stratified squamous epitheIium are present. The waI1 of the cyst is made up of fibrous connective tissue and may contain cartiIage, smooth muscIe, mucous gIands and nerve trunks. The stratified squamous epithelium is indicative of a Iater budding process after the Iining epitheIium of the esophagea1 anIage has begun its transition from pseudostratified ciIiated coIumnar to stratified squamous epitheIium. For a comprehensive review of the

EMBRYOLOGY

Some authors, notably Maier [5], beIieve that bronchogenic cysts and paraesophagea1 cysts are one and the same and we agree with this theory. The trachea and esophagus arise from a common anlage, and this fact aIong with the histoIogica1 simiIarity of the two types of cysts wouId certainIy support this theory. Whether American Journal of Surgery. Volume 96.

September,

19.58

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ParaesophageaI

CiIiated

Cysts

of Mediastinum

FIG. I. Case I. This IiIm demonstrates the we11 rounded lesion in the cardiophrenic angIe. On the IateraI projectiou this lesion was in a posterior position.

histologv of this Iesion, the reader is referred to Anderscin [6], Schlumherger [7], and Ackerman [S]. In our experience any cysts of this type below the carinal region have been closeIy associated with the esophagus, and we have catled them paraesophagea1 ciliated epitheIia1 cysts. These do not communicate with the esophagus except in those rare instances when previous infection has created a fistuIous tract into the esophagus. They are Iocated within the muscularis of the esophagus necessitating the spIitting of the muscIe layers to effect their remova1. In three of the four cases reported in this articIe, the cysts were easiIy shelled out and the muscuIaris closed to eliminate the defect. In Case IV the cyst couId not be shelIed out due to previous infection and had t.o be removed piecemea1. Despite the infection it did not communicate with the esophagus. These paraesophagea1 cysts contain the same type of materia1 and have the same histoIogica1 appearance as bronchogenic cysts. For these reasons, we propose that these cysts be caIIed bronchoesophagea1 cysts rather than bronchogenic or paraesophagea1 cysts. Enterogenous or gastroenterogenous cysts (often caIIed duplication cysts) are an entirely different entity. These contain a11 the eIements normally found in the waIIs of the gastrointestina1 tract. They are thought to be either buds pinched off from the deveIoping foregut or failure of coaIescence of the vacuoIes formed during the deveIopment of the foregut. The reader is referred to exceIIent reviews of this

FIG. 2. Cnsc I. The photomicrograph shows a svction of the wall of the cyst which is lined Py a pseudostrntificd columnar cpithrlium composed ot Interlacing bundles of dense fibrous connective tissue and smooth musclr. (Note cystic structure with cuboid:~l t~pithclium lining. I

subject b)- Lindquist and WuIff [9], Olenik Tandatnick [ ro], and Boyd [I I].

and

SYMPTOMATOLOGY

Bronchoesophageal cysts are often asymptomatic and are usuaIIy found accidentaIIy on a routine chest pIate or at postmortem examination. Such a cyst in chiIdhood, if Iocated in severe rethe carinal region, may produce spiratory distress [2]. Cysts in cIose proximity to the esophagus may produce symptoms such as dysphagia, a sensation of substerna fuIIness and even cardiac manifestations. Creech and DeBakey [2] emphasized the occurrences of cardiac irreguIarities in association with this condition and the disappearance of these symptoms foIIowing the remova of the Iesion. Other writers have reported the association of other types of mediastina1 Iesions with cardiac irreguIarities [12,14,15]. In the four cases reported herein, two patients had symptoms referable to the esophagus and one of these two had some cardiac irreguIarity which she referred to as “paIpitations.” This patient obtained compIete relief of both compIaints foIIowing surgery. The 421

Rumer,

French

and

Froede

FIG. 4. Case III. Posteroanterior esophagram demonstrating the Iesion on the right with indentation of the esophagus. referabIe to her CardiovascuIar system. X-ray studies as early as 1951 reveaIed a partiaIIy calcified posterior mediastinal mass. (Fig. 3.) Thoracotomy was recommended and accepted by the patient. At surgery, an intimately adherent partiaIIy calcified mass was removed from the retrocardiac region of the esophagus. It stripped with some difficuIty but there was no connection with the esophagus at any place. Her postoperative course was uneventful.

FIG. 3. Case II. Posteroanterior esophagram demonstrating a Iesion Iying just above the diaphragm and extending into both sides of the thorax in an extrapleural position.

other patient with dysphagia aIone compIete reIief of the symptoms. CASE

aIso had

REPORTS

CASE III. A forty-eight year old white woman was admitted to the hospita1 in 1956 for study of possibIe Raynaud’s disease. No definite evidence of this disease was found but during the course of her hospita1 work-up, a mass was noted in the posterior mediastinum. (Fig. 4.) On the basis of barium swaIIow test, a diagnosis of a paraesophagea1 mass was made and thoracotomy recommended. At surgery a ciIiated paraesophagea1 cyst (Fig. 5) was found and removed with no difficulty. Her postoperative course was benign except for mild pain in the right arm and shouIder secondary to a trigger point in the upper aspect of the scar. This responded niceIy to physiotherapy and IocaI procaine bIocks.

CASE I. A thirty-five year oId white man was admitted to the hospita1 in 1955 with a mass in the Ieft side of the pIeura1 cavity which had first been noted in 1944. The onIy studies done prior to this time were skin tests which were positive for coccidioidomycosis and histopIasmosis. Studies during this hospita1 admission revealed a mass in the left side of the chest in the posterior mediastinum overIying the body of TII. (Fig. I.) Barium swaIIow test reveaIed a norma mucosa1 pattern of the esophagus. At surgery a ciIiated paraesophagea1 cyst was found and removed with no difEcuIty. (Fig. 2.) The patient had a postoperative hemothorax of undetermined origin which cIeared up on conservative therapy. He was returned to duty having a cIear chest fiIm and no symptoms.

CASE IV. A thirty-five year oId white woman was transferred to the hospital in 1957 with a diagnosis of either a Ieiomyoma of the esophagus or a paraesophageal ciIiated cyst. The diagnosis had been established by a barium swaIIow test (Fig. 6) which had been made because the patient had some vague symptoms of dysphagia and “paIpitations.” The cardiac symptoms were some substernal pain and

CASE II. A thirty-eight year oId white woman was admitted to the hospital in 1954 for study reIative to a mediastina1 mass. She had had some troubIe swaIIowing for the preceding four years, manifested by a sensation of food sticking just before entering the stomach. She had no symptoms 422

ParaesophageaI

CiIiated

FIG. 6. Case demonstrating the esophagus.

FIG. 5. Case III. The photomicrograph shows the wall of the cyst lined by pseudostratifred coIumnar epithehum which demonstrated considerable degeneration. The wall is composed of interlacing bundIes of smooth muscle and dense fibrous connective tissue and contains a large cartilaginous plate.

I\‘. Left anterior oblique csophagram the lesion lying behind the hcsrt :tnd

that the esophagea1 mucosaI pattern is intact, indicating an origin other than from the mucosa of the esophagus. It is our beIief that a11 of these Iesions should be removed surgicaIIy for it is diffIcuIt if not impossible to differentiate them from others occurring in the mediastinum which may be maIignant. The accepted method of treatment is compIete surgica1 removal of the cyst. In many cases these can be sheIIed out easily, but if infection has previousIy been present the) may have to be removed piecemeal. In patients who demonstrate cardiac irreguIarities symptoms have either disappeared or markedI>- improved after remova of the cyst and the dysphagia has been relieved.

palpitation

which were more noticeabIe at the time of elating. Thoracotomy was performed and a paraesophageal cyst removed with some diffLxIty due to a previous infection in the cyst. Despite the infection there was no communication with the esophagus. Her postoperative course was very benign and all her symptoms of dysphagia and palpitation disappeared following surgery.

COMMENTS

Bronchoesophageal cysts are an interesting clinical entity which are being diagnosed and treated more frequentIy than ever before. They

are most frequentIy diagnosed as an incidenta finding on a chest fiIm since many of them are asymptomatic. If symptomatic, the usua1 symptoms are dysphagia, usuaIIy miId in degree, and in a few cases, cardiac irreguIarities have been described as a result of the presence of these cysts in cIose proximity to the heart. As shown in Figures 3 and 6, the retrocardiac position certainIS symptomatoIogy. these Iesions can

Cysts of Mediastinum

SUMMARY I. Four cases of paraesophageal epitheIiaI cysts are presented. 2. This makes a tota of fifty-five cases of this cIinica1 entity in the Iiterature. 3. It is our beIief that these Iesions and those caIIed bronchogenic cysts are one and the same entity, being different onIy in their Iocation and having the same histologica pattern and embryoIogica1 origin. We beIieve that these two

couId account for this On barium swaIIow test be demonstrated and show

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REFERENCES ANDRUS, W. The surgery of media&& tumors. Am. J. Surg., 50: 162, 1940. 2. CREECH, O., JR. and DEBAKEY, M. Ciliated epithelial cysts of the esophagus associated with cardiac abnormalities. J. Tboracic Surg., 28: 64-77, 1954. 3. MINOR, G. R. hlediastinal cyst treated by marsupialization to the esophagus. Ann. Surg., 139: G.

Froede

8. ACKERMAN, L. V. Textbook of Surgical Pathology St. Louis, 1953. C. V. Mosby Co. 9. LINDQUIST, N. and WULFF, H. Mediastinal enterocystoma: report of case in 7-month-old chiId with impending suffocation, operation and recovery. J. Tboracic Surg., 16: 468-476, 1947. IO. OLENIK, J. and TANDATNICK, J. Congenital mediastinal cysts of foregut origin. Am. J. Dis. Child., 71: 46&476, 1946, I I. BOYD. D. Lesions of the suoerior mediastinum: diagnosis and treatment. S.‘Clin. Nortb America,

types of cys,t.s should be referred to as bronchoesophageal cysts. 4. Treatment is surgical removal and shouId be recommended to rule out a possible malignancy. I. HELER,

and

J. and

33: 834-837s

1953. influence of mcdiastinal tumors on the production of cardiac arrhythmias. Texas State J. Med., 28: 361-363, 1932.

12. ADAMSOK, W. The

13. SAUERBRCCH, F. and FICK. W. Operative beseitigung einer kongenitalen cyste ber speiserohre. Zentralbl. f. Cbir., 58: 2938.-2941, rg3r. 14. DONAVAN. hl. A. Pain of unusual duration due to progressive coronary occlusion with associated mediastina1 tumor. Am. Heart J., 32: 786-793,

230-235, ‘954. 4. DESFORCES, G., hlADISON, W. and STARKEY, G. Bloody pleural effusion caused by a bronchogenic cyst. J. Tboracic Surg., 30: 698-701, 1955. 5. MAIER, II. Bronchogenic cysts of the mediastinum. Ann. Surg., 127: 47&50;, 1948. 6. ANDERSON, W. A. D. Textbook of Pathology, 3rd ed. St. Louis, 1957. C. V. Mosby Co. 7. SCHLUMBERGER, H. Atlas of tumor pathology (tumors of the mediastinum), Sect. v, Fascicle I 8. 1951. Armed Forces Institute of Pathology.

1946.

15. RAWLS. W. and AMCOKA, V. An unusual case of paroxysmal auricular fibrillation due to mediastinal malignant Iymphoma. Am. Heart J., 41: 311-315,

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1951.