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INTRADIAPHRAGMATIC CYST: A RARE ENTITY
INTRADIAPHRAGMATIC CYST: A RARE E N T I T Y Benjamin L. Aaron, Lieutenant
Commander,
MC, USN, Oakland,
Calif.
I
N a world of ever-increasing medical communication and knowledge, primary intradiaphragmatic cysts remain a rare pathologic entity. The actual number reported to date is uncertain, in that there are some conflicting figures given by various authors. Kessler and Maier 1 collected 7 cases of such cysts from the world literature to which they added an eighth, a bronchial cyst. Buddington, 2 in 1957, described the ninth case of true intradiaphragmatic cyst, the third bronchial. In 1959, Felder 3 added a tenth cyst (fibrous). Later the same year, Kaganskii 4 reported the case of another fibrous intradiaphragmatic cyst, the eleventh; he noted, however, that the records of the Postgraduate Surgical Clinic of the Kirov Military Medical Academy contained 7 other cysts of the diaphragm but failed to elaborate with respect to detail or type. Balas and Kalmar, 5 in 1962, accounted for the fourth bronchial cyst, making a total of 12 intradiaphragmatic. In contrast to the above resume of cases, Ochsner6 stated that, until 1960, 18 cysts of the diaphragm had been reported: 10 mesothelial, 5 bronchial, and 3 fibrous-walled. He further stated that 3 or 4 of the mesothelial type actually may have been pleural cysts of the diaphragm rather than intradiaphragmatic. His references were not available to cross-check with the above authors' cases; therefore, the exact number remains obscure at this time. The occasion for this report is the removal of a bronchial cyst from the left diaphragm of a 21-year-old female, the particulars of which follow. CASE REPORT A 21-year-old Caucasian female had had recurrent lower anterior chest pain on the left side for approximately 8 months prior to admission to the U. S. Naval Hospital, Oak land, Calif., on Aug. 19, 1963. She denied other respiratory symptoms. Routine x-ray exami nation of the chest at the time of delivery of her infant, 3 months prior to admission, re vealed a lesion on the left diaphragm anterior to the esophageal hiatus, which demonstrated no continuity with the gastrointestinal tract on x-ray study of an upper gastrointestinal series and barium enema. Complete physical examination and routine laboratory studies were within normal limits. X-ray examination of the chest was negative except for a faint homogeneous density seen just above the medial portion of the hemidiaphragm in the paraesophageal region (Fig. 1, A and B). From the Thoracic and Cardiovascular Surgery Branch, Surgical Service, U. S. Naval Hospital, Oakland, Calif. The opinions or assertions contained herein are the private ones of the author and are not to be construed as official or as necessarily reflecting the views of the Medical Department of the Navy or of the Naval Service a t large. Received for publication June 26, 1964. 531
532
AARON
J. Thoracic and Cardiovas. Surg.
0 P Ste 0a r View sh alateral t e v.ew A ^ BJ^ shows ^ S the ^ ^sanfe ^ ^ ^ ^^ t y tne clX°r c^a t s^ou ^ % ^ " e?t f l f ? f ?^ i ^T I ,^ ^ ^'*> * ™°™ ^ (
^ ""SffiSS?^^
the
Vol. 49, No. 3
March, 1965
INTRADIAPHRAGMATIC CYST
533
On Aug. 21, 1963, a left thoraeotomy was performed and a multiloculated yellow cyst, 5 cm. by 3 cm. by 3 cm., containing gray-yellow mucopurulent material, was found imbedded in the muscular diaphragm, 3 cm. anterior and to the left of the esophageal hiatus. The lesion was excised and the rent in the diaphragm was closed in 2 layers without difficulty. The patient's postoperative course was smooth and uncomplicated and she was discharged on the ninth day following the operation. Culture of the cystic fluid showed no bacterial growth in 72 hours. Histologie examination demonstrated cystic spaces lined by pseudostratified, columnar, ciliated epithelium, with underlying mucous glands and plates of cartilage arranged in hap hazard fashion (Fig. 2, A and B). DISCUSSION
Authors reporting on this entity specifically refer to its mtfradiaphragmatic location, since other cystic structures, such as enteric, renal, and pericardial, occur on either side of and in close proximity to the diaphragm, but are not integral to the formation of the diaphragm. 7 Plausible, but certainly theoretical, explanations have been advanced for each of the 3 types of cysts described in this location. Mesothelial cysts are believed to result from an invagination and pinching off of a portion of the advancing edge of the pleuroperitoneal and pleuropericardial membranes prior to their fusion with the septum transversum during the fourth to sixth week of fetal life.8 Bronchial cysts are most likely due to a sequestration of primitive lung-bud tissue caught up in the same fusion process of the components of the future diaphragm. Fibrous-walled cysts can be explained best as degenerative mesothelial or bron chial types wherein all distinctive elements, especially the lining membrane, are destroyed or replaced by fibrous tissue, presumably secondary to chronic inflammation or spontaneous regression. Bronchial cysts occur more often elsewhere, the most frequent site being the upper posterior mediastinum near the tracheal bifurcation. Willis 9 described such sequestrations of primitive lung anlage as "ectopias,"'stating that they are usually unilocular, thin-walled, with mucoid contents, and lined by ciliated pseudostratified columnar epithelium of a respiratory type—the walls contain ing bundles of smooth muscle, mucous and mixed glands, and nodules and plates of cartilage. In the case presented here, the cyst was truly intradiaphragmatic since the pleura was incised to remove it, following which the peritoneal surface of the diaphragm remained intact. This cyst or "ectopia" was multiloculated, as were others reported, and it contained all elements of bronchial structure. As with other cysts reported, the symptoms were mild and rather nonspecific; op erative removal was relatively simple; and postoperative recovery was uncom plicated, as in other reported cases. SUMMARY
By the best count available, the cyst reported herein is the thirteenth intradiaphragmatic and fifth bronchial cyst of the diaphragm recorded. A brief discussion of the origin and development of the 3 types of intradiaphragmatic
AARON
534
J. Thoracic and Cardiovas. Surg.
cysts is presented. This group of lesions constitutes a rarely encountered patho logic entity and it is of interest only to exclude more frequently encountered malignant and benign tumors of the diaphragm and adjacent structures. REFERENCES 1. Kessler, H. J., and Maier, H. C : Intradiaphragmatic Cysts, J . THORACIC SURG. 30: 159, 1955. 2. Buddington, W. T.: Intradiaphragmatic Cyst: Ninth Reported Case, New England J . Med. 257: 613, 1957. 3. Felder, M. E . : Intradiaphragmatic Cyst, Am. J . Surg. 98: 95, 1959. 4. Kaganskii, V. E . : Sluckae kisty diafragmy (Case of Diaphragmatic Cyst), Voprosy Onhologhii (Leningrad) 5: 368, 1959. 5. Balas, A., and Kalmar, M.: On Bronchial Cysts of the Diaphragm, Thoraxchirurgie 9: 513, 1962. 6. Ochsner, A. O., and Ochsner, A. O., J r . : Tumors of the Diaphragm in Diagnosis and Treat ment of Tumors of the Chest, edited by D. M. Spain, New York, 1960, Grune & Stratton, pp. 240-244. 7. Abell, M. R.: Mediastinal Cysts, Arch. Path. 6 1 : 360, 1956. 8. Clough, D. M., and Bierne, M. T.: Benign Mesotlielial Cyst of the Diaphragm, J . THORACIC SURG. 29:
212,
1955.
9. Willis, R. A.: The Borderline of Embryology and Pathology, London, 1958, Butterworth & Co., Ltd., pp. 41, 313-314.
BOARD OF THORACIC SURGERY E X A M I N A T I O N S WRITTEN EXAMINATION : Various centers throughout the country, September 10, 1965. Final date for application is June 1, 1965. ORAL EXAMINATION: October, 1965. Final date for filing application is June 1, 1965. Please address all communications to Board of Thoracic Surgery, Inc., 1151 Taylor Avenue, Detroit, Michigan 48202.
Commander,
MC, USN, Oakland,
Calif.
I
N a world of ever-increasing medical communication and knowledge, primary intradiaphragmatic cysts remain a rare pathologic entity. The actual number reported to date is uncertain, in that there are some conflicting figures given by various authors. Kessler and Maier 1 collected 7 cases of such cysts from the world literature to which they added an eighth, a bronchial cyst. Buddington, 2 in 1957, described the ninth case of true intradiaphragmatic cyst, the third bronchial. In 1959, Felder 3 added a tenth cyst (fibrous). Later the same year, Kaganskii 4 reported the case of another fibrous intradiaphragmatic cyst, the eleventh; he noted, however, that the records of the Postgraduate Surgical Clinic of the Kirov Military Medical Academy contained 7 other cysts of the diaphragm but failed to elaborate with respect to detail or type. Balas and Kalmar, 5 in 1962, accounted for the fourth bronchial cyst, making a total of 12 intradiaphragmatic. In contrast to the above resume of cases, Ochsner6 stated that, until 1960, 18 cysts of the diaphragm had been reported: 10 mesothelial, 5 bronchial, and 3 fibrous-walled. He further stated that 3 or 4 of the mesothelial type actually may have been pleural cysts of the diaphragm rather than intradiaphragmatic. His references were not available to cross-check with the above authors' cases; therefore, the exact number remains obscure at this time. The occasion for this report is the removal of a bronchial cyst from the left diaphragm of a 21-year-old female, the particulars of which follow. CASE REPORT A 21-year-old Caucasian female had had recurrent lower anterior chest pain on the left side for approximately 8 months prior to admission to the U. S. Naval Hospital, Oak land, Calif., on Aug. 19, 1963. She denied other respiratory symptoms. Routine x-ray exami nation of the chest at the time of delivery of her infant, 3 months prior to admission, re vealed a lesion on the left diaphragm anterior to the esophageal hiatus, which demonstrated no continuity with the gastrointestinal tract on x-ray study of an upper gastrointestinal series and barium enema. Complete physical examination and routine laboratory studies were within normal limits. X-ray examination of the chest was negative except for a faint homogeneous density seen just above the medial portion of the hemidiaphragm in the paraesophageal region (Fig. 1, A and B). From the Thoracic and Cardiovascular Surgery Branch, Surgical Service, U. S. Naval Hospital, Oakland, Calif. The opinions or assertions contained herein are the private ones of the author and are not to be construed as official or as necessarily reflecting the views of the Medical Department of the Navy or of the Naval Service a t large. Received for publication June 26, 1964. 531
532
AARON
J. Thoracic and Cardiovas. Surg.
0 P Ste 0a r View sh alateral t e v.ew A ^ BJ^ shows ^ S the ^ ^sanfe ^ ^ ^ ^^ t y tne clX°r c^a t s^ou ^ % ^ " e?t f l f ? f ?^ i ^T I ,^ ^ ^'*> * ™°™ ^ (
^ ""SffiSS?^^
the
Vol. 49, No. 3
March, 1965
INTRADIAPHRAGMATIC CYST
533
On Aug. 21, 1963, a left thoraeotomy was performed and a multiloculated yellow cyst, 5 cm. by 3 cm. by 3 cm., containing gray-yellow mucopurulent material, was found imbedded in the muscular diaphragm, 3 cm. anterior and to the left of the esophageal hiatus. The lesion was excised and the rent in the diaphragm was closed in 2 layers without difficulty. The patient's postoperative course was smooth and uncomplicated and she was discharged on the ninth day following the operation. Culture of the cystic fluid showed no bacterial growth in 72 hours. Histologie examination demonstrated cystic spaces lined by pseudostratified, columnar, ciliated epithelium, with underlying mucous glands and plates of cartilage arranged in hap hazard fashion (Fig. 2, A and B). DISCUSSION
Authors reporting on this entity specifically refer to its mtfradiaphragmatic location, since other cystic structures, such as enteric, renal, and pericardial, occur on either side of and in close proximity to the diaphragm, but are not integral to the formation of the diaphragm. 7 Plausible, but certainly theoretical, explanations have been advanced for each of the 3 types of cysts described in this location. Mesothelial cysts are believed to result from an invagination and pinching off of a portion of the advancing edge of the pleuroperitoneal and pleuropericardial membranes prior to their fusion with the septum transversum during the fourth to sixth week of fetal life.8 Bronchial cysts are most likely due to a sequestration of primitive lung-bud tissue caught up in the same fusion process of the components of the future diaphragm. Fibrous-walled cysts can be explained best as degenerative mesothelial or bron chial types wherein all distinctive elements, especially the lining membrane, are destroyed or replaced by fibrous tissue, presumably secondary to chronic inflammation or spontaneous regression. Bronchial cysts occur more often elsewhere, the most frequent site being the upper posterior mediastinum near the tracheal bifurcation. Willis 9 described such sequestrations of primitive lung anlage as "ectopias,"'stating that they are usually unilocular, thin-walled, with mucoid contents, and lined by ciliated pseudostratified columnar epithelium of a respiratory type—the walls contain ing bundles of smooth muscle, mucous and mixed glands, and nodules and plates of cartilage. In the case presented here, the cyst was truly intradiaphragmatic since the pleura was incised to remove it, following which the peritoneal surface of the diaphragm remained intact. This cyst or "ectopia" was multiloculated, as were others reported, and it contained all elements of bronchial structure. As with other cysts reported, the symptoms were mild and rather nonspecific; op erative removal was relatively simple; and postoperative recovery was uncom plicated, as in other reported cases. SUMMARY
By the best count available, the cyst reported herein is the thirteenth intradiaphragmatic and fifth bronchial cyst of the diaphragm recorded. A brief discussion of the origin and development of the 3 types of intradiaphragmatic
AARON
534
J. Thoracic and Cardiovas. Surg.
cysts is presented. This group of lesions constitutes a rarely encountered patho logic entity and it is of interest only to exclude more frequently encountered malignant and benign tumors of the diaphragm and adjacent structures. REFERENCES 1. Kessler, H. J., and Maier, H. C : Intradiaphragmatic Cysts, J . THORACIC SURG. 30: 159, 1955. 2. Buddington, W. T.: Intradiaphragmatic Cyst: Ninth Reported Case, New England J . Med. 257: 613, 1957. 3. Felder, M. E . : Intradiaphragmatic Cyst, Am. J . Surg. 98: 95, 1959. 4. Kaganskii, V. E . : Sluckae kisty diafragmy (Case of Diaphragmatic Cyst), Voprosy Onhologhii (Leningrad) 5: 368, 1959. 5. Balas, A., and Kalmar, M.: On Bronchial Cysts of the Diaphragm, Thoraxchirurgie 9: 513, 1962. 6. Ochsner, A. O., and Ochsner, A. O., J r . : Tumors of the Diaphragm in Diagnosis and Treat ment of Tumors of the Chest, edited by D. M. Spain, New York, 1960, Grune & Stratton, pp. 240-244. 7. Abell, M. R.: Mediastinal Cysts, Arch. Path. 6 1 : 360, 1956. 8. Clough, D. M., and Bierne, M. T.: Benign Mesotlielial Cyst of the Diaphragm, J . THORACIC SURG. 29:
212,
1955.
9. Willis, R. A.: The Borderline of Embryology and Pathology, London, 1958, Butterworth & Co., Ltd., pp. 41, 313-314.
BOARD OF THORACIC SURGERY E X A M I N A T I O N S WRITTEN EXAMINATION : Various centers throughout the country, September 10, 1965. Final date for application is June 1, 1965. ORAL EXAMINATION: October, 1965. Final date for filing application is June 1, 1965. Please address all communications to Board of Thoracic Surgery, Inc., 1151 Taylor Avenue, Detroit, Michigan 48202.