An Intestinal Duplication Presenting As an Intrathoracic Mass

Herbert V. Swindell, Col, USAF (MC)” and Daniel C. Campbell, Col, USL4F (MC)

N 1950 duplications of the foregut that communicated with the small intestine and passed through the diaphragm into the chest I were described by Gross and his co-workers [6], who reported 3

cases; 2 others were reported in the same year, one in the Case Records of the Massachusetts General Hospital [ l ] and the other by Fisher [ 5 ] . Eight additional cases have been reported subsequently [2-4, 7 , 9-12]. All these cases except one were in infants, were associated with other congenital malformations, and were symptomatic.

We are reporting a duplication occurring in an adult which com- municated with the duodenum and extended into the left hemithorax. T h e previously reported 13 cases are also summarized in an attempt to formulate a rational mode of diagnosis and treatment of these unusual congenital aberrations.

CASE R E P O R T

R. M., a 29-year-old radio repairman, had been stationed in Thule, Green- land, in 1957. At that time he noted that. the cold weather caused him to have colicky pain in his upper abdomen; however, this was a common complaint in the area and he did not seek medical advice. In August, 1957, he was transferred to MacDill Air Force Base, Florida, and was physically well until the onset of cold weather in November, 1957. At that time he suffered an acute attack of what he described as “stomach pains-as though gas was increasing in his stomach.” This pain was colicky in nature and made it difficult for him to breathe. I t started in the upper abdomen and spread beneath the ribs, to both the left and the right sides, but did not radiate to the back. A diagnosis of pneumonia in the left lower lung was made and he was admitted to the hospital for 14 days. H e was treated with penicillin and chlortetracycline (Aureomycin), and when the x-ray picture was “almost clear,” he was returned to duty.

Two weeks later the patient suffered an identical attack. X-ray films were

From Department of Surgery, USAF Hospital, Keesler Air Force Base, Miss., and Department

*Chairman, Department of Surgery, USAF Hospital, Keesler Air Force Base, Miss. ?Chairman, Department of Surgery, Wilford Hall USAF Hospital, Lackland Air Force

This paper represents the personal viewpoint of the authors and is not to be construed

Received for publication July 20, 1964.

of Surgery, Wilford Hall USAF Hospital, Lackland Air Force Base, Tex.

Base, Tex.

as a statement of official Air Force policy.

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In t rat horacic In t est ina 1 Duplication

again taken, the diagnosis being “pneumonitis with pleurisy and a cloudy lung.” He was treated in the hospital for 7 days with penicillin and discharged. Two days later a third attack occurred and he was readmitted to the hospital. X-ray films revealed the same left-lower-lobe clouding and a thoracentesis yielded 30 ml. of clear red fluid. He was discharged from the hospital after 9 days; 3 weeks later he suffered a recurrence and was readmitted for 6 weeks. Repeated sputum examinations were negative for acid-fast organisms. The tuberculosis skin test (P.P.D. No. 1) was positive. Over the New Year holiday he drank beer which apparently induced another attack. As a result of this attack, he was admitted to the hospital for 5 days. He received no medication during this time, but became asymptomatic and returned to duty.

In August, 1958, he noted repeated episodes of what he described as gas pains i n his upper abdomen, and could hear and feel a gurgling which seemed to radiate from the left to the right side. He was seen on sick call on several occa- sions and treated with a green liquid preparation which relieved his discomfort agreat deal. On December 8, 1958, he suffered a severer attack than usual, with steady rather than intermittent discomfort in the upper abdomen. This pain radiated up to the left side of the chest and the left shoulder. He was admitted to the hospital with the diagnosis of pneumonia of the left lower lobe. There was clinical and x-ray evidence of pleural fluid, and a thoracentesis again yielded 30 ml. of clear red fluid. The effusion cleared spontaneously by December 19, 1958; however, a rounded density persisted in the left retrocardiac area, and the patient was transferred to Wilford Hall United States Air Force Hospital for further evaluation.

The physical examination at the time of admission to Wilford Hall USAF Hospital was entirely normal. The presence of the rounded density in the left side of the chest was confirmed by x-ray examination. Barium studies failed to reveal any connection with the stomach or upper gastrointestinal tract. T h e initial impression was either pleural cyst, pulmonary sequestration, or entero- genous cyst.

On January 13, 1959, the patient underwent an exploratory thoracotomy on the left side. A large cystic structure was found in the posterior mediastinum below the level of the hilum which was dissected free and found to extend through the esophageal hiatus. I t also extended superiorly above the hilum to the apes of the chest as an obliterated fibrous cord. T h e diaphragm was opened and the cyst was found to continue into the abdomen, posterior to the stomach, and metlially across the midline. The thoracotomy incision was then closed and the abdomen opened through an upper paramedian incision. T h e cystic structure, which had been delivered into the abdomen, was again identified and found to extend to the right of the midline, turn inferiorly, pass behind the pancreas, and communicate with the fourth portion of the duodenum. The retropancreatic pqrtion could not be removed without considerable risk of injury to the portal vein or the pancreas, so the mass was amputated at the superior edge of the pancreas and removed. The patient was also found to have a first-degree malrota- tion of the midgut with the cecum lying in the upper quadrant. The postopera- tive course was entirely uneventful and he returned to full active duty.

Pathological examination of the specimen revealed it to be a muscular tube lined with gastrointestinal mucosa.

DISC I ‘SSI 0 N

Intestinal duplications result from the formation of diverticula during embryonic life, which may or may not retain their original communication to the gastrointestinal tract. These diverticula must not

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SWINDELL AND CAMPBELL

be confused with Meckel’s diverticula, which are remnants of the om- phalomesenteric duct.

This theory is supported by the work of Lewis and Thyng [8], who found knob-like diverticula in the intestine of pig embryos, with one or two diverticula occurring regularly in the region of the duodenum. The number of diverticula increase along the intestine with growth of the embryo. These investigators also found these diverticula in human embryos. T h e diverticula may be pinched off from the lumen of the normal gastrointestinal tract and develop its own lumen with the cyst occupying any place in the parent organ. Pedunculation may occur, or the cyst may occupy a position in or out of the mesentery, more or less remote from the parent structure.

In the 13 previous cases plus the present case, there were 9 males and 5 females. T h e ages ranged from 3 weeks to 29 years. T h e patient in the case reported here was the eldest in the group, the next eldest being 25 years of age with symptoms from the age of 4.

There were two basic groups of symptomatology. In one group (6 cases) respiratory symptoms and signs-cough, dyspnea, and cyanosis- predominated. These symptoms are explained by the presence in the extrapleural space of a large cystic structure which was distended by fluid or gas, or both, and which interfered with the expansion and ventilation of the lung. In the present case, the presenting symptoms were due to a pleural effusion which was probably secondary to the duplication. In the other group (8 cases), the presenting symptoms were due to gastrointestinal bleeding, and in the majority of these, gastric mucosa with ulceration was demonstrated in the duplication.

X-ray examination of the chest in all 14 cases presented the ab- normal findings of a soft tissue mass, frequently containing air and fluid. These masses were variously interpreted as intestinal loops, cysts, or tumors. T h e findings of a gas-containing cyst in the thorax suggests diaphragmatic hernia or intestinal duplication, since most esophageal duplications do not communicate with the esophageal lumen and do not contain air.

There is an apparent high incidence of spinal anomalies associated with this condition, as evidenced in 9 of the 14 cases. This is supposedly due to excalation of the embryonic notochord to produce simultaneously spinal anomalies and thoracic duplications. T h e presence of spinal anomalies can act as a differential point between duplications and diaphragmatic hernia.

T h e only reasonable therapeutic approach is surgical removal of the duplication. This is particularly true in light of the high incidence of massive hemorrhage associated with this anomaly. Frequently mul- tiple procedures are required to establish the true extent of the lesion or complete its removal. Concerning the necessity of excising all of the

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Intmthorciric Intestinnl lhpl icci t ion

duplication, Gross et al. [6] concluded that when the symptoms are en- tirely due to the presence of a mass and there is accumulation of fluid and gas, excision of the thoracic portion only is necessary. If the symp- toms are due to ulceration and hemorrhage or the cyst is lined with gastric mucosa, then complete removal is indicated.

SUM M A It Y

A case of intestinal duplication extending into the thorax is pre- sented. This is the second case reported in an adult. Previously reported cases are reviewed and the symptomatology and embryology are dis- cussed. Surgical removal is advocated as the treatment of choice.

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Gross, R. E., Neuhauser, E. B., antl Longino, L. A. Thoracic tliverticuli which originate from the intestine. A n n . Stirs. 131:363-375, 1950. Leitler, H. J . , Snotlgrass, J . J . , antl Mishrick, A. S. Intrathoracic alimentary duplication communicating with small intestine. A . M . A . Arch. Siirg. 71:230- 233, 1955. Lewis, F. T., antl Thyng, F. W. T h e regular occurrcnce of intestinal di- verticula in embryos ol the pig, rabbit, antl man. Amer. J. Anat . 7:505-519, 1907. McLetchie, N. G., Purvis, .J. K., antl Sauntlers, K. L. T h e genesis of gastric antl certain intestinal tliverticiila antl eliterogenous cysts. S w g . Gynec. Obstet . 99: 135-141, 1954. Owen, H. W., Holman, C. B., antl Priestley, J. T. Duplication of the stomach: Report of a case. Proc. M n y o Clin . 29228-234, 1954. Spear, H. C., Daughtry, D. C., antl Chesney, J. G. Intestinal duplication cyst of abdominal origin presenting in thorax. J. Thorac. Sztrg. 37:810-814, 1959. Van der Hal, I., antl Moog, G. S. Intra-abdominal en Intrathoracaal Gele- gen Macrotlivertikel van het Jejunum, van Gastrogene Dosaprong het Ver- band met Mediastinal Gastrogene Cystes. Nederl. T . Genc~rsk. 94:2533-2538, 1950.

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