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Mediastinal enterogenic cyst with spinal cord compression
CASE R E P O R T S
Mediastinal Enterogenic Cyst With Spinal Cord Compression By A. M. Piramoon, M.D., F.A.C.S., and K. Abbassioun, M.D. Tehran, Iran Enterogenic cysts are rare congenital anomalies usually presenting as a posterior mediastinal mass, less frequently found in lower cervical or a b d o m i n a l regions. They are often associated with vertebral anomalies and m a y give rise to neurologic deficits. We report a case of mediastinal enterogenic cyst with intraspinal extension t h r o u g h an anterior spina bifida causing cord compression and paraplegia. There h a s been complete recovery after transthoracic total excision of the cyst.
CASE REPORT A 2-yr 10-mo-old boy was transferred because of a posterior mediastinal mass and paraplegia. At the age of 2, the parents noted some irritability, respiratory distress, and vague chest a n d abdominal pains. These s y m p t o m s became progressively worse with development of some cyanosis, and he was admitted to a local hospital for evaluation. His chest x-ray showed a mass in superior posterior mediastinum. During the course of work-up, the child developed progressive weakness of the lower limbs to the extent that he was bedridden 15 20 days prior to his transfer. Physical examination revealed a chronically ill child with an u n h a p p y expression. He was n o r m o cephalic with a head circumference of 47 cm. His neck was supple. He was tachypneic with mild shortness of breath but no labored respiration or cyanosis. Examination of the chest, including percussion and auscultation, was unrevealing. T h e remainder of physical examination was unremarkable save for neurologic findings. He was unable to sit, stand, or walk because of a severe spastic paraplegia. There was sensory i m p a i r m e n t to all modalities of sensations with a loss o f pinprick sensation to the level of C s - T t. N o spinal tenderness or deformity was noted clinically. Roentgenologic examination revealed a superior posterior mediastinal soft-tissue mass projected to the left, m e a s u r i n g 7 • 6 cm in diameter. T h e r e were readily noted spinal deformities involving upper thoracic region, hemivertebrae of T5 on the right, anterior spina bifida of T3 a n d T 4 with an anterior elliptical defect 12 • 5 m m in diameter and mild scoliosis to the right from T2 to T8 (Fig. I). E s o p h a g o g r a m showed marked displacement of e s o p h a g u s anteriorly and to the right (Fig. 2). Myelography showed a complete block to the passage of opaque m e d i u m at the level of vertebral body o f T 6 (Fig. 3). With the possibility of an anterior meningocele or a neurogenic t u m o r in mind, the following surgical procedure was performed: A left posterolateral t h o r a c o t o m y incision was made t h r o u g h the fifth interspace. There was a rather large, almost r o u n d , and well-circumscribed mass in the posterior mediastinum. The cystic nature of the m a s s became evident and it was free all around except at the site o f vertebral defect t h r o u g h which it extended into the spinal canal. Needle aspiration revealed thick, creamy yellow fluid, excluding the possibility o f anterior meningocele. After aspiration of the cyst content, gentle traction was applied on the cyst, a n d the intraspinal portion was gradually delivered through the defect. There was a small portion of the cyst severely adherent to the dura, which was teased off gently. However, this led to a small break in the dura and resulted in the leakage of clear cerebrospinal fluid. The dura was then repaired and the bony defect covered with a portion of parietal pleura. A chest tube was inserted and routine closure of the t h o r a c o t o m y w o u n d performed. His postoperative course was uneventful except for excessive drainage of clear fluid t h r o u g h the chest tube which was t h o u g h t to be due to leakage of cerebrospinal fluid. The chest tube was removed on the 9th postoperative day. N o respiratory e m b a r r a s s m e n t was encountered a n d a chest x-ray on the 15th postoperative day showed complete expansion of the lungs and no sign of hydrothorax. Also, postoperative myelogram showed free passage of the radiopaque material Journal of Pediatric Surgery, Vol. 9, No. 4 (August), 1974
543
544
CASE REPORTS
Fig. 1. Chest x-ray. Note the superior mediastinal mass projecting to the left and spinal anomalies of the upper thoracic region.
Fig. 2. Esophagogram Posteroanterior and lateral projections. Note esophagus displaced anteriorly and to the right, with narrowing.
Fig. 3. Myelogram. Note complete block of the dye column at the level of T6.
CASE REPORTS
545
beyond the site of spinal blockage. Neurologically he began to show signs of recovery o f function from the very first postoperative day. By the time of discharge (4 wk postoperative), he had no sensory deficit, could move his lower limbs freely, and was able to sit by himself, and stand and walk with assistance. The cyst wall showed mucosa similar to that of gastric mucosa composed of glands with chief cells and supported by thick smooth-muscle bundles. Portion of the cyst was lined with fresh granulation tissue and infiltrated by chronic inflammatory cells. There was no serosal layer.
DISCUSSION Reports of similar cases in the literature have come out under many headings, such as neuroenteric cysts, gastroeytoma, enterogenous cysts, and foregut mediastinal cysts. These reported cases fall in one of several categories: purely intraspinal or even intramedullary lesion, 2'4'5 mediastinal cysts adherent to the spinal column without intraspinal extension, mediastinal cysts with intraspinal expansion, 1'3 and cysts with connections to the spine a n d / o r to a reduplicated bowel below the diaphragm. Histologically, the enterogenous cysts lack the serosal layer. Their muscularis coat is composed of two to three well-developed layers, and the mucosa usually has all the characteristic features of gastric mucosa, but can be composed of any type o f alimentary tract mucosa. Mucosa often shows ulcerations with evidences of inflammatory or hemorrhagic changes in the submucosal layer. The content of the cyst usually has pepsin and acid. In the majority of cases, symptomatology begins in early childhood, and the cyst is diagnosed immediately after birth or at very early age, only a few being discovered after the first decade of life. Once a mass in the posterior mediastinum is noted on a chest x-ray, a thorough search should be made for spinal anomalies particularly at the lower cervical or upper thoracic regions. Tomogram of the spine may be found helpful. These anomalies consist of anterior spina bifida, hemivertebrae with or without scoliosis, and incomplete fusion of the neural arches. Surgical extripation of these cysts generally results in permanent cure. Successful removal of purely intraspinal enterogenous cysts has been reported. 4'5 In cases of mediastinal cysts with intraspinal extension, laminectomy resulted in only temporary improvement in neurological status and permanent cure could be achieved only after intrathoracic removal of the cyst. 1'3 As shown in this case, primary transthoracic approach to the cyst resulted in the patient's cure and eliminated the need for laminectomy and its possible future disabilities. However, great care must be exercised to manipulate the intraspinal portion of the cyst as little as possible and to avoid injury to any intraspinal structure which might contain functioning neural tissue.
ACKNOWLEDGMENT We thank Dr. M. Ziai, Professor of Pediatrics, for his helpful advice. We also gratefully acknowledge the great assistance of our colleagues Dr. M. Tafazoli from radiology service and Dr. F. Raafat from pathology service of Ahari Hospital, Children's Medical Center.
REFERENCES 1. Dorsey JF, Tabrisky J: Intraspinal and mediastinal foregut cyst compressing the spinal cord, report of a case. J Neurosurg 24:562, 1966 2. Harriman DGF: An intraspinal enterogenous cyst. J Pathol Bacteriol 75:4-13, 1958 3. Holcomb G W Jr, Matson DD: Thoracic neuroenteric cyst. Surgery 35:115, 1954
4. Knight G, Griffiths T, Williams I: Gastrocytoma of the spinal cord. Br J Surg 42:635, 1955 5. Scoville WB, Manlopuz J, Otis RD, et al: Intraspinal enterogenic cyst. J Neurosurg 20:704, 1963
Mediastinal Enterogenic Cyst With Spinal Cord Compression By A. M. Piramoon, M.D., F.A.C.S., and K. Abbassioun, M.D. Tehran, Iran Enterogenic cysts are rare congenital anomalies usually presenting as a posterior mediastinal mass, less frequently found in lower cervical or a b d o m i n a l regions. They are often associated with vertebral anomalies and m a y give rise to neurologic deficits. We report a case of mediastinal enterogenic cyst with intraspinal extension t h r o u g h an anterior spina bifida causing cord compression and paraplegia. There h a s been complete recovery after transthoracic total excision of the cyst.
CASE REPORT A 2-yr 10-mo-old boy was transferred because of a posterior mediastinal mass and paraplegia. At the age of 2, the parents noted some irritability, respiratory distress, and vague chest a n d abdominal pains. These s y m p t o m s became progressively worse with development of some cyanosis, and he was admitted to a local hospital for evaluation. His chest x-ray showed a mass in superior posterior mediastinum. During the course of work-up, the child developed progressive weakness of the lower limbs to the extent that he was bedridden 15 20 days prior to his transfer. Physical examination revealed a chronically ill child with an u n h a p p y expression. He was n o r m o cephalic with a head circumference of 47 cm. His neck was supple. He was tachypneic with mild shortness of breath but no labored respiration or cyanosis. Examination of the chest, including percussion and auscultation, was unrevealing. T h e remainder of physical examination was unremarkable save for neurologic findings. He was unable to sit, stand, or walk because of a severe spastic paraplegia. There was sensory i m p a i r m e n t to all modalities of sensations with a loss o f pinprick sensation to the level of C s - T t. N o spinal tenderness or deformity was noted clinically. Roentgenologic examination revealed a superior posterior mediastinal soft-tissue mass projected to the left, m e a s u r i n g 7 • 6 cm in diameter. T h e r e were readily noted spinal deformities involving upper thoracic region, hemivertebrae of T5 on the right, anterior spina bifida of T3 a n d T 4 with an anterior elliptical defect 12 • 5 m m in diameter and mild scoliosis to the right from T2 to T8 (Fig. I). E s o p h a g o g r a m showed marked displacement of e s o p h a g u s anteriorly and to the right (Fig. 2). Myelography showed a complete block to the passage of opaque m e d i u m at the level of vertebral body o f T 6 (Fig. 3). With the possibility of an anterior meningocele or a neurogenic t u m o r in mind, the following surgical procedure was performed: A left posterolateral t h o r a c o t o m y incision was made t h r o u g h the fifth interspace. There was a rather large, almost r o u n d , and well-circumscribed mass in the posterior mediastinum. The cystic nature of the m a s s became evident and it was free all around except at the site o f vertebral defect t h r o u g h which it extended into the spinal canal. Needle aspiration revealed thick, creamy yellow fluid, excluding the possibility o f anterior meningocele. After aspiration of the cyst content, gentle traction was applied on the cyst, a n d the intraspinal portion was gradually delivered through the defect. There was a small portion of the cyst severely adherent to the dura, which was teased off gently. However, this led to a small break in the dura and resulted in the leakage of clear cerebrospinal fluid. The dura was then repaired and the bony defect covered with a portion of parietal pleura. A chest tube was inserted and routine closure of the t h o r a c o t o m y w o u n d performed. His postoperative course was uneventful except for excessive drainage of clear fluid t h r o u g h the chest tube which was t h o u g h t to be due to leakage of cerebrospinal fluid. The chest tube was removed on the 9th postoperative day. N o respiratory e m b a r r a s s m e n t was encountered a n d a chest x-ray on the 15th postoperative day showed complete expansion of the lungs and no sign of hydrothorax. Also, postoperative myelogram showed free passage of the radiopaque material Journal of Pediatric Surgery, Vol. 9, No. 4 (August), 1974
543
544
CASE REPORTS
Fig. 1. Chest x-ray. Note the superior mediastinal mass projecting to the left and spinal anomalies of the upper thoracic region.
Fig. 2. Esophagogram Posteroanterior and lateral projections. Note esophagus displaced anteriorly and to the right, with narrowing.
Fig. 3. Myelogram. Note complete block of the dye column at the level of T6.
CASE REPORTS
545
beyond the site of spinal blockage. Neurologically he began to show signs of recovery o f function from the very first postoperative day. By the time of discharge (4 wk postoperative), he had no sensory deficit, could move his lower limbs freely, and was able to sit by himself, and stand and walk with assistance. The cyst wall showed mucosa similar to that of gastric mucosa composed of glands with chief cells and supported by thick smooth-muscle bundles. Portion of the cyst was lined with fresh granulation tissue and infiltrated by chronic inflammatory cells. There was no serosal layer.
DISCUSSION Reports of similar cases in the literature have come out under many headings, such as neuroenteric cysts, gastroeytoma, enterogenous cysts, and foregut mediastinal cysts. These reported cases fall in one of several categories: purely intraspinal or even intramedullary lesion, 2'4'5 mediastinal cysts adherent to the spinal column without intraspinal extension, mediastinal cysts with intraspinal expansion, 1'3 and cysts with connections to the spine a n d / o r to a reduplicated bowel below the diaphragm. Histologically, the enterogenous cysts lack the serosal layer. Their muscularis coat is composed of two to three well-developed layers, and the mucosa usually has all the characteristic features of gastric mucosa, but can be composed of any type o f alimentary tract mucosa. Mucosa often shows ulcerations with evidences of inflammatory or hemorrhagic changes in the submucosal layer. The content of the cyst usually has pepsin and acid. In the majority of cases, symptomatology begins in early childhood, and the cyst is diagnosed immediately after birth or at very early age, only a few being discovered after the first decade of life. Once a mass in the posterior mediastinum is noted on a chest x-ray, a thorough search should be made for spinal anomalies particularly at the lower cervical or upper thoracic regions. Tomogram of the spine may be found helpful. These anomalies consist of anterior spina bifida, hemivertebrae with or without scoliosis, and incomplete fusion of the neural arches. Surgical extripation of these cysts generally results in permanent cure. Successful removal of purely intraspinal enterogenous cysts has been reported. 4'5 In cases of mediastinal cysts with intraspinal extension, laminectomy resulted in only temporary improvement in neurological status and permanent cure could be achieved only after intrathoracic removal of the cyst. 1'3 As shown in this case, primary transthoracic approach to the cyst resulted in the patient's cure and eliminated the need for laminectomy and its possible future disabilities. However, great care must be exercised to manipulate the intraspinal portion of the cyst as little as possible and to avoid injury to any intraspinal structure which might contain functioning neural tissue.
ACKNOWLEDGMENT We thank Dr. M. Ziai, Professor of Pediatrics, for his helpful advice. We also gratefully acknowledge the great assistance of our colleagues Dr. M. Tafazoli from radiology service and Dr. F. Raafat from pathology service of Ahari Hospital, Children's Medical Center.
REFERENCES 1. Dorsey JF, Tabrisky J: Intraspinal and mediastinal foregut cyst compressing the spinal cord, report of a case. J Neurosurg 24:562, 1966 2. Harriman DGF: An intraspinal enterogenous cyst. J Pathol Bacteriol 75:4-13, 1958 3. Holcomb G W Jr, Matson DD: Thoracic neuroenteric cyst. Surgery 35:115, 1954
4. Knight G, Griffiths T, Williams I: Gastrocytoma of the spinal cord. Br J Surg 42:635, 1955 5. Scoville WB, Manlopuz J, Otis RD, et al: Intraspinal enterogenic cyst. J Neurosurg 20:704, 1963