Unusual Neuroenteric Cysts: Diagnosis and Management

Abstract

Six cases of unusual neuroenteric (NE) cysts, occurring in children aged 15 months to 18 years, are reported here. Three of the cysts were extramedullary, while the other three were intramedullary. Two of the extramedullary cysts were located at the lumbosacral region, an unusual site. Only one patient had the cyst located in a ventral relation to the cord in the cervicothoracic region. None of the patients had communication with the mediastinum or intraabdominal viscera. Stigmata of spinal dysraphism were seen in three patients. All the children had variable neurological involvement and it was difficult to differentiate NE cyst from other causes of spinal cord or cauda equina lesion, particularly in the absence of some dysraphic marker. One patient presented with minimal neurological deficits and painful torticollis; this patient was suspected to have an atlantoaxial dislocation. All patients were investigated with MRI, and the only diagnostic feature of an NE cyst was an intradural cyst with an anterior vertebral body anomaly in a single case. It was not possible to radiologically differentiate NE cyst from other intraspinal cysts in the rest of the patients. All patients were operated on by the posterior route; an attempt to excise the cyst nearly in toto was made. Evidence of neural tethering most probably due to cyst contents was seen in three patients. All patients improved after surgery. None showed recurrence of the cyst at follow-up of 3 months to 2 years.

Clinical presentation and surgical outcomes of intramedullary neurenteric cysts

“…This characteristic has been explained by periodic rupture of the cyst contents into the subarachnoid space. 20,30,33 Nevertheless, no patient in our series presented with a meningitis-like syndrome. Riviérez et al 32 postulated that a fluctuation in the rates of active mucin secretion and resorption by the cyst wall may also be related to intermittent symptoms.…”

“…10,24 Additional CT scanning is recommended to help rule out any concurrent bone abnormalities; 22,27 however, MRI can also provide a complete noninvasive assessment of bony abnormalities, especially with continued increases in the precision of associated computer software. 20,26,38 In our series, CT was performed in only 1 case. Each patient underwent plain radiography and MRI, and all of the bone abnormalities were diagnosed preoperatively.…”

“…Progressive or intermittent motor deficits were the main symptoms, accompanied with or without pain or sensory deficits. 10,[20][21][22]25,[32][33][34][37][38][39] In 4 infants, they presented as a meningitis-like syndrome, probably resulting from rupture of the cyst into the subarachnoid space. 8,14,19,26,40 Although commonly associated with other abnormalities, intraspinal NECs may be found without any abnormalities, and bone abnormalities were found in only 8 patients (21.1%) with an intramedullary NEC.…”

“…10,20,22,[24][25][26]33 Cyst-subarachnoid shunting has been verified to be effective for ependymal cysts; 43 however, whether it is suitable for intramedullary NECs remains controversial. The method was thought to be simple and can prevent cyst recurrence, 30 but more authors have rejected it because the spillage of caustic cyst contents into the subarachnoid space might cause inflammation of the neural elements.…”

“…22 To our knowledge, only 38 cases of NEC (with or without concurrent malformations) have been described since the publication of the earliest report in 1954. [2][3][4]8,10,[12][13][14]16,[18][19][20][21][22][24][25][26][27][29][30][31][32][33][34][35][37][38][39][40][41][42]44,45 Most previous publications regarding intramedullary NECs have been case reports. Therefore, the optimal management for this lesion has not been fully determined.…”