Surgical Management of Intracranial Neuroenteric Cysts: The UCSF Experience

Objective Modern surgical experience with intracranial neuroenteric cysts is limited in the literature. We review our 15-year institutional experience with these rare lesions. Design Single-institution retrospective study. Setting Large North American tertiary care center. Participants Histologically confirmed cases of intracranial neuroenteric cyst from January 2000 to September 2014. Main Outcome Measures Pre- and postoperative modified Rankin Scale (mRS) scores, extent of resection, and postoperative complications are reported. Clinical presentation, imaging features, pathology, and operative approach are discussed. Results Five spinal and six intracranial neuroenteric cysts were surgically treated over a 15-year period. Median age at presentation for the intracranial cysts was 38.5 years. Mean cyst diameter was 3.8 cm. Five cysts were located in the pre-pontomedullary cistern, and one was located in the third ventricle. Gross total resection was achieved in four of the five posterior fossa cysts through a far lateral transcondylar approach. Postoperative complications included aseptic meningitis (one), transient abducens palsy (one), and pseudomeningocele requiring reoperation (three). Postoperative mRS scores improved to ≤1 by 6.5 months median follow-up. Conclusions Intracranial neuroenteric cysts are rare lesions with a variable imaging appearance. Complete surgical resection through a far lateral transcondylar approach is possible and usually results in symptom improvement or resolution.     

Bronchogenic cyst.

Two recent patients with bronchogenic cysts, presenting in an atypical manner, stimulated our review of this subject. Twenty patients with bronchogenic cysts have been treated at the Boston Floating Hospital over the past 20 yr. Of these patients, 19 out of 20 were symptomatic, the most common symptom being fever (6 out of 20). Half of our patients had no respiratory symptoms but only one was found to have an asymptomatic mass on chest x-ray. The majority of theses cysts were found within the pulmonary parenchyma, the right lung being affected three times more commonly than the left. These facts are at odds with the reports in the literature. Three were found in the neck. Bronchogenic cysts are generally thought to be small, solitary, and limited in area. However, a significant number in our series (8 out of 20) were large, multiple or multicystic, and involved segments, lobes, multiple lobes, and, on one occasion, an entire lung. The correct pre-operative diagnosis was made in only 30% of the cases. The fact that the diagnosis was often missed led to long delays in treatment resulting in recurrent infectious complications and repeated hospitalizations. The most common cause of error in diagnosis was failure to follow a pneumonia to complete resolution. Despite the frequent delays in diagnosis, surgical treatment was curative in all patients.     

Esophageal cyst,a case report and a review of the literature

The esophageal cyst is a rare disease only 20 cases of which have been reported in the literature in Japan. We recently treated such a patient and at thoracotomy, we found the cyst to be located the submucosal layer of the esophagus. The cyst could be easily extirpated and the patient made an uneventful recovery. Gross findings revealed thick, yellow and mucus-like contents inside the cyst. Microscopically, the cyst was lined with ciliated columnar epithelium and there was no evidence of cartilage. The 21st such occurrence in Japan is reported herein and a discussion is made of related literature.     

A Rare Presentation of a Giant Epidermoid Inclusion Cyst Mimicking Malignancy

Epidermoid inclusion cysts, infundibular cysts, and retention cysts are dense, well-encapsulated benign soft tissue lesions that develop after a portion of the epidermis has become implanted in the dermis on a follicular surface such as the scalp, face, or trunk. However, on acral surfaces such as the palms and soles, these cysts present <10% of the time. Typically, these lesions will not progress to sizes >5 cm. However, a rare subtype of epidermal cysts known as giant epidermal cysts tends to be much larger. Unlike epidermal inclusion cysts, giant cysts lack a central punctum, present on areas of thick skin lacking hair follicles, and affect an older patient population. Because of their large size and unique characteristics, few cases of giant epidermal cysts localized to the foot have been reported. We present a case report of a 57-year-old male with a rapidly growing, large, ulcerated, atypical epidermal inclusion cyst that had developed on the medial aspect of his hallux with underlying osseous changes. In this particular case, the location, overlying skin changes, and rapid growth of the lesion raised suspicion for malignancy. In the present report, we discuss the unusual features of this particular cyst and the etiologies, treatment course, and short-term outcomes regarding this unique tumor.     

Mucus-secreting presacral cyst

A mucus-secreting presarcal cyst was found in a 49-year-old woman who complained of dysuria. A routine rectal digital examination revealed a retrorectal mass. Diagnostic imaging demonstrated a large presacral cystic tumor. The cyst was removed through a transsacral approach. Histologically, the cyst membrane consisted of squamous and columnar mucus-secreting epithelium, and was diagnosed as a mucus-secreting presacral cyst.     

Giant chylolymphatic mesenteric cyst and its successful enucleation: A case report

INTRODUCTION

Cysts of the mesentery are among surgical rarities. The clinical presentation is not characteristic and in addition, the preoperative imaging although suggestive is not diagnostic in this case ultrasound and CTscan was consistent with giant mesenteric cyst. In most cases, the diagnosis is confirmed after surgical exploration.

PRESENTATION OF CASE

A 42 yrs old male patient on exploratory laparotomy had a 14cm×10cm×10 cm cysts which was seen arising from the mesentery ofdistal jejunum 80cm from the duodeno jejunal flexure. The cyst was enucleated successfully from themesentery without entailing resection. The cyst contained milky white fluid consistent with a chylolymphatic cyst. The diagnosis was confirmed on histopathology which revealed a cyst wall with lymphoidaggregates. After 3 years of follow-up, the patient is doing well and there is no evidence of recurrence.

DISCUSSION

The cysts may be asymptomatic or maymanifest with abdominal pain, distension lump or intestinal obstruction. Our patient was symptomatic with mild and long standing abdominal pain. The definitive diagnosis of these lesions is difficult prior to surgical exploration as there are no pathognomonic symptoms or characteristic imaging findings.

CONCLUSION

Cysts of the mesentery are among surgical rarities. In most of the cases the diagnosis is confirmed after surgical exploration and removal of thecyst. We would like to emphasize the importance of successful enucleation of the cyst irrespective of its size due to its independent blood supply as opposed to enterogenous cyst which requires bowel resection and anastomosis.     

Neurenteric cyst of the posterior fossa. Case report and review of the literature

Intracranial neurenteric cysts are rare entities. The term is currently used to describe epithelial cysts that are lined with a presumed endodermal-derived epithelium and are mostly located in the posterior fossa.Preoperative diagnosis is often difficult because of their clinical presentation, which may resemble a subarachnoid hemorrhage, and the radiological aspect, which can mimic vascular pathologies.We describe a posterior fossa neurenteric cyst in a 27-year-old woman, who presented with sudden headache as the only symptom and who was addressed to our hospital for subarachnoid hemorrhage. Diagnostic angiography was negative and MRI revealed a prepontine cystic lesion. The patient underwent a posterolateral approach on the right side, with subtotal resection of the lesion.We discuss the embryologic, diagnostic and therapeutic aspects of these cysts and review the literature.     

Presacral epidermoid cyst: Report of a case

(Received for publication on Dec. 26, 1996; accepted on July 8, 1997)     

Lymphoepithelial Cyst of the Pancreas: Report of a Case

A lymphoepithelial cyst (LEC) is an extremely rare benign lesion of the pancreas. During a medical check-up, a 77-year-old man without any symptoms was found to have a cyst in the body of the pancreas. His serum carbohydrate antigen 19-9 level was slightly elevated. Computed tomography showed a multilocular, low-attenuating cyst on the superior surface of the pancreatic body. Thus, we performed distal pancreatectomy with splenectomy. Histological examination revealed that the cyst wall was lined with squamous epithelium and surrounded by abundant mature lymphoid tissue. Keratinous substances were present in the cyst. An LEC of the pancreas is associated with a good prognosis and, although unusual, it should be considered in the differential diagnosis of pancreatic cystic lesions. Minimal resection of the cyst should be performed whenever possible, and extensive surgery avoided. For patients with a high surgical risk, fine-needle aspiration biopsy may be considered.     

DORSAL ENTERIC CYSTS—A STUDY OF EIGHT CASES

Dorsal enteric cysts have been recognized in association with a wide variety of developmental anomalies of the back, the spine, the central nervous system, the mediastinum and the gut. These lesions can be lethal and they caused the deaths of three of the eight patients in this study: two from meningitis and one from erosion into the aorta. Two of these deaths might have been prevented if they had been fully investigated earlier and the life-threatening components of these complex lesions removed. In the most recent case, however, the lesion was detected antenatally, expediting postnatal investigation and surgery. Three of the five survivors have neurological sequelae attributable to their intraspinal pathology.     

“Juxtafacet cysts”, a misleading name for cystic formations of mobile spine (CYFMOS)

To present 58 cystic space-occupying formations of the spinal canal in 53 cases; these formations are called “juxtafacet cysts”. Fifty-Three patients (33 women and 20 men, with an average age of 60.8 years) were evaluated retrospectively by neurosurgery. All of the patients had received simple X-P, computed tomography (CT) and magnetic resonance imaging (MRI) before surgery. The neurological findings were evaluated on admission and in a follow-up review. Surgical intervention was performed on all patients and they underwent gross-total cyst removal. During surgery, the origin of a cyst was well observed. Follow-up data ranged from 6 to 46 months. Patient outcome was graded on a scale of excellent, good, or poor. Histological findings were evaluated. In 53 patients 58 cysts were identified. Four of the patients had multiple cysts. All cysts were associated with mobile spine. Fifty-five cysts were found in the lumbo-sacral region, two cysts were found in the cervico-thoracic region and one cyst in the thoracic region. Forty-two patients presented back pain and 52 patients presented radicular pain. Four patients had a cauda equina syndrome. Sensory disturbance was observed in 24 cases and motor weakness was observed in 21 cases. Claudication was observed in 19 cases. All cases with cervico-thoracic or thoracic cysts presented myelopathy. The duration of these clinical symptoms ranged from 10 days to 10 years. After surgery there was no case of a recurrent cyst during the follow-up period. Thirty-four cases had an excellent outcome, 18 a good outcome, and one a poor outcome. Out of 58 cysts 32 were joint cysts (11 synovial cysts, 21 ganglion cysts). A further 19 were flavum cysts, one was a posterior longitudinal ligament (PLL) cyst and six others were unknown pseudo cysts. In 34 of the cysts we found hemosiderin deposits and in eight amyloid deposits. Present investigation and findings in literature show a clear comparison of these cystic formations and the mobile part of the spine. An anatomical relation to a vertebral joint (“facet”) is only found in some of the cases (32 of 58). Further to that, the name “cyst” is not correct either, because most of the cystic formations are presented without a cell lining on their internal wall and therefore they are pseudo-cystic. We think that these cystic formations should be called “cystic formations of mobile spine” (CYFMOS) rather than “juxtafacet cysts”. A surgical intervention is the best treatment for these cysts if they cause a compression of nerve roots or/and of the spinal cord.     

Cerebellopontine angle epithelial cyst. A case report

A case of epithelial cyst in the cerebellopontine angle is reported. The cyst wall showed glandular epithelium with areas of non-keratinized stratified epithelium and flattened cuboidal cells. The glandular areas stained positively with antibodies to cytokeratin. In addition, the cyst wall contained areas of arachnoid tissue. This, and the unusual position of the cyst, suggest that the epithelial elements in the cyst wall may be metaplastic in origin. Similar previously described cysts were considered to be endodermal in origin.     

Bronchogenic Cysts: A Rare Congenital Cystic Malformation of the Lung

Purpose Bronchogenic cysts are rare congenital cystic malformations of the lung. We retrospectively analyzed ten cases of bronchogenic cyst (BC) to reinforce the importance of recognizing this malformation.Methods Between 1985 and 2000, ten pediatric patients with BC were treated surgically in our department. Their clinical presentation, radiological, operative, and pathological findings were analyzed retrospectively.Results There were five boys and five girls, ranging in age from 16 days to 6 years (mean 6.5 months). The clinical signs and symptoms included respiratory distress in seven patients (70%), cyanosis in four (40%), chronic cough and fever in five (50%), and dysphasia in two (20%). Routine chest X-ray revealed a pulmonary air-filled cyst in six patients (60%) and a pulmonary nodular opacity in four (40%). The diagnosis was supported by computed tomography in four patients and by ultrasonography in two. Eight of the patients were treated by cyst excision and two by lobectomy. The pathological diagnosis made from all specimens was bronchogenic cyst, with squamous metaplasia in two.Conclusion In newborns, infants, and even children, the development of dyspnea, cyanosis, chronic cough, and fever should alert us to the suspicion of a cystic malformation in the lung, such as a bronchogenic cyst, especially if an air-filled cyst is seen on a plain chest X-ray.     

AN EPITHELIAL CYST OF THE SUPRASELLAR REGION

A case of a 26 year old woman with increasing headaches due to a suprasellar cyst is described. Histology was consistent with an epithelial cyst with features resembling both Rathke's cleft cysts and enterogenous cysts. The differential diagnosis and treatment of suprasellar cysts is discussed.     

Unusual multiloculated serpiginous ganglion of the foot

Ganglions are cystic lesion more commonly seen around the wrist joint. Gangliomas of plantar aspect of the foot are rare. We have presented a case of an unusual serpiginous ganglioma of the plantar aspect of the foot. Less literature is available regarding plantar foot gangliomas. The treatment is challenging because of occurrence of the lesion at weight bearing zone. Available options for treatment includes conservative with splint, intralesional injection,arthroscopic excision and open excision.     

Choledochal cyst or pancreatic (retention) cyst: A case report

Right upper quadrant cystic lesions can be difficult to differentiate using noninvasive imaging modalities. The following case report dicuss the common cystic lesions of the hepatoduodenal ligament and right sided pancreas.     

Ciliated hepatic foregut cyst

A male infant was found to have a large congenital hepatic cyst, first noted in late gestation by prenatal ultrasound scan. The cyst communicated with the biliary tree and was eventually removed completely by an extended right hepatectomy. Histopathologic examination showed a thick-walled, unilocular cyst lined predominantly by ciliated, stratified squamous epithelium with an outer wall composed of smooth muscle cells and fibrous tissue. These features are diagnostic of a ciliated hepatic foregut cyst, a rare congenital malformation with histologic similarities to bronchogenic cysts. The young age of our patient, prenatal detection, large size of the cyst, and a clear communication with the biliary tree have not been previously described with ciliated hepatic foregut cysts.     

Management of an epidermoid cyst of the intrahepatic ducts

Epidermoid cysts of the biliary tree have not previously been described. A baby boy presented with a prenatally diagnosed echolucent intrahepatic cyst. Postnatal radioisotope study of the liver demonstrated that the cyst communicated with the biliary tree. Follow-up ultrasound at 6 months demonstrated that the cyst was filled with echogenic material consistent with either blood or biliary debris. Due to the potential for obstruction and cholangitis, surgery was planned. The cyst was located at the confluence of the right and left hepatic ducts and involved all of the common hepatic duct. The entire cyst was resected except for the patch containing 3 duct orifices: the opening of both hepatic ducts as well as the orifice leading to the common bile duct. A Roux-en-Y cyst jejunostomy was created to allow drainage of both left and right hepatic ducts. The connection also provided access to the cyst remnant through the common duct for future endoscopic monitoring of potential malignant transformation.     

Pseudomesenteric cyst

Mesenteric cyst and cystic mesenteric tumour are very rare abdominal growth, which is generally encountered, in the second decade of life. Pseudomesenteric cyst is another variant with traumatic and infective etiology is rare in itself. Only 14 cases have been reported previously in the Japanese literature. Emergency operation was performed in only 3 patients. The etiology of the pseudocyst manifested by acute abdomen was unknown [1]. A case of pseudomesenteric cyst presented in the emergency with acute abdomen is reported here.