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.     

Double cervical cyst derived from a single thyroglossal duct tract

Thyroglossal duct cysts are one of the most common congenital cervical pathologic findings in children. This type of cyst can be located anywhere between the base of the tongue and the sternal manubrium. We report the case of a patient with a double thyroglossal cyst, one located in the hyoid region and the other in the thyroid gland. The 2 cysts were connected by a permeable tract, which confirms that an involution failure of the embryonic remains of the thyroglossal duct was responsible for the development of the cyst. Intrathyroid cysts of the thyroglossal duct, though uncommon, should be included in the differential diagnosis of thyroid cysts in children.     

Congenital inclusion cysts of the anterior fontanelle

BACKGROUND: Congenital inclusion cysts of the anterior fontanelle are rare lesions. Both dermoid and epidermoid cysts are located in the midline of the scalp and occupy the subgaleal space. METHODS: We report 7 cases, 4 boys (57.1%), and 3 girls (42.9%), with ages ranging from 3 months to 16 years (mean 40.85 +/- 68.56 months; median 10 months). Four patients (57.1%) were white and 3 (42.9%) were Afro-Brazilian. RESULTS: The cysts had manifested soon after birth in all patients. They gradually enlarged, with no intracranial extensions. Four patients had dermoid cysts and the others had epidermoid cysts. All of them underwent surgery with complete excision of the cyst and no capsular rupture. There were no complications and no deaths. CONCLUSIONS: Congenital inclusion cysts of the anterior fontanelle are rare lesions that usually manifest at birth. The diagnosis is usually easy and surgery is mandatory, with a good prognosis. Recurrence is rare.     

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.     

Laparoscopic Spleen-Preserving Distal Pancreatectomy in a Solitary True Pancreatic Cyst

Background:

Solitary true pancreatic cysts (STPCs), or epithelial cysts, are benign lesions that are extremely rare in adult patients. Advances in radiographic techniques have improved the ability to identify pancreatic cystic lesions. We report a case of a large and symptomatic STPC in a 47-year-old female patient who was treated successfully with spleen-preserving laparoscopic distal pancreatectomy. We also review the clinical and pathologic features of all reported STPCs within the past 25 years.

Database:

To compose the review, we did a search of the international literature for STPCs that had occurred in adults. Fourteen related articles were found describing cases of STPCs. Clinical and pathologic information was collected for all of the reported pancreatic cysts, and a database was formed. STPCs are detected more frequently in women than in men. The mean age of occurrence is 43.2 years, and the mean cyst size is 5.6 cm. Fifty percent of true cysts are located in the head of the pancreas. Size and site are responsible for the symptoms caused, although 22.8% were asymptomatic. Diagnosis was made postoperatively in all cases by histopathologic studies. No case of malignancy was reported in any STPC.

Conclusions:

STPCs are rare and benign lesions commonly discovered incidentally during abdominal imaging. Surgical treatment is considered the appropriate therapy for large and symptomatic STPCs. The definitive diagnosis is established by histopathologic and immunohistochemical studies.     

A New Procedure for Treating a Sebaceous Cyst: Removal of the Cyst Content with a Laser Punch and the Cyst Wall with a Minimal Postponed Excision

Three main techniques are used to excise sebaceous cysts: conventional wide excision, minimal excision, and punch biopsy excision. A new method with two steps is proposed. First, a laser is used to make a small hole for removal of the content. Then the cyst wall is removed entirely with a minimal excision about 1 month later. With this method, the cyst is completely removed with only a small scar. It offers a good alternative for eradication of uninfected cysts, especially large cysts or cysts located in areas of thick skin or cosmetic concern.     

MESENTERIC CYST

Mesenteric cyst is one of the rarest abdominal tumours, with approximately 820 cases reported since 1507. The incidence varies from 1 per 100000 to 250000 admissions. The lack of characteristic clinical features and radiological signs may present great diagnostic difficulties. The cyst may present in one of three ways: (i) non-specific abdominal features; (ii) an incidental finding; or (iii) an acute abdomen. Abdominal pain is the major presenting symptom. Abdominal mass is found in more than 50% of cases and 40% of cases are discovered incidentally. More than one aetiological mechanism is probably involved in the development of mesenteric cysts. Mesenteric cysts have been reported from the duodenum to the rectal mesentery but are most commonly located in the ileal mesentery. Malignant cysts occur in less than 3% of cases. Enucleation of the cyst is the treatment of choice. Knowledge of these tumours is important due to the various complications associated with suboptimal surgical management. Two cases of mesenteric cysts are presented including a recurrent mesenteric cyst in a post-partum woman demonstrating the inferior technique of internal marsupialization. The association of mesenteric cyst with pregnancy is discussed.     

Huge echinococcal cyst of the liver managed by hepatectomy: Report of two cases

IntroductionEchinococcocal cysts are predominantly located in the right liver. They are usually solitary and asymptomatic, but large cysts can cause compression symptoms.Case reportsWe report two cases of huge (25 cm and 20 cm in diameter, respectively) echinococcal cysts located in the left liver, which presented as a large palpable mass causing compression symptoms. Diagnosis was established with CT scan showing a cystic mass with the characteristic daughter cysts and reactive layer (pericystic wall) consisting of fibrous connective tissue and calcifications. Both patients were treated radically with left hepatectomy and had uneventful postoperative course and no recurrence upon follow-up.DiscussionThe treatment of liver echinococcal cysts represent a unique surgical challenge. Even though conservative approaches are less technically demanding, the radical approach with resection has better outcome with less recurrences, when performed by experienced surgeons.ConclusionResection rather than drainage is the management of choice for such huge liver echinococcal cysts.     

Bilateral dentigerous cysts and ectopic teeth in the maxillary sinuses: A case report and literature review

IntroductionDentigerous cysts are the second most common form of benign odontogenic cysts. These cysts are commonly single lesions. Bilateral and multiple dentigerous cysts are very rare.Case presentationThis article presents a rare case report of bilateral dentigerous cysts associated with two ectopic teeth located atypically in the maxillary sinuses in a 19-year-old male manifesting as bilateral upper jaw pain, post-nasal discharge and recurrent sinusitis which was managed via the endonasal endoscopic approach by enucleation of the cysts and teeth removal.ConclusionThis paper presented our experience in the management of a rare case of bilateral dentigerous cysts associated with ectopic teeth in the maxillary sinuses. Ct scan of the paranasal sinuses confirmed the diagnosis and the endonasal endoscopic approach is superior to the external approaches in preserving physiological function in managing these patients.     

Laparoscopic management of an isolated primary omental hydatid cyst: A case report and literature review

Primary isolated omental hydatid cysts are extremely rare. Usually, omental hydatid cysts are secondary, either resulting from spontaneous spread of cysts or occurring after operations involving hydatidosis in other regions. Here, we report a case of a primary isolated omental hydatid cyst treated with a laparoscopic approach.We report a 16-year-old female who admitted to hospital with right upper quadrant pain. Abdominal ultrasonography and computed tomography (CT) revealed a cyst located between the liver and right kidney. The cyst was enucleated from the omentum and removed with a laparoscopic approach. Histopathologic examination revealed a hydatid cyst. There was no recurrence during 3 years of follow-up. Primary isolated omental hydatid disease should be considered in patients in endemic regions with intra-abdominal cysts. Laparoscopic surgery seems to be effective and safe for hydatid cysts in accessible locations.     

Laparoscopic management of benign cystic lesions of the liver

We present our experience in the laparoscopic management of benign liver cysts. The aim of the study was to analyze the technical feasibility of such management and to evaluate safety and outcome on follow-up. Between September 1990 and October 1997, 31 patients underwent laparoscopic liver surgery for benign cystic lesions. Indications were: solitary giant liver cysts (n = 16); polycystic liver disease (PLD; n = 9); and hydatid cysts (n = 6). All giant solitary liver cysts were considered for laparoscopy. Only patients with PLD and large dominant cysts located in anterior liver segments, and patients with large hydatid cysts, regardless of segment or small partially calcified cysts in a safe laparoscopic segment, were included. Patients with cholangitis, cirrhosis, and significant cardiac disease were excluded. Data were collected prospectively. The procedures were completed laparoscopically in 29 patients. The median size of the solitary liver cysts was 14 cm (range, 7–22 cm). Conversion to laparotomy occurred in 2 patients (6.4%), to control bleeding. The median operative time was 141 min (range, 94–165 min) for patients with PLD and 179 min (range, 88–211 min) for patients with hydatid cysts. All solitary liver cysts were fenestrated in less than 1 h. There were no deaths. Complications occurred in 6 patients (19%). Two hemorrhagic and two infectious complications were noted after management of hydatid cysts. Three patients were transfused. The median length of hospital stay was 1.3 days (range, 1–3 days), 3 days (range, 2–7 days), and 5 days (range, 2–17 days) for solitary cyst, PLD, and hydatid cysts, respectively. Median follow-up was 30 months (range, 3–78 months). There was no recurrence of solitary liver cyst or hydatid cysts. One patient with PLD presented with symptomatic recurrent cysts at 6 months, requiring laparotomy. We conclude that laparoscopic liver surgery can be accomplished safely in patients with giant solitary cysts, regardless of location. The laparoscopic management of polycystic liver disease should be reserved for patients with a limited number of large, anteriorly located cysts. Hydatid disease is best treated through an open approach. Received for publication on Aug. 21, 1999; accepted on Sept. 2, 1999     

Shunting procedures in the management of intracranial cerebrospinal fluid cysts in infancy and childhood

Summary Widely diverging opinions on the optimal therapy for intracranial cerebrospinal fluid cysts (CSF), mainly arachnoid cysts and the Dandy-Walker cysts, exist. Excision of the cyst walls in the treatment of the Dandy-Walker cyst has been replaced by shunting procedures, but the recommended method for primary treatment of arachnoid cysts in childhood is still cyst wall excision. Membrane excision is, however, often complicated by recurrence, subsequently requiring shunting-procedures. In a series of 19 cases primary shunting of intracranial CSF cysts proved to be a reliable method. In those cases where hydrocephalus (ventricular dilatation) is present at the time of the primary operation the ventricles should be shunted as well as the cyst. The catheter from the ventricle and that from the cyst should be connected to the same valve, otherwise an increased risk of intracranial herniation exists. The prognosis for infants and children suffering from intracranial CSF cysts is in general good; in 17 out of 19 cases mental development was normal and in 15 out of 19 motor development was normal. The risk of permanent motor damage seems to be particularly high when an arachnoid cyst is located on the quadrigeminal plate.     

Large bronchogenic cyst of stomach: A case report

IntroductionBronchogenic cysts are congenital cysts arising as an abnormal budding from primitive tracheobronchial tree. They are lined by pseudostratified columnar or cuboidal ciliated epithelium and contain smooth muscle fibers, submucosal bronchial glands and/or cartilage. They are most frequently located in the mediastinum or the lung parenchyma. Intramural occurrence of bronchogenic cyst in the gastric wall is very rare.Presentation of caseWe present a case of 65-year-old lady with a 7 × 8 cm lesion in the gastric cardia suspicious of gastrointestinal stromal tumor. Because of the large size, total gastrectomy with Roux-en-Y esophagojejunal anastomosis was performed. The postoperative course was uneventful. Histopathological examination revealed a sub-mucosal cyst lined by PCCE with presence of smooth muscle fibers and focal mucous glands. Final diagnosis of bronchogenic cyst was made. On the last follow up at one year, she was symptom free.DiscussionOn extensive Medline/Pubmed search, only 38 cases of gastric bronchogenic cysts were found to be reported till date. They are typically located in the posterior gastric wall close to the gastric cardia. On radiological imaging, they appear as well defined intramural cystic lesion without any characteristic features. Surgical resection is considered in symptomatic cases or in case of diagnostic dilemma.ConclusionGastric bronchogenic cysts often mimic gastrointestinal stromal tumor on preoperative imaging. They should be included in the differential diagnosis while dealing with an intramural gastric lesion close to the cardia or gastroesophageal junction.     

“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.     

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.     

Excision of an asymptomatic cervical intradural neurenteric cyst through the anterior approach: a study of two cases and a review of the literature

BACKGROUND: Spinal neurenteric cysts are very rare lesions, especially after the second decade of life. They account for 0.3% to 0.5% of all spinal tumors and occur most commonly in ventral locations. The cysts are usually removed via a posterior approach. PURPOSE: To present the clinical and radiologic results of patients with spinal neurenteric cysts who were treated via anterior approach procedures. STUDY DESIGN: This report is composed of two cervical neurenteric cyst cases that are compared with published studies. METHODS: We present two patients, 41- and 39-year-old women, each with a cervical intradural neurenteric cyst. Both of these patients had apparent neck pain without neurological deficit. MRI revealed neurenteric cysts located at C7 and C7-T1 levels. After anterior corpectomy, the intradural cysts were removed, and then fusion was performed. RESULTS: The postoperative period went well. The follow-up cervical MRI studies were performed at 3, 6, and 18 months postoperatively, and there were no abnormalities found. CONCLUSIONS: This study has led to the conclusion that although neurenteric cysts are rare in adults, they can still be present with only persistent neck pain and without neurological deficits. This may lead to misdiagnosis. The importance of MRI is not controversial in the early diagnosis. An anterior approach may be considered the first preference for surgical technique in patients with ventrally located neurenteric cysts.     

Paraovarian Cysts of Neoplastic Origin Are Underreported

Introduction:

We suspected that paraovarian cysts of neoplastic origin may be underreported. This study was designed to evaluate our data on the pathologic characteristics of cystic lesions located in the paraovarian area and compare them with previous studies that claimed the vast majority of these lesions were simple paraovarian cysts and only few (1.69% to 5%) were neoplastic ones.

Methods:

This is a retrospective analysis of the clinical, surgical, ultrasonographic, and pathologic features of 59 women operated on for cystic paraovarian lesions at our institution from January 2002 to April 2006.

Results:

Forty-four women (74.6%) had simple paraovarian cysts, and 15 (25.4%) had benign neoplastic paraovarian cysts (7 cystadenomas and 8 cystadenofibromas). There were no cases of malignant tumor. There was no difference in the clinical presentation of the women with either type of cyst. Preoperative ultrasound examinations (n=50) demonstrated more complex cysts with internal papillary projections in the group with neoplastic paraovarian cysts (41.7% compared with 7.9%, P=0.01). The macroscopic pathologic examinations revealed a significantly increased percentage of gross papillary excrescences in the group of neoplastic paraovarian cysts (10/ 15, 66.7%) compared with the group with simple paraovarian cysts (3/44, 6.8%, P<0.01). Other pathologic features did not differ between the 2 study groups.

Discussion:

Our analysis revealed a higher percentage of paraovarian cysts of neoplastic origin (∼25%) than the figures quoted in most previous reports.

Conclusion:

Intraoperative inspection for diagnosing the cyst type and more frequent use of endobag devices to avoid spillage of cystic fluid are recommended.     

Bilateral symmetrical cysts in the upper tibiae in a skeletally mature patient: might they be simple bone cysts?

A 55-year-old Japanese woman presented with right knee pain of 1-month duration. Radiological studies revealed bilateral mild osteoarthritic changes in the medial knee joint compartment and symmetrical cysts in the upper tibial metaphyses, extending to the epiphyses. Intraosseous ganglion was considered the most probable diagnosis. However, intraoperatively, serous fluid-filled cavities were recognized; these were curetted and filled with hydroxyapatite granules. Histopathological examination of the cyst wall revealed thin fibrous tissue formed of collagen fibers without a lining cell layer, with scattered lymphocytes, histiocytes, irregular masses of fibrin-like material, and periosteal osteocartilagenous callus formation; a picture compatible with simple bone cysts. Bilateral symmetrical cysts of the upper tibial metaphyses extending to the epiphyses are extremely rare. A literature review revealed that the age incidence, and bony locations of multiple and epiphyseal simple bone cysts are atypical in relation to the classic metaphyseal simple bone cysts. Also, multiple and epiphyseal simple bone cysts have a better prognosis than the classic metaphyseal ones. Four clinicoanatomic varieties of simple bone cysts are recognized; classic metaphyseal, nontubular, epiphyseal, and multiple.     

Case report. An unusual cause of a patient presenting with an acutely locked knee: multiple benign fat pad cysts

Benign ganglion (synovial) cysts within the knee are uncommon and especially so when located in the infrapatellar fad pad. They cause many non-specific symptoms but usually present as a swelling. We report the case of a young gentleman with multiple benign synovial cysts within the fat pad causing a locked knee, which was treated with arthroscopically assisted open excision. We can find no previous reports of isolated multiple fat pad cysts causing acute locking of the knee.     

Laparoscopic Management of a Cystic Duct Cyst

Background:

Choledochal cysts are rare cystic dilatations of the biliary tree. Though their cause is uncertain, these cysts are usually referred for surgical resection because of their association with developing malignancy. Traditionally, choledochal cysts have been classified under 5 main types. Not included in this classification are cysts of the cystic duct, a condition that is even rarer, with only 14 cases reported in the literature to date. We describe one such rare case of a cyst of the cystic duct that we successfully treated via laparoscopic resection.

Methods and Results:

A 41-year-old male was found to have a biliary abnormality on a routine follow-up computed tomography (CT) scan for an unrelated medical condition. Further magnetic resonance cholangiopancreatography (MRCP) imaging identified a cystic dilation consistent with a Type II choledochal cyst. Laparoscopic resection was performed using a total of 5 trocars, at which time a cyst of the cystic duct was found instead of the expected Type II choledochal cyst. Intraoperative cholangiography was used as a surgical adjunct to confirm the anatomy, and resection of the cyst was completed without complications.

Conclusions:

Our case adds to the body of reports showing that cysts of the cystic duct, while extremely rare, do occur and need to be recognized. Given the preoperative similarity between cystic duct cysts and other choledochal cysts, proposal for a new “Type VI” category for choledochal cysts may be considered so that clinicians can be prepared for this variation. Once recognized, cysts of the cystic duct can be safely and effectively removed by laparoscopic excision, as we have demonstrated.