Spinal neurenteric cyst. Report of 4 cases and review of the literature

Neurenteric cysts are rare congenital lesions of the spine that are lined with entodermal epithelium. We describe 4 unusual cases of neurenteric cysts in patients aged between 3 and 16 years. The cyst was situated anteriorly in 3 patients and posteriorly in 1. All the patients had varied clinical and radiological features. The child with dorsal neurenteric cyst had stigmata of spinal dysraphism along with other vertebral anomalies. Another child had an associated syringomyelia along with evidence of severe arachnoiditis. All 4 patients underwent laminectomy and total microsurgical excision of the cysts through a posterior approach. The patient with diastematomyelia developed early recurrence of the cyst and underwent reoperation. Three patients showed an excellent recovery after surgery. The child with arachnoiditis failed to recover neurologically.     

Neurenteric cyst with xanthomatous changes in the prepontine area: unusual radiological findings

Preoperative diagnosis of neurenteric cysts can be difficult because the imaging findings of a neurenteric cyst may be similar to those of an arachnoid cyst. The authors report a case of a neurenteric cyst with xanthomatous changes in the prepontine area. This 4-year-old girl was admitted to their institution with intermittent neck pain and vomiting. Computed tomography showed a hypodense mass in the prepontine area. Magnetic resonance imaging showed a cystic lesion measuring approximately 4 x 3 cm. The brainstem was displaced posteriorly, and the cisterns in both cerebellopontine angles were widened. The signal intensity of the cyst was similar to that of cerebrospinal fluid. Adjacent to the basilar artery there was a solid component of the mass that enhanced after administration of Gd. Intraoperatively, the authors found a cystic mass containing clear fluid with a yellowish solid nodule. On the basis of histopathological findings, the lesion was diagnosed as a neurenteric cyst with xanthomatous changes.     

Intramedullary neurenteric cyst presenting as infantile paraplegia: a case and review

The authors report a case of a 3-month-old male child with paraplegia in whom magnetic resonance imaging (MRI) revealed a nonenhancing intramedullary cystic lesion extending from the level of D1 to D7 without any other associated anomaly. Intraoperatively, these findings were confirmed and the spinal cord was found to have splayed circumferentially into a papery thin rim. The patient underwent marsupialization of the cyst with subtotal excision of the cyst wall. Histopathological examination revealed ciliated pseudostratified columnar epithelium consistent with the diagnosis of a neurenteric cyst. Intramedullary neurenteric cysts are rare developmental malformations, and out of the 13 previously reported cases, only 3 were evaluated by MRI. This is the first case report in the literature of an intramedullary neurenteric cyst presenting as infantile paraplegia. In the present report, the embryology, etiopathogenesis, radiological imaging and management of this rare clinical entity are discussed and a detailed literature review is presented.     

Intramedullary cervical neurenteric cyst mimicking an abscess

We describe a cervical intramedullary neurenteric cyst in a 12-year-old male patient who presented with gradual onset and progressively worsening neck pain, spastic quadriparesis and impaired sensation in the C(2) dermatome. MR imaging revealed a well-defined peripherally enhancing cystic intramedullary lesion with a posteroinferior enhancing nodule at the C(2)-C(3) level mimicking an abscess. There was no evidence of spinal dysraphism. The lesion was completely resected through a posterior approach and the patient showed radical improvement in his symptomatology. At follow-up after 3 years, he was asymptomatic and the MR imaging showed no evidence of any residual or recurrent cyst. The case presented here is unique, since a spinal neurenteric cyst showing intense peripheral contrast enhancement mimicking an abscess is unusual. The radiological features, pathogenesis and surgical considerations in cervical intramedullary neurenteric cysts are discussed and the relevant literature is briefly reviewed.     

Extreme lateral approach in a case of acute-onset quadriplegia due to high cervical neurenteric cyst

Spinal neurenteric cysts are very rare intradural developmental lesions, predominantly localized anterior to the cervical cord and arising from misplaced endodermal cells in the 3rd week of embryonic life. An acute onset of symptoms can occur due to hemorrhage, but has not as yet been reported in relation to infection in the cyst. We report an 18-year-old male who presented with a rapid onset of respiratory distress and quadriplegia over a period of 6 h requiring intubation and ventilatory support. There was no respiratory effort with a dense sensory level from the neck. MRI of the cervical spine revealed an intradural extramedullary ventrally located cystic lesion at the C(2-3) level. There was no evidence of systemic infection elsewhere in the body. The lesion was radically excised using an extreme lateral approach as it provided excellent visualization of the cyst, thus permitting safe dissection without retraction of the cord. The cyst was tense and the contents turbid, thick, creamy and mucinous without any evidence of fresh or altered blood. Histopathological examination confirmed the cyst to be a neurenteric cyst. The cystic fluid, which was sent for culture, grew coagulase-negative Staphylococcus aureus. This is the first documented case of rapid neurological deterioration due to infection in a neurenteric cyst. In spite of an early effective surgical management, outcome was poor due to the rapid and profound neurological deficit.     

Intramedullary spinal neurenteric cyst with fluid-fluid level

Spinal neurenteric cysts are rare intradural extramedullary lesions of the spine, commonly located in the cervical and thoracic regions. The majority localize ventral to the spinal cord and are associated with other vertebral anomalies. Here, the authors report a rare case of a 3-year-old boy presenting with a 1-week history of meningismus followed by rapid-onset (over a few hours) paraplegia. Magnetic resonance imaging revealed an intramedullary cystic lesion with a fluid-fluid level in the cervicothoracic region of the spinal cord without associated bony or soft tissue abnormalities. To the best of the authors' knowledge, such clinical and radiological presentation of a spinal neurenteric cyst has never been reported. A brief review of the pertinent literature is presented, and the possible pathophysiology of such a presentation is also discussed.     

Craniocervical neurenteric cyst without associated abnormalities

Spinal neurenteric (NE) cyst is an uncommon congenital cyst and frequently found in the cervical region. The clinical symptoms associated with this entity depend on the site of the lesion and are not typical for all such cysts. A definitive diagnosis can only be made by biopsy and histological examination. MRI can confirm these cystic masses and is the method of choice for their imaging investigation. They are often connected by a fibrous tract, fistula or cleft to structures derived from the primitive gut in the thoracic or abdominal cavities and are commonly associated with anterior spina bifida or other vertebral anomalies. We report a case of craniocervical NE cyst without associated abnormalities and discuss the implications for clinical diagnosis and management by a thorough review of the literature.     

Neurenteric cyst of the upper cervical spine: excision via posterior approach

Neurenteric cyst of the cervical spine is an uncommon congenital lesion and usually associated with many craniocervical anomalies. Solitary neurenteric cyst of the upper cervical spine is very rare. Surgery is the main treatment method. An 11-year-old female child presented with the symptoms of neck pain that radiated to both shoulders and arms. Magnetic resonance imaging of the patient showed a cystic mass lesion ventral to the spinal cord and lying at the level from C1 to C3. She underwent surgical resection via a posterior approach and complete cyst resection was performed. Although removal of a neurenteric cyst through a posterior approach may result in spinal cord injury, this method is easier and safer than the anterior approach, especially in children.     

Surgical experience in symptomatic congenital intraspinal cysts

Symptomatic congenital intraspinal cysts are uncommon but not rare. Since these cysts may have various manifestations, a careful differential diagnosis is needed. We retrospectively reviewed findings in 3 women and 2 men (age range, 10-50 years) with 6 symptomatic cysts of the thoracic or thoracolumbar spine. In addition, we statistically analyzed the patients' Nurick myelopathy grades before and after treatment. Pain and spastic paraparesis were the most frequent manifestations. Radiographs showed the widening of the spinal bony canal in 3 patients with extradural arachnoid cysts. All patients received limited laminectomy and appropriate surgical procedures. Pathology reports indicated neurenteric (n = 1), arachnoid (n = 4), and epidermoid (n = 1) cysts. Their functional status significantly improved by a mean of 2.6 Nurick points (p = 0.0002). Our findings confirm that these cysts have various manifestations. Congenital intraspinal cyst should be included in the differential diagnosis for patients with back pain, radiculopathy, cauda equina syndrome, Brown-Sequard syndrome, or myelopathy. The advent of MR imaging and increased knowledge about the pathogenesis of these cysts have improved the ease and accuracy of their early diagnosis; in addition, postoperative prognoses are excellent if surgery is performed early.     

Concurrent development of a thymic cyst and mediastinal Hodgkin's disease

A case is presented of a young adult female who presented with nodular sclerosing Hodgkin's disease and a mediastinal mass that proved to be a thymic cyst. Prior to this illness, a chest x-ray had been obtained that did not show a mediastinal mass. This case illustrates that thymic cysts may arise simultaneously with mediastinal Hodgkin's disease, rather than as a result of treatment or being present congenitally.     

Mediastinal foregut duplication cysts

Foregut duplication cysts are rare congenital anomalies of enteric origin. In majority of the patients, the diagnosis is made in infancy. The authors report 4 cases of mediastinal foregut duplication cyst in children diagnosed on CT/MRI and confirmed on histopathology. In none of the cases the cysts had intraspinal extension nor heterotopic gastric mucosa.     

小儿原发性纵隔肿瘤和囊肿的诊治

目的 探讨小儿原发性纵隔肿瘤和囊肿的诊断及治疗.方法 回顾性分析79例小儿原发性纵隔肿瘤和囊肿的临床资料.所有病例均经手术和病理证实.结论 本组病例中,小儿原发性纵隔肿瘤和囊肿占同期我科收治的原发性纵隔肿瘤及囊肿的13.96%(79/566).其中神经源性肿瘤21.5%(18/79)、肠源性囊肿15.2%(12/79)、畸胎瘤17.7%(14/79),其他肿瘤和囊肿44.30%(35/79).无临床症状而于查体时发现12例,占15.19%.手术切除74例,手术切除率93.7%(74/79),经治疗后康复出院76例,死亡3例.结论 小儿纵隔肿瘤和囊肿的术前诊断依据胸部X线、CT扫描及超声检查,必要时可以结合MRI扫描判断病变范围,而确诊则依赖于术中所见及术后病理检查.其病情复杂,变化快,可危及患儿生命,除淋巴瘤外宜选手术治疗.

Abstract：

Objective To investigate the diagnosis and treatment of primary mediastinal tumors and cysts in children.Methods The data of 79 patients admitted from 1966 to 2009 with primary mediastinal tumors and cysts were retrospectively reviewed.All patients underwent surgery and pathological examination.Results The primary mediastinal tumors and cysts in children accounted for 13.96% of primary mediastinal tumors and cysts at the same period.In these 79 patients,neurogenic tumors were diagnosed in 18 (21.5%)patients,gastrointestinal cyst was 15.2%(12/79),teratoma was 17.7%(14/79),and the others were 44.30%(35/79).Twenty-two patients without clinical symptoms were diagnosed by health checkup,accounting for 15.2% in all cases.Tumors in 74 patients(93.7%)were resected,and the mortality rate was 3.8%(3/79).Conclusions The preoperative diagnosis of mediastinal tumors and cysts in children primarily bases on chest X ray,CT scan,ultrasonography,and MRI scan in some cases,but the final diagnosis depends on the intraoperative findings and pathologic examination.Except of lymphoma,primary mediastinal tumors and cysts should be treated by surgery appropriately.     

Rare case of congenital cystic adenomatoid malformation associated with polycystic kidney disease

Congenital cystic adenomatoid malformation (CCAM) is a rare pulmonary abnormality that results from aberrant fetal lung development. It about 4-26% of cases it can be associated with other congenital abnormalities. We describe a case of congenital cystic adenomatoid malformation 2 associated with polycystic kidney disease. The association of these two congenital malformations is exceptional. Only four similar cases have been reported in the literature. A 2-year-old girl was referred to the Department of Paediatric Surgery and Oncology Medical University of Lodz with pneumonia and left pneumothorax. For three weeks prior to referral the patient was treated with antibiotics. Chest x-ray revealed hyperinflation of left upper lobe with mediastinal shift to right. Computer tomographic scan of the lung revealed multiple cyst in the left upper lobe, left-site pneumothorax and mediastinal shift to the right. The patient underwent thoracotomy. Intraoperatively, multiple cysts in the left upper lobe were found and left upper lobectomy was performed. Histologic study was compatible with type 2 congenital cystic adenomatoid malformation. Ultrasound examination showed multilocular cysts in both kidneys. The dimensions of the cysts were: MWR4. 54x45x45 mm and 25x21x24 mm on the left and right sides, respectively. Significant increase in cyst size on the left side was observed. Ten months after first hospitalization resection of the cystic lower pole of the left kidney was performed. The presence of even a single renal cyst in a child with CCAM is an indication for further follow up examinations.     

Intraspinal neurenteric cyst containing gastric mucosa: CT and MRI findings

A case of neurenteric intraspinal cyst with vertebral anomalies is reported; the cyst contained gastric mucosa. MRI and CT provided useful information.     

小儿纵隔肠源性囊肿的诊断与治疗

目的：探讨纵隔肠源性囊肿病因、临床诊断及治疗。方法：回顾性分析近20年17例纵隔肠源性囊肿患儿的临床资料。结果：1例为壁内型食道囊肿，16例为纵隔肠源性囊肿，16例中2例同时合并腹腔内的肠重复畸形；临床上多数患儿以呼吸系统症状就诊，12例合并椎体畸形，12例行胸部B超检查，4例行胸部MRI检查，均为边界清晰的囊性肿物，8例行^99TmTc扫描，后纵隔内可见核素^99TmTc浓集灶。结果：分析本组临床资料，得到如下结论：第一，小儿纵隔肠源性囊肿的临床特征有①具有呼吸系统压迫症状，患侧胸廓隆起，呼吸活动度差，呼吸音减弱或消失；②后纵隔囊肿合并椎体畸形；③胸部超声、CT和/或MRI检查，后纵隔存在边缘清楚，内壁光滑的单房性含液囊肿；④^99TmTc扫描发现后纵隔囊肿内有异位的胃肠粘膜。第二，本病的根治方法是手术治疗。     

Respiratory distress due to a posterior mediastinal mass following failed repair of esophageal atresia

Respiratory distress due to masses in the posterior mediastinum have been extensively described, including duplication cysts of the esophagus. Retained esophagus following failed repair of esophageal atresia (EA) and esophageal replacement was described only once. Double atresia and midportion esophageal cyst is also a rare entity. We describe a baby with respiratory distress following esophageal replacement for failed repair of EA with tracheoesophageal fistula who was cured by the removal of an overlooked mediastinal cystic mass of esophageal origin at the age of 16 months.     

A case of neurenteric cyst.

An eight-month-old female infant with a neurenteric cyst situated in the lumbar region is presented because of its rarity and because it can mimic other neurological malformations which occur on the same site. Microscopic examination of the surgically excised cyst revealed Water-Paccini corpuscles, lymphoid follicles, intestinal mucosa and meningeal tissue which originated from two ectoderm and endoderm-germ layers.     

Giant ependymal cyst of the temporal horn -- an unusual presentation. Case report with review of the literature.

Primary benign cystic lesions in the brain are uncommon. However, extracerebral cysts like arachnoid cyst, epidermoid cyst and craniopharyngiomas are fairly common lesions. Also, colloid cyst in the third ventricle, dermoid cyst and endodermal cyst in the extracerebral location are not uncommon. On the contrary, intraventricular ependymal and choroidal cysts in the intraventricular location are infrequent. Surgical intervention is warranted in cysts, which produce a mass effect and raised intracranial pressure. We present an interesting case of a giant intraventricular ependymal cyst in the temporal horn in a 14-year-old boy, who presented with recent onset of headaches and epilepsy. He also had long-standing progressive proptosis of the left eye and left temporal bossing. Excision of this cystic lesion was curative. Interesting clinical and neuroimaging features are presented.     

Congenital pancreatic cyst arising from occlusion of the pancreatic duct

Congenital pancreatic cysts, particularly solitary cysts, are rare causes of abdominal masses in newborns. The etiology of these cysts is unknown and they can vary in location within the pancreas. We present a novel case of an infant with a solitary congenital cyst of the pancreatic duct, itself, as a result of occlusion of the pancreatic duct. Solitary congenital pancreatic cysts are a rare cause of abdominal masses in infants. We recently evaluated a 7-week-old infant with progressive abdominal distension since birth. This child was found to have a large solitary pancreatic cyst. This cyst resulted from occlusion of the main pancreatic duct at the level of the ampulla resulting in cystic degeneration of the pancreatic duct. Based on our review of the literature we believe this is the first documented case of such a cyst.     

Huge dermoid cyst of the spleen

Primary splenic cysts are uncommon lesions of the spleen. Splenic cysts are classified as primary or secondary on the basis of presence or absence of an epithelial lining. The primary cysts are further subdivided as parasitic or non-parasitic. The congenital non-parasitic cysts are rarely met in clinical practice and it constitutes approximately 10% of all splenic cysts. Congenital cysts are true cysts with an epithelial. Secondary cysts are in most cases posttraumatic. Patient at admission had lower abdominal pain and splenic cyst was asymptomatic. In routine abdominal pain investigation we found cyst of the spleen in diameter 2 cm. We made serodiagnostic tests for echinococcosis which were negative. After 3 years she came with left upper quadrant enlargement with tangible abdominal mass, increasing abdominal girth, decrease of appetite and sometimes vomiting. CT scan showed cyst enlargement in diameter 8x6 cm. Operative treatment was necessary and splenectomy was done. However, splenectomy remains a relatively safe procedure, associated with few complications and avoiding any future problems.