Prenatal Diagnosis and Intervention in a Fetal Enterogenous Cyst.

The ultrasound diagnosis of a fetal intra-abdominal cyst is typically established during the second or third trimester in the majority of cases. They primarily arise from the gastrointestinal or genitourinary system during the development of intra-abdominal structure and if isolated may resolve spontaneously. Enteric or enterogenous or enteric duplication cysts, which are congenital developments from the intestine, are most common. This case of an enterogenous cyst is presented because of its extreme rarity, its large size, and the need for prenatal intervention. Early identification and definitive treatment are necessary for proper management of this condition.

Intralobar sequestration associated with gastric enterogenous cyst: gastric lung!!

Intralobar sequestration (ILS) is a rare anomaly that may manifest as recurrent pneumonia or rarely as haemoptysis. The co-existence of ILS with enterogenous cyst is extremely rare with only a single case reported. We describe a 6-month-old male infant, with haemoptysis. Evaluation revealed a consolidated segment of the left lung with a pseudoaneurysm of the pulmonary artery and spina bifida. Thoracoscopic excision of the lesion revealed ILS. Recurrent haemoptysis led to re-evaluation and thoracotomy for excision of the cyst which contained gastric mucosal content. We report a rare case of concomitant ILS with an enterogenous cyst and review the relevant literature.

Surgical Experience and Management of Intracranial Neurenteric Cysts: Single-Center Experience and Review of the Literature.

Introduction Neurenteric cysts (NECs) are rare, congenital lesions lined by endodermal cell-derived columnar or cuboidal epithelium. Based on previous studies, gross total removal of the capsule has been presumed to be the ideal surgical goal. Objective This series was undertaken to further understand the risk of recurrence based on the extent of capsule resection. Methods Records were retrospectively reviewed for all patients with radiographic or pathological evidence of intracranial NEC from 1996 to 2021. Results A total of eight patients were identified; four of eight (50%) presented with headache, and four had signs of one or more cranial nerve syndromes. One patient (13%) presented with third nerve palsy, one (13%) had sixth nerve palsy, and two (25%) with hemifacial spasm. One patient (13%) presented with signs of obstructive hydrocephalus. Magnetic resonance imaging demonstrated T2 hyper- or isointense lesions. Diffusion-weighted imaging was negative in all patients (100%) and T1 contrast-enhanced imaging demonstrated minimal rim enhancement in two patients (25%). In three of eight (38%), a gross total resection (GTR) was achieved, while in four (50%), a near-total resection, and in one (13%), a decompression was performed. Recurrences occurred in two (25%) patients, one with decompression and another with near-total resection, among these 1/2 required repeat surgery after a mean follow-up of 77 months. Conclusion In this series, none from GTR group demonstrated recurrence, while 40% of those receiving less than GTR recurred, underpinning the importance of maximally safe resection in these patients. Overall patients did well without major morbidity from surgery.

An Unusual Case of Neurenteric Cyst in a Patient with Split Cord Malformation.

Neurenteric cyst in a split cord malformation is a rare finding. We report an adult female becoming acutely symptomatic secondary to an expanding neurenteric cyst, though previous imaging had demonstrated stability. We discuss our workup and management with surgical resection and possible etiologies of her acute decline.

A case report of a giant ileocecal cystic prolapse through the anus and literature review.

Intussusception refers to the invagination of a proximal loop of the bowel into an adjacent distal segment. This condition is rare in adults, especially when it involves a complete folding of the ileocecal area out of the body cavity. Meanwhile, enterogenous cysts are congenital malformations that are largely identified in childhood following symptoms of bowel obstruction. While surgical treatment is ultimately required for both diseases, deciding on the type of surgery and the right time to operate can be a challenge for clinicians. It is especially difficult to decide on treatment for an adult with the coincidental occurrence of both conditions and no definitive pathologic diagnosis prior to surgery. Here, we present the case study of a 19-year-old female patient who presented with a prolapsed anus due to intussusception caused by a large ileocecal mass. The patient was admitted to the emergency department with a "massive anal mass." She remained symptomatic after receiving conventional conservative treatment and had to undergo emergency surgery after developing an intestinal obstruction. While the patient's intraoperative condition also confirmed the preoperative CT findings, the situation became more complicated during surgery. The postoperative pathological report indicated the presence of an enterogenous cyst. After recovery from surgery, the patient was successfully discharged. Intussusception or intestinal obstruction caused by an intestinal mass is a surgical indication, and removal is the only way to cure the condition. This case study provides a helpful reference for general surgeons, especially anorectal surgeons, imaging physicians, and pathologists, and informs the diagnosis and treatment of this patient population.

Adenocarcinoma arising in an ectopic enterogenous cyst: A rare case report and review of literature.

Enterogenous cyst (EC) is a rare congenital lesion generally located in the central nervous system, such as in the cerebral hemispheres, posterior fossa, or spinal canal. They are usually benign lesions, and malignant transformation is rare. A 42-year-old woman felt an obvious pain in the lump and went to a local hospital for local lumpectomy. After 7 months, she again felt pain in the buttocks and difficulty in urinating and defecation. The computed tomography (CT) scan showed a mass in the pelvis. Sacrococcygeal cyst excision was performed 10 days later, and postoperative pathology showed epidermoid cyst. Shortly after, the patient recovered and was discharged from the hospital; the pain in the buttocks continued to recur. Puncture and drainage were performed five times. Later, the patient went to our hospital for treatment, and pelvic MRI showed multiple abnormal signal shadows in the presacral and sacrococcygeal regions, some of which were considered abscesses, and some were cystic lesions. She underwent tumor resection and was diagnosed with EC with locally moderately differentiated adenocarcinoma. Four months later, the patient's symptoms of swelling and pain recurred. MRI examination showed multiple high-signal T2 shadows in the anterior sacral and subcutaneous tissues of the buttocks, and enhanced scan showed partial marginal enhancement. After assessment, the patient was given a radiation dose of 60 Gy/25F. ECs in the anterior sacral and soft tissue of the buttocks are very rare, and the case of carcinomatous transformation has never been reported. Therefore, we discussed the clinicopathological features of ectopic ECs and reviewed the literature.

Supratentorial Neurenteric Cysts: Systematic Literature Review and Case Report.

BACKGROUND: Neurenteric cysts (NC) are uncommon congenital lesions with histopathologic properties derived from the gastrointestinal or respiratory tract. They are typically located in the intradural extramedullary compartment but rarely seen in the supratentorial region. The occurrence of supratentorial NCs (S-NC) presents an interesting quandary regarding their embryopathogenesis. METHODS: We present a case report and systematic literature review on S-NCs following PRISMA guidelines. RESULTS: A 57-year-old woman presented with a seizure and paresthesias of the face, hands, and feet. Magnetic resonance imaging showed a right temporo-occipital cystic lesion, which was managed with surgical resection. Histologically, the cyst was type A. The patient was without recurrence at 10 months. Including this case, 88 S-NCs have been reported in the literature. Common presenting symptoms are headaches and seizures. They were mostly treated with craniotomy, preferably with gross total resection, although subtotal resection may be necessary because of adhesions. Resection usually led to symptom improvement (61%). Malignant transformation was seen in 3%. Recurrence was seen in 17%, with a mean time to recurrence of 4.2 years, and was significantly more common after subtotal resection than gross total resection. CONCLUSIONS: If surgically resected, the cyst wall specimen should be sent for pathology review, because of the potential risk for malignancy. If conservatively managed, serial imaging is warranted to track for changes that may indicate transformation. The embryopathogenesis of these rare congenital lesions remains incompletely understood, but the most comprehensive theory involves enteric cell migration to the neuroectoderm during embryogenesis.

Tubular Ileal Duplication Causing Perforative Peritonitis in an Adult: A Rare Presentation.

Background Enteric duplication cysts are a rare congenital abnormality that present more commonly in children than adults. Clinical presentation varies from vague abdominal pain, abdominal lump, iron deficiency anemia to intestinal obstruction due to intussusception or mass effect. We report a tubular ileal duplication in an adult male presenting with an acute abdomen due to perforative peritonitis. Case A 20-year-old male presented to the emergency department with complains of right lower abdominal pain. On clinical examination and ultrasound scan patient was suspected to have a complicated acute appendicitis (rupture). However, a computed tomography scan was suggestive of perforation in the distal ileum. Emergency exploratory laparotomy revealed a perforated isolated ileal tubular duplication.

Anterior cervical transvertebral approach for resection of an intraspinal ventral lesion: illustrative case.

BACKGROUND: The anterior cervical corpectomy and fusion approach has been reported for the removal of ventral cervical tumors. However, the normal cervical vertebral body and the adjacent intervertebral discs have to be sacrificed. In this paper, the authors describe a novel anterior cervical transvertebral approach for the resection of cervical intraspinal ventral lesions. OBSERVATIONS: A patient presented with an anteriorly placed extramedullary cyst. An anterior cervical transvertebral open-window and close-window approach was designed and applied to resect an intraspinal ventral enterogenous cyst. With this novel technique, a square was cut through the whole vertebral body at the four sides. After the cyst resection, the bone block was restored and fixed with a titanium miniplate. The lesion was totally resected, and the compression of the spinal cord was relieved. The physiological function of the cervical spine was kept intact after the operation. There was no postsurgical complication. The cervical alignment was normal at the 1-year postoperative follow-up. LESSONS: The anterior cervical transvertebral open-window and close-window approach was developed and confirmed to be effective for the resection of cervical intraspinal lesions. The cervical physiological structure and function can be restored with this new technique.

Conservative management of recurrent enterogenous cysts of the cervical spine: A case report.

Enterogenous cysts are rare benign congenital tumours of the central nervous system. The aim of management is complete resection to minimise the chance of recurrence. To date, management of recurrence has favoured further surgical resection. We describe the case of a recurrent enterogenous cyst of the cervical spine, initially treated with decompression via laminectomy and fenestration. Magnetic Resonance Imaging (MRI) follow up has demonstrated spontaneous recurrence and deflation of the cyst on multiple occasions. We propose that conservative management of recurrent enterogenous cysts may be a valid management option following fenestration or partial resection of the cyst, and that recurrence may not always warrant further surgical intervention.

Intracranial neurenteric cyst: A case report and differential diagnosis of intracranial cystic lesions.

Neurenteric cysts are uncommon, benign lesions that are rarely located intracranially. These cysts are likely due to aberrant embryologic development of the notochord. Clinically, neurenteric cysts may present with symptoms of mass effect, or they can be asymptomatic and incidentally discovered. Imaging features of neurenteric cysts have significant overlap with other intracranial cystic lesions, which can make diagnosis difficult. We present a case of a 35-year-old female with a histopathologically confirmed neurenteric cyst in the premedullary and left cerebellomedullary cistern, with associated symptoms of headache, dizziness, tinnitus, and dysphagia. The patient underwent surgical resection, with improvement in symptoms. We present a review of literature, and a discussion of typical features of multiple intracranial cystic lesions. We hope to promote accurate preoperative diagnosis, to allow for appropriate surgical technique to reduce the risk of recurrence.

Endodermal Cysts of the Central Nervous System: Review of the Literature and a Case Report.

CONTEXT: Endodermal cysts are rare benign developmental cysts lined by mucin-secreting and/or ciliated, cuboidal, or columnar epithelium of probably endodermal origin. AIMS: Endodermal cysts are rarely intracranial, frequently located in the posterior fossa. Supratentorial location is the most infrequent and only few cases are reported in the literature, included our case. SETTINGS AND DESIGN: The authors report a case of intracranial supratentorial endodermal cyst with a review of the literature. SUBJECTS AND METHODS: A 40-year-old woman was admitted to our department because of progressive gait disorder for 3 months due to right brachial and crural motor deficit associated to right crural sensory disorder (tactile hypesthesia) and right Babinski response at neurological examination due to an endodermal cyst located in the left frontoparietal convexity. DISCUSSION: Total resection of endodermal cysts is recommended, despite their location and adhesion to the surrounding structures, due to its high risk of recurrence. Fenestration of the cystic content into the subarachnoid cistern may cause obstructive hydrocephalus or chemical meningism. RESULTS: Although rare, surgeons should be aware that these lesions must be differentiated clinically, radiologically, and histologically from other supratentorial cystic lesions.

A rare case of thoracic spinal intradural extramedullary enterogenous cyst with acute onset: case report and literature review.

The intraspinal enterogenous cyst, also called an neurenteric cyst, is a rare congenital disease. It was reported to be local to the C1 to L2 spinal segments, with the majority located in the cervicothoracic region. Most patients present with symptoms of progressive focal pain, myelopathic signs or radicular symptoms. We report a rare case of thoracic spinal intradural extramedullary enterogenous cyst with rapidly progressive weakness of both lower extremities. Additionally, we analysed the literature concerning the clinical features, diagnosis and prognosis of this disease.

Supratentorial neuroenteric cyst in children: a case report and brief literature review.

Neurenteric cysts are rare developmental lesions typically found outside the central nervous system but when they do, we most likely find them in a spinal (cervical or dorsal) intradural extramedular location, often associated with dysraphism. The more unusual intracranial cases have been published because of its rarity, occurring mostly as a posterior fossa extra-axial cyst, in adults. Supratentorial cases are distinctly infrequent, especially in children, resulting in few case reports and even fewer case reviews. We describe a case of a child with a supratentorial neurenteric cyst and present a brief review of the literature about these cysts in children, a noticeable gap in the literature.

Enterogenous Cyst Presenting as Mediastinal Mass in a Newborn.

Duplication cyst is a rare congenital malformation. Enterogenous cyst are foregut duplication cyst with or without a vertebral defect. We report a case of a 36hr old neonate with mediastinal enterogenous cyst associated with vertebral defects. The embryology, differentials, and management of enterogenous cyst in the newborn have been discussed.

Enterogenous cyst of the small bowel mesentery: a case report and review of the literature.

INTRODUCTION: Enterogenous cysts are a very rare congenital abnormality that can be found anywhere within the gastrointestinal tract, most commonly in the small intestine. The incidence is approximately one in 4500-10,000 live births. Diagnosis can be suggested by ultrasound (US), computed tomography (CT) scans or magnetic resonance imaging (MRI) findings, although histological examination confirms the definitive diagnosis. PATIENTS: We present a case of enterogenous cyst in an adult female who underwent a resection of the tumour. RESULTS: After two years of observation, there is no evidence of tumour recurrence.

Recurrent neurenteric cysts compressing the brainstem.

BACKGROUND: Neurenteric cysts, also referred to as endodermal cysts and enterogenous cysts, are rare lesions of the neuroaxis occurring most frequently within the spinal cord and rarely intracranially. In the literature describing these lesions, examples of intraoperative imaging and cytology preparations are rare to non-existent. CASE DESCRIPTION: Here, we describe a case of a recurrent posterior fossa neurenteric cyst compressing the brainstem in a 47-year-old female and causing incontinence and progressive quadriparesis. Intraoperative findings and cytologic and histologic features are presented. CONCLUSION: Neurenteric cysts are generally considered to be benign and slow-growing though recurrence is common. This case of a recurrent neurenteric cyst is illustrated by intraoperative macroscopic and cytologic images.

Giant Craniospinal Intramedullary Neurenteric Cyst in Infant-Case Report and Review of Literature.

BACKGROUND: Neurenteric cysts are rare congenital lesions derived from an anomalous connection between the primitive ectoderm and endoderm. CASE DESCRIPTION: A 2-month-old infant presented with an insidious onset of upper limb weakness. Magnetic resonance imaging of the spine revealed a contrast-enhancing solid mass located anterior to the spinal cord at the level of the cervicothoracic junction, which was isointense on T1-weighted images and hyperintense on T2-weighted images. The lesion was associated with marked spinal cord compression and also with a giant intramedullary cyst that extended proximally into the cranium. Spinal dysraphism was noted. The patient underwent cervicothoracic laminotomy, drainage of the cyst contents, and a complete excision of the solid component. Neurologic function recovery was observed postoperatively. CONCLUSIONS: We report and discuss the clinical presentation, pathogenesis, and neuroradiologic findings in an infantile case of a giant craniospinal intramedullary neurenteric cyst. Early recognition is beneficial because of its benign nature and good clinical outcome following total surgical resection.

Isolated Dorsal Thoracic Neuroenteric Cyst with Spinal Cord Compression: Case Reports in Pediatrics.

BACKGROUND: Neuroenteric cysts are rare developmental anomalies of the central nervous system that account for approximately 0.7%-1.3% of all spinal cord lesions. The majority of spinal neuroenteric cysts are located ventral to the spinal cord. Dorsally located neuroenteric cysts are rare and are almost always associated with other spinal anomalies. Isolated dorsal spinal neuroenteric cysts, without other associated spinal anomalies, are extremely rare. These lesions can be mistaken for the more commonly encountered spinal cysts (e.g., arachnoid cysts, ependymal cysts, cystic teratomas). CASE DESCRIPTION: We present the first pediatric case of a 9-month-old boy with an isolated dorsal thoracic neuroenteric cyst and cord compression that underwent surgical resection. The patient tolerated the procedure well and remained neurologically stable after surgery. Pathology was consistent with a neuroenteric cyst. Complete resection was not achieved at the time of surgery. In retrospect, knowing the lesion is a neuroenteric cyst, a more aggressive approach aiming for a complete surgical resection should have been undertaken. To our knowledge, only 2 adult cases of isolated dorsal spinal neuroenteric cysts have been reported in the literature. CONCLUSIONS: Neuroenteric cysts can occur dorsal to the spinal cord without any associated spinal anomalies. The gold standard treatment for neuroenteric cysts is surgical resection, and the goal of surgery is complete resection.

Enterogenous cyst of pediatric testis: a case report.

BACKGROUND: An enterogenous cyst is a rare entity categorized as an intestinal cyst. In most cases, enterogenous cysts are seen in the mediastinum, peritoneal cavity, spinal canal, subarachnoid space, and cerebral ventricle. CASE PRESENTATION: A 14-year-old Asian (Japanese) boy reported feeling pain in his left groin, and a mass was found. We did not perform orchiectomy because intraoperative frozen sections showed no malignant findings. On histological examination the resected specimens contained columnar epithelium surrounded by smooth muscle. Based on these findings, an enterogenous cyst was diagnosed. Few cases of enterogenous cysts of the testis have been described. CONCLUSION: We encountered a case of an enterogenous cyst of a pediatric testis.