Resection of Intracranial Giant Cavernous Malformation: Case Report and Literature Review

Cerebral cavernous malformations are a rare and congenital vascular malformation that can present as a challenge in neurosurgical management. The term “giant cerebral cavernous malformations” still does not have a clear definition in the literature, with a wide variety of results. It is known, however, that there is an association between the size of the cavernoma and postoperative sequelae, especially in those with a size greater than 3 cm in its largest diameter. We present a case report of resection of a giant brain cavernoma measuring approximately 8 cm in its largest diameter, emphasizing on clinical presentation, diagnoses and postoperative evolution. Additionally, we performed a comprehensive review of the existing literature on the subject, addressing the epidemiology, pathophysiology, diagnostic methods, treatment options, and prognosis associated with this condition.

Median Nerve Somatosensory Evoked Potentials in Patients with Chiari Malformation

Abnormal SEP reflects dysfunction of the medial lemniscus and posterior cervical cord. These structures are likely to be affected in Chiari malformation. Therefore, SEP abnormalities may provide valuable information in patients with CM. However, the consistency of SEP abnormality or normality with the damage is a matter of research. Knowing whether median nerve somatosensory evoked potential (SEP) is useful in revealing subclinical damage in patients with Chiari malformation is important in the treatment and follow-up plan of the disease. The aim of this study was to investigate the relationship between median nerve SEP values and the severity of cerebellar ectopia in patients with Chiari type 1 malformation. Median nerve SEP values were obtained from 30 healthy individuals and 146 individuals with Chiari malformation. The cerebellar ectopia degree and McRae line length were measured. SEP values were not significantly different between groups. The McRae line was found to be significantly shorter in the control group than in the Chiari malformation group (p = 0.031). There was no correlation between the degree of cerebellar ectopia and the length of the McRae line (r = 0.002, p = 0.979). Neither cerebellar ectopy degree nor McRae line length had a relationship with SEP values (r = -0.153, p = 0.066;r = -0.056, p = 0.500, respectively). There was no difference in cerebellar ectopy degree or SEP values between the groups with cerebellar ectopy with and without a syrinx (p = 0.899;p = 0.080, respectively). Likewise, McRae line length was not found to be related to the presence of a syrinx (p = 0.139). Median nerve SEP examination was not beneficial for diagnosing asymptomatic-oligosymptomatic Chiari malformation as a subclinical injury, whether accompanied by syringomyelia or not.

Cardiac Malformations in Congenital Hypothyroidism: A Case Report

Introduction: Congenital hypothyroidism is the most common causes of preventable mental retardation. It is associated with other births defects like cardiac malformations. Descriptions in Sub Saharan Africa are rare, justifying the present report. Case Report: We reported the cases of 3 female patients, diagnosed with hypothyroidism, presenting in addition pulmonary stenosis. The diagnosis was late in all the patients and we noticed clinical improvement under levothyroxine. Conclusion: Association congenital hypothyroidism and cardiac defect is not rare. Our patients are female with no history of consanguinity, presenting congenital hypothyroidism with a gland in situ associated with pulmonary stenosis. Systematic screening of other births defects is thus recommended in affected patients.

Sclerotherapy in a case of multiple glomuvenous malformation

In this study, we present a representative case of multiple glomuvenous malformation in a 12-year-old female patient. Approximately five years ago, the patient developed multiple blue-purple papules and plaques on her hypogastrium and right thigh, which progressively enlarged and presented tenderness. Histopathological examination confirmed the diagnosis of glomuvenous malformation. Following two injections of lauromacrogol, a significant improvement was observed in the lesions, including a resolution of tenderness. These findings suggest that sclerotherapy not only exhibits evident therapeutic efficacy but also effectively alleviates pain while addressing both aesthetic and therapeutic concerns.

Giant vascular malformations invading the skull:A case report

BACKGROUND Vascular malformations(VMs)arise as a result of errors in the process of angiogenesis and are usually present at birth,but may not become apparent until after birth.However,giant VMs of the head and face are uncommon,with few reported cases,and the prognosis for their surgical intervention is unclear.CASE SUMMARY A 12-year-old girl was admitted to the hospital with findings of an enlarged right temporal scalp.After admission,computed tomography(CT)angiography of cerebral ateries showed a right occlusal gap and a right temporal artery venous malformation.Furthermore,cerebral angiography showed a right temporal lobe VM with multiple vessels supplying blood.The patient underwent surgery to remove the malformed vessels and the eroded skull.Two hours after the surgery,the patient's right pupil was dilated,and an urgent CT scan of the skull showed a right subdural haematoma under the incision,which was urgently removed by a second operation.After surgery,we gave continuous antibiotic anti-infection treatment,and the patient recovered well and was discharged two weeks later.CONCLUSION Surgical removal of giant haemangiomas is risky and adequate preoperative(including interventional embolisation)and intraoperative preparations should be made.

Appendiceal bleeding caused by vascular malformation: A case report

BACKGROUND Acute lower gastrointestinal bleeding(LGIB)is a common occurrence in clinical practice.However,appendiceal bleeding is an extremely rare condition that can easily be overlooked and misdiagnosed.The preoperative detection of appen-diceal bleeding often poses challenges due to the lack of related guidelines and consensus,resulting in controversial treatment approaches.CASE SUMMARY We presented a case of a 33-year-old female who complained of hematochezia that had lasted for 1 d.Colonoscopy revealed continuous bleeding in the appen-diceal orifice.A laparoscopic appendectomy was performed immediately,and a pulsating blood vessel was observed in the mesangium of the appendix,accor-dingly,active bleeding into the appendicular lumen was considered.Pathological examination revealed numerous hyperplastic vessels in the appendiceal mucosa and dilated capillary vessels.CONCLUSION The preoperative detection of appendiceal bleeding is often challenging,colo-noscopy is extremely important,bowel preparation is not routinely recommend-ed for patients with acute LGIB or only low-dose bowel preparation is recom-mended.Laparoscopic appendectomy is the most appropriate treatment for appendiceal bleeding.

Multimodal imaging for the diagnosis of oligodendroglioma associated with arteriovenous malformation:A case report

BACKGROUND The rare co-occurrence of oligodendroglioma and arteriovenous malformation(AVM)in the same intracranial location.CASE SUMMARY In a 61-year-old man presenting with progressive headaches,is described in this case study.Preoperative multimodal imaging techniques(computed tomography,magnetic resonance imaging,magnetic resonance spectroscopy,digital subtraction angiography,and computed tomography angiography)were employed to detect hemorrhage,cystic and solid lesions,and arteriovenous shunting in the right temporal lobe.The patient underwent right temporal craniotomy for lesion removal,and postoperative pathological analysis confirmed the presence of oligodendroglioma(World Health Organization grade II,not otherwise specified)and AVM.CONCLUSION The preoperative utilization of multimodal imaging examination can help clinicians reduce the likelihood of misdiagnosis or oversight of these conditions,and provides important information for subsequent treatment.This case supports the feasibility of craniotomy for the removal of glioma with AVM.

Ectopic recurrence following treatment of arteriovenous malformations in an adult:A case report and review of literature

BACKGROUND Digital subtraction angiography(DSA),the gold standard for the diagnosis of intracranial arteriovenous malformations(AVMs),can show clean nidus resection,leading to a perceived cure.Most cases of intracranial AVM recurrence have been reported in pediatric patients.The conventional understanding indicates that AVMs arise when abnormal blood vessels develop between the fourth and eighth weeks of embryonic development,which coincides with the typical period of blood vessel formation in the brain.As such,recurrent ectopic AVM are rare in adults.CASE SUMMARY Herein,we present the case of a 31-year-old adult with a history of an intracranial AVM originally diagnosed with a symptomatic de novo cerebellar AVM formation.Recurrence was observed five years following angiographically-confirmed excision of the initial AVM.DSA performed prior to initial AVM resection indicated no cerebellar abnormalities.Moreover,the recurrent arteries exhibited differences in arteries and draining veins.In addition to reporting this case,we analyzed six previously-reported adult patients with similar ectopic recurrent AVMs.These cases are summarized to review and explore the potential causes of ectopic AVM recurrence in adults,which increase the likelihood of acquired AVM.CONCLUSION The clinical course of the reported patients demonstrated the possibility of ectopic AVM recurrence in adults.The median time between the diagnosis of the initial AVM and the occurrence of ectopic recurrent AVM in adults was 11 years(range:5–20 years).Magnetic resonance imaging follow-up for more than 10 years may be required in adult AVM-treated patients.

CT and MRI Findings of Intracranial Cavernous Hemangioma Malformation

Objective:To investigate the computed tomography(CT)and magnetic resonance imaging(MRI)features of cavernous hemangioma malformation(CHM)to enhance diagnostic accuracy.Methods:The CT and MRI findings and clinical information of 23 patients with CHM were retrospectively analyzed.Results:CT examinations were conducted in 7 cases,while MRI was utilized in 23 cases.Additionally,SWI was employed in 5 cases and enhanced imaging techniques were applied in 14 cases.Among the observed lesions,20 cases presented with a singular lesion,whereas 3 cases exhibited multiple lesions.The lesions were located in 8 frontal lobes,6 cerebellums,2 brainstems,6 temporal lobes,1 basal ganglia,3 parieto-occipital lobes,and 2 thalamus regions.The nodules appeared as quasi-circular lesions with clear or well-defined boundaries.They presented as isodense lesions on CT scans,with one lesion showing peritumoral edema.On MRI,T1-weighted imaging(T1WI)demonstrated isointense signals,while T2-weighted imaging(T2WI)showed isointense and hyperintense signals.Additionally,10 lesions exhibited a low signal ring on T2WI.Diffusion-weighted imaging(DWI)revealed nodular or isointense low signals,while susceptibility-weighted imaging(SWI)displayed enlarged areas of low signal.Fourteen lesions underwent contrast-enhanced scanning,with 2 lesions showing no obvious enhancement,1 lesion demonstrating mild to moderate enhancement,and 11 lesions exhibiting significant enhancement.Notably,6 of these enhanced lesions were surrounded by small blood vessels.Conclusion:Cavernous hemangioma malformation is more commonly found in individual cases.CT alone lacks specificity,making it prone to misdiagnosis.A more comprehensive evaluation of cavernous hemangioma malformation can be achieved through a combination of MRI,DWI,SWI,and enhanced examination,providing valuable references for clinical assessment.

Concentration is more important than dose when using intralesional Pingyangmycin injection for treatment of vascular malformations

Vascular anomalies represent a wide spectrum of pathology with different clinico-pathological characteristics. Approximately 60% of them occur in the head and neck region. A variety of therapeutic modalities have been reported to manage vascular anomalies over the years, including surgical resection,

Emergent single-balloon enteroscopy for overt bleeding of small intestinal vascular malformation

A 28-year-old man presented with anemia symptoms and intermittent tarry stool passage for three days. No stigmata of hemorrhage were identified using esophagogastroduodenoscopy, ileocolonoscopy, and contrast-enhanced computed tomography. He then developed massive tarry stool passage with profound hypovolemic shock and hypoxic respiratory failure. Emergent angiography revealed active bleeder, probably from the jejunal branches of the superior mesenteric artery, but embolization was not performed due to possible subsequent extensive bowel ischemia. His airway was secured via endotracheal intubation with ventilator support, and emergent antegrade singleballoon enteroscopy was performed at 8 h after clinical overt bleeding occurrence; the procedure revealed a 2-cm pulsating subepithelial tumor with a protrudingblood plug at the distal jejunum. Laparoscopic segmental resection of the jejunum with end-to-end anastomosis was performed after emergent endoscopic tattooing localization. Pathological examination revealed a vascular malformation in the submucosa with an organizing thrombus. He was uneventfully discharged 5 d later. This case report highlights the benefit of early deep enteroscopy for the treatment of small intestinal bleeding.

Cystic malformation of cystic duct:10 cases and review of literature

AIM:To evaluate cystic malformations of the cystic duct.METHODS:Over a 2-year period,we came across 10 cases of cystic malformation of the cystic duct among patients who were investigated in our radiology department with ultrasonography,multidetector computed tomography,or magnetic resonance imaging for abdominal complaints.Radiological diagnosis of cystic malformation of the cystic duct was made on the basis of a dilated,nonvascular cystic structure near the porta hepatis,and visualization of a clear communication with either the gallbladder,normal caliber cystic duct and/or common bile duct(CBD) on at least one imaging modality.RESULTS:Four of 10 patients had saccular dilatation of the cystic duct.Six patients had fusiform dilatation of the cystic duct,and two of these had only mild fusiform dilatation.Two patients had associated CBD dilatation and one had associated cystic duct calculi and malignancy.CONCLUSION:Cystic malformations of the cystic duct should be recognized as a distinct type of choledochal cyst and should be added as type Ⅵ cyst in Todani's classification.

Detection of Congenital Uterine Malformation by Using Transvaginal Three-dimensional Ultrasound

This study assessed the clinical application of transvaginal three-dimensional ultrasound （3D TVUS） in the diagnosis of congenital uterine malformation. A retrospective study was performed on 62 patients with congenital uterine malformation confirmed hysteroscopically and/or laparoscopically. The patients were subjected to transvaginal two-dimensional ultrasound （2D TVUS） and 3D TVUS. The accuracy rate was compared between the two methods. The accuracy rate of 3D TVUS was （98.38%, 61/62）, higher than that of 2D TVUS （80.65%, 50/62）. 3D TVUS coronal plane imaging could demon- strate the internal shape of the endometrial cavity and the external contour of the uterine fundus. It al- lowed accurate measurement on the coronary plane, and could three-dimensionally show the image of cervical tube, thereby providing information for the diagnosis of some complex uterine malformation. 3D TVUS imaging can obtain comprehensive information of the uterus malformation, and it is superior to 2D TVUS for the diagnosis of congenital uterine malformations, especially complex uterine anomaly.

Neonatal colon perforation due to anorectal malformations: Can it be avoided?

Anorectal malformations (ARM) are common anomalies in neonates. Diagnostic and therapeutic delays in the management of ARM may lead to colonic perforation, and even death. Physical examination of the perineum is often sufficient to diagnose ARM in neonates. Notwithstanding, delayed diagnosis of ARM has become increasingly familiar to surgeons, as evidenced by the number of recent publications on this topic in the literature. In this commentary, we discuss spontaneous colonic perforation due to delayed diagnosis of ARM in neonates, and highlight the importance of early diagnosis in assuring good outcomes with surgical management. At this point, a thorough examination of the perineum during the initial newborn assessment is mandatory, particularly in those patients presenting with abdominal signs or symptoms.

Small intestinal vascular malformation bleeding:A case report with imaging findings

The small intestine is approximately 5-6 m long and occupies a large area in the abdominal cavity. These factors preclude the use of ordinary endoscopy and X-ray to thoroughly examine the small intestine for bleeding of vascular malformations. Thus,the diagnosis of intestinal bleeding is very difficult. A 47-year-old man presented at the hospital 5 mo ago with dark stool. Several angiomas were detected by oral approach enteroscopy,but no active bleeding was observed. Additionally,no lesions were detected by anal approach enteroscopy;however,gastrointestinal tract bleeding still occurred for an unknown reason. We performed an abdominal vascular enhanced computed tomography examination and detected ileal vascular malformations. Ileum angioma and vascular malformation were detected by a laparoscopic approach,and segmental resection was performed for both lesions,which were confirmed by pathological diagnosis. This report systemically emphasizes the imaging findings of small intestinal vascular malformation bleeding.

A child with severe inner ear malformations with favorable hearing utilization and balance functions after wearing hearing aids

Infants with congenital deafness caused by severe bilateral inner ear malformations frequently suffer from severe hearing loss and poor balance. Unfortunately, the use of hearing aids is usually ineffective in recovering hearing, necessitating cochlear implants. We report a case of a 6-year-old boy with congenital deafness and bilateral inner ear malformations(right side, incomplete partition type I [IP-I]; left side, common cavity deformity). Hearing aids had a remarkable effect in this patient, enabling sufficient and favorable hearing recovery such as to allow the patient to engage in daily conversations. Per-rotatory nystagmus was recorded on an electronystagmogram for both right and left rotations in a damped rotational chair test. It is rare for deaf children with severe bilateral inner ear malformation to demonstrate favorable development in hearing and good equilibrium function. Our findings suggest that auditoryevestibular hair cells in this patient may have been partially preserved despite IP-I in the right ear and common cavity deformity of the left ear.

The application of ultrasound in the management of cerebral arteriovenous malformation

Ultrasound is used in the diagnosis, treatment and follow-up of cerebral arteriovenous malformation （AVM）. Several parameters including flow velocity, flow volume, resistance index, pulsatility index, vasomotor reactivity and their influencing factors are reviewed. The applications of ultrasound in the preoperative evaluation, intraoperative monitor and postoperative follow-up of AVM, are summarized. Although some limits exist, ultrasound can provide more reliable information about AVM, if lesions are classified according to their characteristics, compared in different conditions between preoperation and postoperation, feeding and non-feeding side, patients and healthy adults, and if ultrasound method is combined with other examinations and different developed ultrasound techniques. With the appearance and development of new ultrasound technique, its application will be wider in management of AVM..

Diagnosis and treatment of venous malformations in China: consensus document

Venous malformations(VMs) are the most common vascular developmental anomalies. There are many controversies over VMs in Chinese clinical medical practice. Experts on the panel from vascular-anomaly centers in China reviewed the etiology, pathophysiology, epidemiology, classification, clinical presentations, diagnosis, and treatment of VMs. The aim of this consensus document is to provide recommendations for, and assist clinicians and patients in, the diagnosis and treatment of VMs.

Current Controversies and the State of the Art in Endovascular Treatment of Vascular Malformations

Vascular anomalies constitute some of the most difficult diagnostic and therapeutic enigmas that can be encountered in the practice of medicine. The clinical presentations are extremely protean and can range from an asymptomatic birthmark to fulminant。

Clinical Characteristics, Imaging Findings and Surgical Outcomes of Chiari Malformation Type I in Pediatric and Adult Patients

A growing number of children and adolescents are being diagnosed as Chiari malformation type I （CM- I ） for behavioral disorders, developmental delay, seizures, or abnormal orpharyngeal function. The aim of this study was to compare the clinical characteristics, imaging findings and surgical outcomes of CM- I in pediatric and adult patients. Between January 2014 and June 2017, 84 patients with CM- I underwent surgical treatment in our department. We divided the patients into two groups： pediatric group （n=l 1, age 〈18 years） and adult group （n=73, age 〉18 years）. Data on clinical characteristics, imaging findings, surgical outcomes, and prognosis were retrospectively reviewed and compared between these two groups. For clinical presentation, scoliosis （36.4%） and developmental delay （36.4%） were more common in pediatric patients, whereas, sensory disturbance （58.9%） and motor weakness （41. 1%） were more common in adult patients. Imaging findings showed that the incidence of hydrocephalus and craniovertebral junctional abnormalities was significantly higher in pediatric group than in adult group （P〈0.05）. Compared to adult group, pediatric group showed a better improvement or resolution of syrinx and tonsillar herniation after surgical treatments （P〈0.05）. The total Chicago Chiari Outcome Scale （CCOS） score in pediatric patients at the last follow- up was significantly higher than that in adult patients （P=0.002）. In conclusion, the clinical characteristics and imaging findings appeared to be different in pediatric and adult patients with CM- I. The surgical outcomes of pediatric patients were shown to be significantly better than those of adult patients.