RESUMO
BACKGROUND Conjoined twins are a rare congenital anomaly with various types, depending on the connection site. Managing these cases requires a structured approach leading to separation surgery. This report provides a detailed description of the anesthetic strategies and challenges of pygopagous conjoined female twins who underwent 2 surgeries before separation at 17 weeks of age. CASE REPORT The female twins were conjoined at the sacral level (S4), with fused thecal sacs at L5 and cord tethering, with the conus terminating below L3 in both patients. They had separate rectal ampullae that fused into a single anal canal in the midline in a Y formation. The babies underwent 2 surgeries prior to separation: meningocele repair for twin B and anal canal dilation and sphincter mapping for both twins, followed by a ventriculoperitoneal shunt insertion for twin B. The separation surgery included dividing the bony vertebral fusions and dural sacs, untethering the spinal cords and nerves, and correctly allocating the intrapelvic muscles, guided by sphincter muscle mapping. Anesthesia was managed by 2 distinct teams each time, with duplicated equipment and color-coded medications to prevent errors. Due to the critical condition of twin B, general anesthesia was administered to her first. The report also addresses the challenges faced during the 3 surgical procedures. CONCLUSIONS Anesthetic management for pygopagus twins presents complex challenges. Despite limited experience with similar cases, successful management was achieved through planning, effective communication, and rehearsal of unfamiliar setups. Attention to detail and involvement of highly experienced teams were crucial to the success of the procedures.
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Gêmeos Unidos , Humanos , Gêmeos Unidos/cirurgia , Feminino , Lactente , Meningocele/cirurgia , Anestesia Geral , Derivação VentriculoperitonealRESUMO
ABSTRACT: Presacral tumours are rare developmental tumours associated with midline closure defects. A fifteen-month-old girl presented with pain in the lower abdomen and dysuria. After examination, the child underwent investigations and was diagnosed as a presacral mass with intergluteal extension and caudal mass in the subcutaneous tissue of the left gluteal region. Complete excision of mass along with coccygectomy was done. Histopathology showed presacral as a keratinous cyst and gluteal as a dermoid cyst. Later on, the child also developed a left facial dermoid cyst which was also excised.
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Cisto Dermoide , Meningocele , Humanos , Cisto Dermoide/diagnóstico , Cisto Dermoide/cirurgia , Feminino , Diagnóstico Diferencial , Lactente , Meningocele/diagnóstico , Meningocele/cirurgia , Imageamento por Ressonância Magnética , Sacro/cirurgiaRESUMO
Background: Posterior fossa craniotomy is commonly performed for various pathologies. However, intra-cranial infection following craniotomy causes morbidity. Pseudomeningocele is one of the main complications following posterior fossa operation. This study aimed to test the hypothesis that the risk of intra-cranial infection is increased in patients who undergo posterior fossa craniotomy with pseudomeningocele compared with those without pseudomeningocele. Methods: We retrospectively analyzed the data of patients undergoing posterior fossa craniotomy for the management of neurological pathologies at our institute from 2011 to 2020. A total of 308 craniotomies were included, and the primary outcome of interest was the occurrence of intra-cranial infection. Standard statistical methods were used to explore associations between several parameters, including pseudomeningocele, intra-cranial infection, and wound leak. Results: Of the 308 craniotomies, 41 (13.3%) developed intra-cranial infection and 59 (19.2%) involved pseudomeningocele. Of cases involving pseudomeningocele, 27 (45.8%) developed an intra-cranial infection compared with only 14 of 249 without pseudomeningocele (5.6%; p < 0.001). In the multi-variable analysis, pseudomeningocele was associated with intra-cranial infection (odds ratio [OR] 8.56; 95% confidence interval [CI] 3.145-23.299; p < 0.001) and wound leak (OR 91.339; 95% CI 10.437-799.364; p < 0.001). Conclusion: The findings indicate that patients undergoing posterior fossa craniotomy are at a greater risk of intra-cranial infection if there is pseudomeningocele after the operation.
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Fossa Craniana Posterior , Craniotomia , Infecção da Ferida Cirúrgica , Humanos , Estudos Retrospectivos , Masculino , Feminino , Craniotomia/efeitos adversos , Pessoa de Meia-Idade , Fossa Craniana Posterior/cirurgia , Adulto , Infecção da Ferida Cirúrgica/epidemiologia , Infecção da Ferida Cirúrgica/etiologia , Idoso , Meningocele/etiologia , Meningocele/cirurgia , Fatores de Risco , Adulto Jovem , Complicações Pós-Operatórias/epidemiologiaRESUMO
We report on an elderly woman with sciatica due to disc herniation, experiencing complete pain resolution following surgery. Four weeks later, she developed refractory, excruciating pain with foot palsy, prompting a lumbar magnetic resonance imaging that revealed no recurrent disc herniation. Upon exploration, intermittent nerve entrapment through the dural tear and pseudomeningocele was identified. Following dural repair and nerve repositioning, the postoperative course and long-term outcome were uneventful.In contrast to recurrent disc herniation, sciatica resulting from nerve entrapment by a pseudomeningocele is exceedingly rare. Its differential diagnosis is crucial in cases with seemingly unspectacular magnetic resonance imaging findings, as paralysis can occur in symptomatic patients. The radiological presence of a postoperative pseudomeningocele could be overlooked, and the cause of sciatica might become apparent solely during surgical exploration.
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Deslocamento do Disco Intervertebral , Imageamento por Ressonância Magnética , Meningocele , Ciática , Humanos , Feminino , Ciática/etiologia , Diagnóstico Diferencial , Meningocele/cirurgia , Meningocele/diagnóstico por imagem , Deslocamento do Disco Intervertebral/cirurgia , Deslocamento do Disco Intervertebral/complicações , Deslocamento do Disco Intervertebral/diagnóstico por imagem , Deslocamento do Disco Intervertebral/diagnóstico , Idoso , Vértebras Lombares/cirurgia , Vértebras Lombares/diagnóstico por imagemRESUMO
Neurofibromatosis type 1 (NF1) is a multisystem neurocutaneous disorder. Scoliosis and dural ectasia are features of the associated mesodermal dysplasia. Lateral thoracic meningoceles can develop in NF1 and progressively enlarge due to cerebrospinal fluid (CSF) pulsations. Large meningoceles can cause compressive symptoms in the thorax. We are reporting a case of a NF1 presenting with acute onset respiratory distress, who also had chronic orthostatic headaches. CT chest showed unruptured enlarging bilateral lateral thoracic meningoceles causing lung compression. MRI of the brain and spine showed features of CSF hypotension, explaining the headaches. CSF hypotension with unruptured meningoceles is extremely rare. Management of the condition is challenging since surgical removal is prone to complications due to underlying mesodermal abnormalities. Cystoperitoneal shunting to relieve lung compression may worsen CSF hypotension. A shunt with a programmable valve allowed controlled drainage and successfully relieved lung compression without worsening of orthostatic headaches in our case.
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Meningocele , Neurofibromatose 1 , Humanos , Neurofibromatose 1/complicações , Meningocele/diagnóstico por imagem , Meningocele/complicações , Meningocele/cirurgia , Imageamento por Ressonância Magnética , Tomografia Computadorizada por Raios X , Masculino , Feminino , Hipotensão Intracraniana/diagnóstico por imagem , Hipotensão Intracraniana/etiologia , Hipotensão/etiologia , Vértebras Torácicas/diagnóstico por imagem , Vértebras Torácicas/cirurgia , Diagnóstico Diferencial , Doenças RarasRESUMO
BACKGROUND: Neurofibromatosis type 1 is a genetic disease that affects multiple organs and systems, leading to various clinical manifestations. In Neurofibromatosis type 1, rare intrathoracic meningoceles often occur alongside bone dysplasia. These meningoceles contain cerebrospinal fluid and can be mistakenly diagnosed as 'pleural effusion'. CASE PRESENTATION: In this case report, we mistakenly identified 'cerebrospinal fluid' as 'pleural effusion' and proceeded with drainage. This error posed significant risks to the patient and holds valuable implications for the future diagnosis and treatment of similar patients. CONCLUSIONS: In patients with Neurofibromatosis type 1 complicated by spinal deformity, there is a high incidence of intrathoracic meningoceles. Treatment strategies may differ based on the specific features of the lesions, and collaboration among multiple disciplines can significantly improve patient outcomes.
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Erros de Diagnóstico , Meningocele , Neurofibromatose 1 , Derrame Pleural , Humanos , Neurofibromatose 1/diagnóstico , Neurofibromatose 1/complicações , Meningocele/diagnóstico , Derrame Pleural/diagnóstico , Tomografia Computadorizada por Raios X , Masculino , FemininoRESUMO
Thoracic meningocele is a rare medical condition that is usually linked to neurofibromatosis type I.1 Respiratory and neurologic symptoms characterize it.2 Although there have been some improvements in surgical techniques, the condition has a high recurrence rate, with most cases recurring within a year of surgery.3 A 56-year-old woman was observed due to respiratory and pyramidal signs. A chest computed tomography scan and magnetic resonance imaging revealed a thoracic meningocele, occupying the lower sectors of the right hemithorax, communicating with the cerebrospinal fluid space at the T10-T11 level. Multidisciplinary surgery was performed. After selectively intubating both bronchi, the patient was placed in prone position and a posterior median thoracic spine approach was performed. After T10-T11 laminectomy, 3 dural longitudinal incisions were performed. The first incision was placed in the middle to deflate the collection, the second was made on the right side to obtain a complete view of the meningocele, and the third was made on the right lateral side to exclude the meningocele. The lateral dura at the last incision was sutured to the dura propria lining the vertebral body of T11 and T10. The paramedian and median incisions were closed, with Tachosil placed above and below the sutures. Subsequently, the patient was placed in a supine position, the right lung was deflated, and a triportal thoracoscopic approach was performed to dissect and remove the lesion.4 The breach was closed using Tachosil (Baxter Healthcare Corp, Deerfield, Illinois, USA) and fibrin glue. An early 1-month computed tomography and magnetic resonance imaging confirmed the surgery was successful.
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Meningocele , Humanos , Pessoa de Meia-Idade , Feminino , Meningocele/cirurgia , Meningocele/diagnóstico por imagem , Vértebras Torácicas/cirurgia , Vértebras Torácicas/diagnóstico por imagem , Imageamento por Ressonância Magnética , Laminectomia/métodos , Procedimentos Neurocirúrgicos/métodos , Tomografia Computadorizada por Raios XRESUMO
INTRODUCTION: Anterior sacral meningocele is a rare congenital disorder, occurring isolated or in syndromic disease. CASE REPORT: A 15-year-old patient who complained of abdominal pain and urinary dysfunction was managed surgically. Imaging diagnosed a giant presacral meningocele and agenesis of the coccyx. DISCUSSION: The presentation of sacral meningocele can be poorly symptomatic, which is why some patients are diagnosed late. Sometimes, diagnosis is suggested by non-specific abdominal symptoms or complications. Abdominal-pelvic radiological examination and lumbar spine MRI are essential, and treatment must be surgical. There are several surgical approaches, but currently no consensus. CONCLUSION: An unusual huge presacral cystic mass in a young patient may be isolated or part of a syndrome, and can be asymptomatic for a long time, leading to late diagnosis. The surgical approach should be based on multidisciplinary discussion. We operated on a giant anterior sacral meningocele in a child using a posterior approach, with a satisfactory result.
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Meningocele , Sacro , Humanos , Meningocele/cirurgia , Adolescente , Sacro/cirurgia , Imageamento por Ressonância Magnética , Masculino , Cóccix/cirurgia , Procedimentos Neurocirúrgicos/métodos , Feminino , Resultado do Tratamento , Anormalidades Múltiplas , Região Sacrococcígea/anormalidadesRESUMO
PURPOSE: To analyze the relationship between spinal cord and vertebral abnormalities from the point of view of embryology. METHODS: We analyzed the clinical and radiological data of 260 children with different types of spinal cord malformations in combination with vertebral abnormalities. RESULTS: Among 260 individuals, approximately 109 presented with open neural tube defects (ONTDs), 83 with split cord malformations (SCMs), and 83 with different types of spinal lipomas. Pathological spina bifida emerged as the most frequent vertebral anomaly, affecting 232 patients, with a higher prevalence in ONTD. Vertebral segmentation disorders, including unsegmented bars, butterfly vertebrae, and hemivertebrae, were present in 124 cases, with a higher prevalence in SCM. The third most common spinal anomaly group consisted of various forms of sacral agenesis (58 cases), notably associated with blunt conus medullaris, spinal lipomas, and sacral myelomeningocele. Segmental aplasia of the spinal cord had a typical association with segmental spinal absence (N = 17). CONCLUSION: The association between SCM and neuroenteric cyst/canal and vertebral segmentation disorders is strong. High ONTDs often coincide with pathological spina bifida posterior. Type 1 spinal lipomas and focal spinal nondisjunction also correlate with pathologic spina bifida. Segmental spinal absence or dysgenesis involves localized spinal and spinal cord aplasia, sometimes with secondary filar lipoma.
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Anormalidades Múltiplas , Hérnia Diafragmática , Lipoma , Meningocele , Defeitos do Tubo Neural , Disrafismo Espinal , Criança , Humanos , Coluna Vertebral/anormalidades , Medula Espinal/patologia , Meningocele/patologia , Defeitos do Tubo Neural/patologia , Imageamento por Ressonância MagnéticaRESUMO
BACKGROUND: Human studies of genetic risk factors for neural tube defects, severe birth defects associated with long-term health consequences in surviving children, have predominantly been restricted to a subset of candidate genes in specific biological pathways including folate metabolism. METHODS: In this study, we investigated the association of genetic variants spanning the genome with risk of spina bifida (i.e., myelomeningocele and meningocele) in a subset of families enrolled from December 2016 through December 2022 in a case-control study in Bangladesh, a population often underrepresented in genetic studies. Saliva DNA samples were analyzed using the Illumina Global Screening Array. We performed genetic association analyses to compare allele frequencies between 112 case and 121 control children, 272 mothers, and 128 trios. RESULTS: In the transmission disequilibrium test analyses with trios only, we identified three novel exonic spina bifida risk loci, including rs140199800 (SULT1C2, p = 1.9 × 10-7), rs45580033 (ASB2, p = 4.2 × 10-10), and rs75426652 (LHPP, p = 7.2 × 10-14), after adjusting for multiple hypothesis testing. Association analyses comparing cases and controls, as well as models that included their mothers, did not identify genome-wide significant variants. CONCLUSIONS: This study identified three novel single nucleotide polymorphisms involved in biological pathways not previously associated with neural tube defects. The study warrants replication in larger groups to validate findings and to inform targeted prevention strategies.
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Meningocele , Defeitos do Tubo Neural , Disrafismo Espinal , Criança , Humanos , Estudos de Casos e Controles , Bangladesh , Disrafismo Espinal/genéticaRESUMO
OBJECTIVE: To describe the presentations, the diagnosis, our treatment approaches, and the outcomes for 11 patients with fallopian canal meningocele (FCM). STUDY DESIGN MULTICENTER: Retrospective case series. SETTING: Tertiary referral centers. PATIENTS: Patients (N = 11) with radiographically or intraoperatively identified, symptomatic FCM. INTERVENTIONS: Surgical repair of cerebrospinal fluid (CSF) leak and meningocele versus observation. MAIN OUTCOME MEASURES: Presentation (including symptoms, radiographic imaging, and comorbidities), management (including surgical approach, technique for packing, use of lumbar drain), clinical outcomes (control of CSF leak, meningitis, facial nerve function), and revision surgery. RESULTS: Patients presented with spontaneous CSF leak (n = 7), conductive (N = 11) and sensorineural hearing loss (n = 3), nonpositional intermittent vertigo (n = 3), headaches (n = 4), and recurrent meningitis (n = 1). Risk factors in our series included obesity (n = 4), Chiari 1 malformation (n = 1), and head trauma (n = 2). Noncontrast computed tomography of the temporal bone and magnetic resonance imaging were positive for FCM in 10 patients. Eight patients were managed surgically via a transmastoid approach (n = 4), combined transmastoid and middle fossa (N = 3), or middle fossa alone (n = 1); three were managed conservatively with observation. Postoperative complications included worsened facial nerve palsy (n = 1), recurrent meningitis (n = 1), and persistent CSF leak that necessitated revision (n = 1). CONCLUSIONS: Facial nerve meningoceles are rare with variable presentation, often including CSF otorrhea. Management can be challenging and guided by symptomatology and comorbidities. Risk factors for FCM include obesity and head trauma, and Chiari 1 malformation may present with nonspecific otologic symptoms, in some cases, meningitis and facial palsy. Layered surgical repair leads to high rates of success; however, this may be complicated by worsening facial palsy.
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Paralisia de Bell , Traumatismos Craniocerebrais , Paralisia Facial , Meningite , Meningocele , Humanos , Paralisia de Bell/complicações , Vazamento de Líquido Cefalorraquidiano/cirurgia , Vazamento de Líquido Cefalorraquidiano/complicações , Otorreia de Líquido Cefalorraquidiano/etiologia , Otorreia de Líquido Cefalorraquidiano/cirurgia , Traumatismos Craniocerebrais/complicações , Paralisia Facial/complicações , Meningocele/diagnóstico por imagem , Meningocele/cirurgia , Meningocele/complicações , Estudos Multicêntricos como Assunto , Obesidade/complicações , Estudos RetrospectivosRESUMO
OBJECTIVE: To review surgical techniques used in the endoscopic transnasal repair of pediatric basal meningoencephaloceles and compare perioperative outcomes in children <2 and ≥2 years old. DATA SOURCES: MEDLINE, EMBASE, and CENTRAL. REVIEW METHODS: Data sources were searched from inception to August 22, 2022, using search terms relevant to endoscopic transnasal meningoencephalocele repair in children. Reviews and Meta-analyses were excluded. Primary outcomes were the incidence of intraoperative and postoperative complications, including cerebrospinal fluid leak, recurrence, and reintervention. Quality assessments were performed using Newcastle-Ottawa Scale, ROBIN-I, and NIH. RESULTS: Overall, 217 patients across 61 studies were identified. The median age at surgery was 4 years (0-18 years). Fifty percent were female; 31% were <2 years. Most defects were meningoencephaloceles (56%), located transethmoidal (80%), and of congenital origin (83%). Seventy-five percent of repairs were multilayered. Children ≥2 years underwent multilayer repairs more frequently than those <2 years (P = 0.004). Children <2 years more frequently experienced postoperative cerebrospinal fluid leaks (P = 0.02), meningoencephalocele recurrence (P < 0.0001), and surgical reintervention (P = 0.005). Following multilayer repair, children <2 years were more likely to experience recurrence (P = 0.0001) and reintervention (P = 0.006). CONCLUSION: Younger children with basal meningoencephaloceles appear to be at greater risk of postoperative complications following endoscopic endonasal repair, although the quality of available evidence is weakened by incomplete reporting. In the absence of preoperative cerebrospinal fluid leak or meningitis, it may be preferable to delay surgery as access is more conducive to successful repair in older children.
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Encefalocele , Meningocele , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Encefalocele/cirurgia , Endoscopia/métodos , Meningocele/cirurgia , Cirurgia Endoscópica por Orifício Natural/métodos , Nariz/cirurgia , Complicações Pós-Operatórias/epidemiologia , Masculino , Recém-NascidoRESUMO
In this case report, we aimed to describe the clinical presentation, surgical approach, and follow-up of a patient with rare anterior meningocele associated with rectothecal fistula. An 17-year-old female patient was admitted to the emergency department with meningitis. On further examinations, an anterior sacral meningocele accompanied by rectothecal fistula was detected. Appropriate antibiotic treatment was arranged and surgical plan was made with the pediatric surgery clinic. The patient underwent meningocele repair via posterior approach and colostomy operation. The patient did not experience any neurological issues after the surgery. The colostomy was reversed 3 months later, and third-month follow-up MRI showed complete regression of the meningocele sac with no neurological complications. Anterior meningocele accompanied by a rectothecal fistula is a rare and complicated case. Only seven cases of coexisting ASM and RTF have been reported in literature. Although both anterior and posterior approaches have been used for the treatment of ASM, the choice of treatment is essentially based on the patient's clinical and imaging findings.
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Fístula , Meningocele , Doenças da Coluna Vertebral , Feminino , Criança , Humanos , Adolescente , Meningocele/cirurgia , Fístula/complicações , Doenças da Coluna Vertebral/complicações , Sacro/cirurgia , Imageamento por Ressonância Magnética/efeitos adversosRESUMO
The first documented description of an anterior sacral meningocele was Bryant's in 1823. Anterior sacral meningocele patients have constipation as a universal symptom; urinary incontinence is also common. All the symptoms are directly related to the pressure from a pelvic mass on adjacent structures. When the patient stands, a headache often develops because the spinal fluid pressure decreases as the meningocele sac fills. Finally, a scimitar-shaped sacrum on a neuroradiological anteroposterior plain assessment is pathognomonic. The coccyx may be absent, and the lower sacral laminae may be absent or incomplete. The surgical options for this rare clinical condition are still matter of debate.Anterior sacral meningocele is a pathology that lacks a current classification and neurosurgical therapeutic standards, even though a similar dynamic has been shown by the related traumatic pseudomeningocele. Anterior approaches (retro- and transperitoneal meningocele neck occlusion with internal cerebrospinal fluid (CSF) cyst drainage) and posterior approaches (posterior sacral laminectomy, dural sac ligation, and CSF cyst drainage) are the available surgical strategies.We now report the case of an adult patient for whom a posterior approach was suggested and performed and report her postoperative surgical follow-up. The surgical rationale is also discussed.
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Cistos , Meningocele , Adulto , Feminino , Humanos , Vazamento de Líquido Cefalorraquidiano , Descompressão , Laminectomia , Meningocele/complicações , Meningocele/diagnóstico por imagem , Meningocele/cirurgia , Punções , Sacro/diagnóstico por imagem , Sacro/cirurgiaRESUMO
BACKGROUND: Neurofibromatosis type 1 (NF1) gives rise to a variety of spinal pathologies that include dural ectasia (DE), vertebral malalignments (VMA), spinal deformities (SD), syrinx, meningoceles, spinal nerve root tumours (SNRT), and spinal plexiform tumours (SPT). The relationship between these and the progression of these pathologies has not been explored before in detail and this paper aims to address this. METHODS: Data was retrospectively collected from adult NF1 multi-disciplinary team meetings from 2016 to 2022 involving a total of 593 patients with 20 distinct predictor variables. Data were analyzed utilizing; Chi-Square tests, binary logistic regression, and Kaplan-Meier analysis. RESULTS: SNRT (19.9%), SD (18.6%), and (17.7%) of VMA had the highest rates of progression. SD was significantly associated (p < 0.02) with the presence and progression of all spinal pathologies except for SPT. Statistically significant predictors of SD progression included the presence of DVA, VMA, syrinx, meningocele, and SNRT. Kaplan-Meier analysis revealed no statistically significant difference between the times to progression for SD (85 days), SNRT (1196 days), and VMA (2243 days). CONCLUSION: This paper explores for the first time in detail, the progression of various spinal pathologies in NF1. The presence and progression of SD is a key factor that correlated with the progression of different spinal pathologies. Early identification of SD may help support clinical decision-making and guide radiological follow-up protocols and treatment.
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Meningocele , Neurofibromatose 1 , Neoplasias da Medula Espinal , Neoplasias da Coluna Vertebral , Siringomielia , Adulto , Humanos , Neurofibromatose 1/diagnóstico por imagem , Estudos Retrospectivos , Coluna Vertebral/patologia , Neoplasias da Medula Espinal/patologia , Radiografia , Neoplasias da Coluna Vertebral/patologiaRESUMO
Currarino syndrome is characterized by a triad of anorectal malformations, sacral defects, and presacral masses. Although it is not extremely rare, this report presents a surgical case of Currarino syndrome with syringomyelia and discusses related literature. The patient is a girl, aged 2 years and 2 months, who presented with marked constipation, chronic cystitis, and lower limb weakness. After examining the patient through magnetic resonance imaging, we diagnosed her with rectal compression due to meningocele and syringomyelia. The base of the meningocele was detached, and the spinal cord was untethered. One week after surgery, her lower limb weakness and constipation improved. Following up on symptoms and performing imaging is essential to determine a treatment plan for Currarino syndrome. (Received 28 February, 2023; Accepted 22 March, 2023; Published 1 August, 2023).
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Meningocele , Siringomielia , Humanos , Feminino , Siringomielia/complicações , Siringomielia/diagnóstico por imagem , Siringomielia/cirurgia , Meningocele/complicações , Meningocele/diagnóstico por imagem , Meningocele/cirurgia , Reto/cirurgia , Reto/anormalidades , Reto/patologia , Constipação Intestinal/etiologia , Constipação Intestinal/cirurgia , Imageamento por Ressonância Magnética , Debilidade MuscularRESUMO
INTRODUCTION: Intrasacral meningoceles are cysts associated with herniating arachnoid with no nerve root within due to an area of weakness of the dura mater. They are thought to be congenital, but they are usually not symptomatic until adulthood. Surgical treatment is generally indicated in the presence of symptoms. METHODS: We selected cases belonging to the IB category of Nabors et al.'s classification who underwent surgery between 2008 and 2021 at Giannina Gaslini Hospital. Exclusion criteria were prior history of trauma, infections, or operations. Patients' clinical details, associated conditions, surgical techniques, peri- and postoperative complications, and outcomes were collected retrospectively from clinical charts. We compared our series to literature: keywords "intrasacral meningocele" were used on the search engine MEDLINE - Pubmed. RESULTS: We identified 23 cases: 5 of the 14 symptomatic patients had a complete resolution, and 5 had a substantial clinical improvement after surgery. Cyst recurrence and major postoperative complication occurred in none. Among 59 articles considered for evaluation, 50 were excluded and remaining 9 articles underwent full-text analysis. DISCUSSION AND CONCLUSION: The pathogenesis of instrasacral meningoceles is still not completely understood and the spectrum of symptoms is wide. A posterior surgical approach with sacral laminectomy is preferred, although in selected cases it is possible to perform a supplemental anterior approach (sometimes endoscopic). In our surgical series, the largest one published in the literature, a good clinical outcome was achieved in most patients with no cyst's recurrence, pointing out the importance of surgical interruption of communication between cyst and subdural space.
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Cistos Aracnóideos , Cistos , Meningocele , Humanos , Adulto , Meningocele/diagnóstico , Meningocele/cirurgia , Estudos Retrospectivos , Laminectomia , Cistos/cirurgia , Endoscopia , Cistos Aracnóideos/cirurgiaRESUMO
INTRODUCTION: Sprengel's deformity is a rare congenital anomaly of the shoulder rim. It is the most common congenital anomaly of the shoulder, associated with cosmetic deformity and abnormal shoulder function. Nonsurgical management can be considered for mild cases. Surgical intervention is indicated in moderate to severe cases with the goal of improving cosmetic appearance and function. The best surgical results are obtained in children aged 3-8 years. Correct diagnosis is very important because Sprengel's deformity can be accompanied by additional abnormalities, even in mild cases, and lack of a diagnosis delays proper treatment of the child. The severity of the defect may progress, so it is important to correctly identify children with Sprengel's deformity, even those with a mild form of the defect. CASE PRESENTATION: We report a case of prenatal sonographic diagnosis of Sprengel's deformity with additional features, as yet undescribed and missed - although visible - on prenatal magnetic resonance imaging (MRI). Cesarean delivery was performed due to preterm rupture of membranes, and a postnatal MRI confirmed the unusual constellation of Sprengel's anomaly with lateral meningocele, vestigial posterior meningocele, and lipoma tethering of the cord to the dural sac at the cervical-thoracic junction. CONCLUSION: Diagnosis of Sprengel's deformity is possible with prenatal ultrasound. Asymmetry of the cervical spine, discontinuity of the vertebral arch and abnormal vertebral bodies, as well as the asymmetric position of the shoulder blades with the presence of an omovertebral bone are signs that can help diagnose the defect.
Assuntos
Anormalidades Congênitas , Meningocele , Articulação do Ombro , Criança , Recém-Nascido , Feminino , Gravidez , Humanos , Escápula/anormalidades , Escápula/cirurgia , Articulação do Ombro/diagnóstico por imagem , Articulação do Ombro/cirurgia , Articulação do Ombro/anormalidades , Imageamento por Ressonância Magnética , Anormalidades Congênitas/diagnósticoRESUMO
BACKGROUND: Amniotic band syndrome (ABS) is a rare congenital disease characterized by a broad spectrum of congenital anomalies resulting from the strangulated developing organ(s) by the detached fibrous amniotic band. The prevalence of CNS involvement in ABS is rare, but the mortality rate in these cases is high, while morbidity among the surviving patients is inevitable. CASE REPORT: Three-month-old male, 9-month-old female, and newborn female babies were presented with head lump(s), severe facial cleft, syndactyly, and finger amputation. The patient's head imaging confirmed meningoencephalocele as the cause of the head lump in 2 patients; meanwhile, a porencephalic cyst was identified as the origin of head lumps in the other patient. VP shunt placement surgery was performed as the initial management in 2 patients, while one patient directly underwent meningoencephalocele resection surgery. Craniofacial and limb reconstructions were planned as the follow-up management in all cases. Unfortunately, one patient died of complications from suspected aspiration, while another never returned for follow-up treatment. CONCLUSION: Here, we report 3 ABS cases with CNS involvement. Despite the severe disfigurement and disability, the inexistence of fatal malformation might lead to long-term survival. The treatment of malformation(s) that might predispose to another fatal condition and surgery(-ies) to improve functional outcomes and patient's social acceptability should be prioritized in managing the surviving ABS patients.
Assuntos
Síndrome de Bandas Amnióticas , Neoplasias do Sistema Nervoso Central , Fissura Palatina , Meningocele , Segunda Neoplasia Primária , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Síndrome de Bandas Amnióticas/cirurgia , Síndrome de Bandas Amnióticas/complicações , Neoplasias do Sistema Nervoso Central/complicações , Fissura Palatina/complicações , Encefalocele/diagnóstico por imagem , Encefalocele/cirurgia , Ossos Faciais , Meningocele/complicações , NeurocirurgiõesRESUMO
This is a letter to share the clinical experience we had with neonates born with a specific feature of spina bifida. The lesion is like a meningocele containing skim amount of fluid, with a tiny dimple around the center of the cutaneous lesion which causes inward retraction of the dysplastic coverage. This feature is in favor of accompanied split cord malformation (SCM) type I, and the central nidus is in continuous with the dural sleeve around the bony septum of SCM. By recognition of this clinical clue, surgical repair can be planned to perform with appropriate provision, particularly foreseeing the amount of intraoperative bleeding and duration of anesthesia in the newborn patients.