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American Journal of Translational... 2022The purpose of this study was to investigate the clinical value of microsurgery in the treatment of congenital neural tube defect (CNTD) in newborns.
OBJECTIVE
The purpose of this study was to investigate the clinical value of microsurgery in the treatment of congenital neural tube defect (CNTD) in newborns.
METHODS
Eighty-five CNTD newborns withlipomyelomeningocele admitted to our hospital from March 2016 to December 2018 were retrospectively selected as study subjects. They were divided into a study group (SG, 43 cases, that received meningocele repair combined with tethered cord release within 6 h to 30 d after birth) and the control group (CG, 42 cases, that received meningocele repair combined with tethered cord release past 30 d after birth) according to the treatment regimen. Newborns in both groups were evaluated for short-term and long-term outcome of the surgery and the degree of postoperative untethering, and both groups were followed up dynamically to record changes in gross motor function and quality of life and assess risk factors.
RESULTS
In terms of short-term outcomes, the total effective rate was 93.02% in SG and 85.71% in CG ( > 0.05); in terms of the long-term outcomes, the total effective rate was 88.37% in SG and 69.05% in CG ( < 0.05). The postoperative release of the newborns was evaluated according to the Kirollos grading system, which showed that SG had 40 (93.02%) cases of grade 1 untethering, 3 (6.98%) cases of grade 2 untethering, and 0 case of grade 3 untethering, and CG had 30 (71.43%) cases of grade 1 untethering, and 12 (28.57%) cases of grade 2 untethering. At 6 months postoperatively, there were no significant differences in gross motor function and quality of life scores between the two groups ( > 0.05), but at 1 year, 3 years and 4 years postoperatively, the gross motor function and quality of life scores of newborns in the SG were significantly higher than those in the CG ( < 0.05). Multivariate logistic regression analysis showed that age > 1 month was an independent risk factor for surgical outcome ( < 0.05).
CONCLUSION
Microsurgery has better short-term and long-term outcomes for newborns with CNTD, and the newborns showed an improvement in the long-term postoperative motor function and quality of life.
PubMed: 36105063
DOI: No ID Found -
Cureus Jul 2022A meningocele is a congenital neural tube defect, and the majority of the meningocele cases are identified perinatally. We present the case of a 67-year-old patient with...
A meningocele is a congenital neural tube defect, and the majority of the meningocele cases are identified perinatally. We present the case of a 67-year-old patient with a sacral meningocele undiagnosed until the removal of a symptomatic epidermal inclusion cyst adjacent to it. Cerebrospinal fluid leakage occurred due to an incision in an undiagnosed meningocele adjacent to the epidermal inclusion cyst. Repair of the cerebrospinal fluid leakage was performed successfully without any deficit. The present case underscores the importance of considering a meningocele as a differential diagnosis for a mass occurring in the midline of the back at any age.
PubMed: 36046288
DOI: 10.7759/cureus.27385 -
European Review For Medical and... Aug 2022Encephalocele is a rare congenital neural tube defect (NTD) characterized by herniation of intracranial contents through a defect in the skull. In our study,...
OBJECTIVE
Encephalocele is a rare congenital neural tube defect (NTD) characterized by herniation of intracranial contents through a defect in the skull. In our study, encephalocele was diagnosed in our clinic and its association with hydrocephalus was evaluated. The effect of this association on the prognosis was discussed.
PATIENTS AND METHODS
Patients who underwent surgery and follow-up with the diagnosis of encephalocele in the neurosurgery clinic of our hospital in an 8-year period from 2013 to 2021 were retrospectively examined.
RESULTS
Patient records were obtained from the case notes of patients who underwent excision and repair for encephalocele. Of the 78 patients included in the study, 88.4% underwent surgery in the neonatal period. Moreover, 47% of the patients are male, and 31% are female. Encephalocele was present in 62.8% of patients and meningocele in 37.2%. Furthermore, 82.1% of encephalocele sacs were located in the occipital region. Chiari type 3 malformation was present in 57.6% of patients. Hydrocephalus developed in 56.4% of patients. There was an additional syndrome in 10.3% of the cases. The most common additional syndromes were corpus callosum dysgenesis with 39.7% and colpocephaly with 29.5%. The additional disease was present in 43.6% of patients. Preoperative and postoperative examination findings of more than half of patients were normal, but 33.3% were apathetic. Furthermore, 67.9% of patients, who underwent complete repair, survived, and 32.1% died. Hydrocephalus was present in 73.5% of patients with encephalocele (p<0.05). Hydrocephalus developed in 77.8% of patients with Chiari type 3 malformation (p<0.05). Hydrocephalus was found in 88.0% of patients with Ex (p<0.05).
CONCLUSIONS
Encephalocele, which is a subgroup of NTD, differs clinically by its location and accompanying additional anomalies. In encephaloceles, the risk of morbidity and mortality can only be reduced with the multidisciplinary approach. Hydrocephalus and Chiari type 3 malformation are common in patients with encephalocele. These associations adversely affect the prognosis of the disease. Further research should be conducted on the evaluation of risk factors of NTD and methods of prevention from NTD. In this regard, we recommend that the training be repeated at certain intervals and that people's awareness should be raised.
Topics: Encephalocele; Female; Humans; Hydrocephalus; Infant, Newborn; Male; Neural Tube Defects; Prognosis; Retrospective Studies
PubMed: 35993634
DOI: 10.26355/eurrev_202208_29407 -
Clinical Case Reports Aug 2022The medical information was collected for Currarino syndrome. The patient had anal surgery for congenital anal stenosis when 2 years old. Clinical manifestations were...
The medical information was collected for Currarino syndrome. The patient had anal surgery for congenital anal stenosis when 2 years old. Clinical manifestations were pelvic cystic mass and low abdominal pain. The pelvic mass was found with a diameter of about 20 cm during the transabdominal exploration. At the second day after operation, the patient complained of neck pain. Neurosurgeon performed surgical treatment and diagnosed it as anterior sacral meningocoele. Currarino syndrome has female pelvic mass, sacral malformation, and congenital anorectal malformation, blindly puncture or drainage before operation should not be permitted.
PubMed: 35990386
DOI: 10.1002/ccr3.6229 -
SAGE Open Medical Case Reports 2022The occipital bone is an uncommon location for meningoceles protrusion. This condition occurs generally after a severe traumatism or surgical procedure. However, in some...
The occipital bone is an uncommon location for meningoceles protrusion. This condition occurs generally after a severe traumatism or surgical procedure. However, in some rare cases, the herniation can happen spontaneously. Nontraumatic clival meningoceles present an extremely rare entity and correspond to a herniating pachymeningeal collection containing cerebrospinal fluid through a zone of fragility in the clivus. Clinical presentation ranges from simple headache or rhinorrhea to severe complications such as recurrent bacterial meningitis or nerve compression. Computed tomography provides an analysis of the bone and magnetic resonance imaging provides a superior contrast resolution, helping to distinguish among the various types of clival lesions. We report the case of a young woman with a long history of idiopathic intracranial hypertension, who presented with a worsening headache. Magnetic resonance imaging confirmed a clival meningocele without other complications and the patient was put under medical surveillance.
PubMed: 35966125
DOI: 10.1177/2050313X221117334 -
Neurology India 2022The culprit of trigeminal neuralgia (TGN) may occur at any point between the nerve's root entry zone (REZ) and Meckel's cave. Meckel's cave meningoencephaloceles are... (Review)
Review
BACKGROUND
The culprit of trigeminal neuralgia (TGN) may occur at any point between the nerve's root entry zone (REZ) and Meckel's cave. Meckel's cave meningoencephaloceles are rare middle cranial fossa defects that usually remain asymptomatic but may contain prolapsed trigeminal nerve rootlets and result in TGN. Their management and surgical outcomes remain poorly understood.
OBJECTIVES
To perform a systematic review of clinical presentation and surgical outcomes of middle fossa defects presenting with trigeminal nerve-related symptoms.
MATERIALS AND METHODS
A systematic review was conducted in accordance with the PRISMA guidelines for all reports of middle cranial fossa defects causing trigeminal nerve-related symptoms. The pathophysiology, presentation, surgical management, and outcomes are discussed and illustrated with a case.
RESULTS
Initial search from inception to March 2021 identified 33 articles for screening. After applying inclusion and exclusion criteria, 6 articles were included representing a total of 8 cases in addition to our case (n = 9). All 9 patients were females and 33.3% (n = 3) presented with classic trigeminal neuralgia. "Empty sella" syndrome and radiologic signs of intracranial hypertension were present in 40%-62%. No patient presented with cerebrospinal fluid leak. The preferred treatment modality was surgical with subtemporal extradural repairs using combinations of autologous fat and muscle grafts and synthetic dura. Postoperative outcomes were only available in 55.5% (n = 5) of the cases, and nearly all reported complete symptom resolution, except for one case in which the meningoencephalocele wall was incised, along with trigeminal rootlets adhered to it. Our patient had immediate and durable symptom relief after a 4-year follow-up.
CONCLUSIONS
MEC containing prolapsed trigeminal nerve rootlets can cause typical trigeminal neuralgia from chronic pulsatile stress. This supports the hypothesis that the compressive or demyelinating culprit can locate more ventrally on the course of the trigeminal nerve. Subtemporal extradural surgical repairs can be safe, effective, and durable. Incising the MEC wall should be avoided as it may have trigeminal rootlets adhered to it.
Topics: Cranial Fossa, Middle; Dura Mater; Encephalocele; Female; Humans; Male; Meningocele; Trigeminal Nerve; Trigeminal Neuralgia
PubMed: 35864609
DOI: 10.4103/0028-3886.349629 -
Annals of Medicine and Surgery (2012) Jul 2022Joubert syndrome (JS) is defined by the characteristic set of cerebellum and midbrain abnormalities that communally result in the indicative "molar tooth sign" on the...
INTRODUCTION AND IMPORTANCE
Joubert syndrome (JS) is defined by the characteristic set of cerebellum and midbrain abnormalities that communally result in the indicative "molar tooth sign" on the axial MRI report. The incidence of estimated to be from 1:80,000 to 1:100,000.
CASE PRESENTATION
Clinical features can be noticed shortly after birth that includes hypotonia episodic tachypnea and apnea that may be followed by developmental delays and speech apraxia. Polydactyly, cleft lip or palate, tongue abnormalities, hypotonia, encephalocele, meningocele, hydrocephalus, kidney problems, pituitary abnormality, and autistic-like behavior are the other deformities that can be seen with JS. Seizures may also occur. Motor disability and mental health range from mild to severe forms.
CLINICAL DISCUSSION
Treatment for JS is symptomatic and supportive. The prognosis depends on cerebellar vermis development.
CONCLUSION
JS can be missed if special attention were not given to radiological findings.
PubMed: 35860112
DOI: 10.1016/j.amsu.2022.104113 -
Journal of Neurosurgery. Case Lessons Jun 2022Anterior sacral meningocele (ASM) is a defect in the closure of the neural tube. Patients can be asymptomatic or present with genitourinary, neurological, reproductive,...
BACKGROUND
Anterior sacral meningocele (ASM) is a defect in the closure of the neural tube. Patients can be asymptomatic or present with genitourinary, neurological, reproductive, or colorectal dysfunction. Magnetic resonance imaging (MRI) is the gold standard test because it can assess communication between the spinal subarachnoid space and the lesion and identify other abnormalities. Surgical correction is the definitive treatment because untreated cases have a mortality rate of more than 30%.
OBSERVATIONS
A 24-year-old woman with Marfan syndrome presented with polyuria, recurrent urinary tract infections, and renal injury for 3 months along with a globose abdomen, with a palpable mass in the middle and lower third of the abdomen that was massive on percussion. MRI showed an ASM consisting of two cystic lesions measuring 15.4 × 14.3 × 15.8 and 6.7 × 6.1 × 5.9 cm, respectively, compressing the distal third of the right ureter and causing a hydroureteronephrosis. Drainage and ligature of the cystic lesion were performed. The urinary outcome was excellent, with full recovery after surgery.
LESSONS
ASM should be suspected in all abdominal masses with progressive symptoms in the setting of Marfan syndrome. Computed tomography and MRI are important to investigate genitourinary anomalies or other types of dysraphism to guide the best surgical approach.
PubMed: 35855205
DOI: 10.3171/CASE22154 -
Journal of Neurosurgery. Case Lessons Sep 2021Surgical treatment of intrathoracic meningoceles, commonly associated with neurofibromatosis type 1 (NF1), aims to reduce sac size for symptomatic relief. The procedures...
BACKGROUND
Surgical treatment of intrathoracic meningoceles, commonly associated with neurofibromatosis type 1 (NF1), aims to reduce sac size for symptomatic relief. The procedures can be divided into cerebrospinal fluid diversion and definitive repair. The authors describe the management of an intrathoracic meningocele in a 56-year-old female with preexisting NF1.
OBSERVATIONS
The patient presented with progressive dyspnea. Magnetic resonance imaging revealed a left hemithoracic meningocele arising from the thecal sac at C7-T2. Two attempts at diversion by cystoperitoneal shunts resulted in recurrence. For definitive repair, T2-3 costotransversectomy was performed, and intradural closure of the meningocele opening was performed utilizing spinal dura and autologous fascia lata graft. Trapezius muscle regional flap was turned for reinforcement. Persistent leak warranted reoperation 7 days later. A transthoracic approach was undertaken using video-assisted thoracoscopic resection of the sac at aortic arch level, with reinforcement by latissimus dorsi flap and synthetic materials. Mechanical pleurodesis was performed. Intradural repair of the meningocele opening was revised.
LESSONS
Inherent dural abnormality makes repair difficult for meningoceles associated with NF1. A combined intradural and thoracoscopic approach with regional muscle flap and synthetic material reinforcement is a unique method for definitive treatment. Some essential points of perioperative management are highlighted.
PubMed: 35855190
DOI: 10.3171/CASE21404 -
Surgical Neurology International 2022The authors describe clinical and imaging findings, surgical technique, and outcomes in myelocystocele.
BACKGROUND
The authors describe clinical and imaging findings, surgical technique, and outcomes in myelocystocele.
METHODS
We describe a surgical procedure performed in six patients, four males and two females, with myelocystocele treated at our hospital. We review the images obtained at the time of diagnosis and after surgery. The patients' age range was 12-56 months and had undergone surgery for terminal myelocystocele between 2015 and 2020. All patients had a large lumbar mass covered with healthy skin and presented spontaneous movements at birth. Two patients presented VACTERL syndrome.
RESULTS
A watertight closure of the soft tissues was performed in all cases. None of the patients presented postsurgical complications, such as cerebrospinal fluid leak or infection. All the patients had undergone excision of the meningocele sacs, the tethering bands were lysed, and the filum was detethered. The mean follow-up period was 34 (12-56) months. A motor deficit was seen in 2 patients (33.3%).
CONCLUSION
Prenatal diagnosis and early corrective surgical intervention are recommended to prevent deterioration in neurological function. VACTERL association is a common condition and should be investigated.
PubMed: 35855164
DOI: 10.25259/SNI_299_2022