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JA Clinical Reports Feb 2024Diagnosed intrathoracic meningocele is an uncommon complication of neurofibromatosis type 1. We report an anesthesia management for a rare case undergoing thoracoscopic...
BACKGROUND
Diagnosed intrathoracic meningocele is an uncommon complication of neurofibromatosis type 1. We report an anesthesia management for a rare case undergoing thoracoscopic resection of a huge intrathoracic meningocele.
CASE PRESENTATION
A 51-year-old woman was scheduled for thoracoscopic meningectomy under general anesthesia. We monitored intrathecal pressure during anesthesia to prevent a decrease in intrathecal pressure. During surgery, the intrathecal pressure occasionally increased by around 5 cmHO immediately after the insertion of the drainage tube and occasionally decreased by up to 10 cmHO during the careful slow aspiration of the cerebrospinal fluid (CSF). The pressure rapidly recovered after the interruption of the procedures. She was discharged on postoperative day 4 without major complications.
CONCLUSIONS
The CSF pressure was fluctuated by procedures during thoracoscopic resection of a huge meningocele. A CSF pressure monitoring was useful to detect the sudden change of CSF pressure immediately, which can cause intracranial hemorrhage.
PubMed: 38369643
DOI: 10.1186/s40981-024-00697-1 -
Journal of Neurosurgery. Case Lessons Mar 2024Anterior cervicothoracic myelomeningoceles are a rare pathology. In reported cases, treatment has included shunting, isolated resection and repair without deformity...
BACKGROUND
Anterior cervicothoracic myelomeningoceles are a rare pathology. In reported cases, treatment has included shunting, isolated resection and repair without deformity correction, or isolated deformity correction without meningocele repair. The authors describe a pediatric patient with an anterior cervicothoracic myelomeningocele presenting with progressive neurological decline, who underwent simultaneous treatment of the myelomeningocele to detether the spinal cord and achieve major correction of the scoliotic deformity.
OBSERVATIONS
A 15-year-old girl was born with C7-T1-T2 hemivertebrae and anterior cervical myelomeningocele at C7-T1. She developed progressive cervical thoracic scoliosis, left hemiparesis initially, and additional right hemiparesis eventually. She underwent surgical repair via C7, T1, and T2 corpectomies with intradural detethering of the spinal cord. The scoliosis was treated with C7-T2 Ponte osteotomies and C2-T5 posterior fixation, followed by anterior reconstruction with a titanium cage and anterior plate from C6 to T3. The myelomeningocele was adequately treated with good correction of the patient's deformity. The patient had postoperative improvement in her strength and solid arthrodesis on postoperative imaging.
LESSONS
The authors describe the successful treatment of an anterior cervicothoracic myelomeningocele and associated scoliosis in a child. This is a unique report of a combined strategy to achieve both deformity correction and detethering of the spinal cord.
PubMed: 38437675
DOI: 10.3171/CASE23710 -
The Canadian Veterinary Journal = La... Oct 2023This report describes the clinical, computed tomography, and magnetic resonance imaging findings for a Jacob sheep lamb diagnosed with meningoencephalocele and...
This report describes the clinical, computed tomography, and magnetic resonance imaging findings for a Jacob sheep lamb diagnosed with meningoencephalocele and supernumerary ectopic limb. Key clinical message: This case demonstrates the utility of tomographic imaging in diagnosing congenital malformations in sheep and can be used to assess the extent of the lesion. This may help to determine any viable treatment, or, as in the case presented here, determine that the extent of the lesion precludes surgical intervention.
Topics: Animals; Sheep; Encephalocele; Meningocele; Occipital Bone; Extremities; Tomography, X-Ray Computed; Magnetic Resonance Imaging; Sheep Diseases
PubMed: 37780471
DOI: No ID Found -
Surgical Neurology International 2024Antiphospholipid syndrome is a complex autoimmune condition associated with the formation of recurrent thrombosis in any vascular bed throughout the body. Jugular vein...
BACKGROUND
Antiphospholipid syndrome is a complex autoimmune condition associated with the formation of recurrent thrombosis in any vascular bed throughout the body. Jugular vein thrombosis is very rare with only a 0.9% occurrence and is not typically associated with cerebrospinal rhinorrhea as a result of raised intracranial pressure.
CASE DESCRIPTION
A 54-year-old patient presented with a 9-month history of cerebrospinal fluid (CSF) rhinorrhea and headache on a background of antiphospholipid syndrome. Investigations showed a superior vena cava (SVC) and right internal jugular vein (IJV) obstruction with moderately elevated intracranial venous pressures. Her magnetic resonance imaging (MRI) brain was consistent with a CSF leak. The patient underwent successful endovascular stenting of her obstructed SVC and right IJV followed by surgical repair of a herniating meningocele in the posterior left ethmoid air cells.
CONCLUSION
CSF rhinorrhea is uncommon and never previously reported associated with SVC thrombosis induced by antiphospholipid syndrome. A combination of endovascular techniques and surgical repair is recommended for this challenging presentation.
PubMed: 38468657
DOI: 10.25259/SNI_32_2023 -
BMJ Case Reports Sep 2023A woman in her early 20s with a history of lumbosacral lipomyelomeningocele with associated partial sacral agenesis and tethered cord was referred for possible sacral...
A woman in her early 20s with a history of lumbosacral lipomyelomeningocele with associated partial sacral agenesis and tethered cord was referred for possible sacral neuromodulation treatment of her refractory faecal incontinence. Anorectal testing revealed a thinned and weakened anal sphincter complex with absent left-sided bulbocavernosus and anal wink reflexes.Through a two-stage approach, she underwent successful placement of a chronic tined quadripolar sacral nerve lead and implanted pulse generator. Despite significant distortion of normal bony anatomic landmarks, bilateral S3 and S4 foraminal entry was possible, with intraoperative motor and sensory testing providing key confirmation of appropriate lead placement. The patient had a substantial improvement in her baseline frequency and urgency with a near-complete resolution of faecal incontinence episodes during her test phase.
Topics: Female; Humans; Fecal Incontinence; Spinal Dysraphism; Sacrococcygeal Region; Electric Stimulation Therapy
PubMed: 37669822
DOI: 10.1136/bcr-2023-254835 -
British Journal of Neurosurgery Jun 2024Postoperative intracranial complications are rare in spine surgery not including cranial procedures. We describe an uncommon case of pseudohypoxic brain swelling (PHBS)...
BACKGROUND
Postoperative intracranial complications are rare in spine surgery not including cranial procedures. We describe an uncommon case of pseudohypoxic brain swelling (PHBS) and secondary hydrocephalus after transforaminal lumbar interbody fusion (TLIF) presenting as impaired consciousness and repeated seizures.
CASE PRESENTATION
A 65-year-old man underwent L4-5 TLIF for lumbar spondylolisthesis and began experiencing generalized seizures immediately postoperatively. Computed tomography (CT) revealed diffuse cerebral edema-like hypoxic ischemic encephalopathy. He was transported to our hospital, at which time epidural drainage was halted and anti-edema therapy was commenced. His impaired consciousness improved. However, he suffered secondary hydrocephalus due to continuous bleeding from a dural defect and spinal epidural fluid collection 3 months later. Following the completion of dural repair and insertion of a ventriculoperitoneal shunt, his neurologic symptoms and neuroimaging findings improved significantly.
CONCLUSIONS
PHBS can be considered in patients with unexpected neurological deterioration following lumbar spine surgery even with the absence of documented durotomy. This might be due to postoperative intracranial hypotension-associated venous congestion, and to be distinguished from the more common postoperative cerebral ischemic events-caused by arterial or venous occlusions-or anesthetics complications.
Topics: Humans; Male; Aged; Postoperative Complications; Lumbar Vertebrae; Hydrocephalus; Spinal Fusion; Spondylolisthesis; Brain Edema; Meningocele; Ventriculoperitoneal Shunt; Tomography, X-Ray Computed
PubMed: 34553665
DOI: 10.1080/02688697.2021.1958157 -
Surgical Neurology International 2024Delayed cerebrospinal fluid (CSF) leaks are a known complication following intradural spinal tumor surgery. The placement of subfascial drains in these patients...
BACKGROUND
Delayed cerebrospinal fluid (CSF) leaks are a known complication following intradural spinal tumor surgery. The placement of subfascial drains in these patients undergoing requisite intradural surgery is controversial. Here, we demonstrated that placing a subfascial drain on partial suction for 48 h, with early ambulation, proved to be safe and effective in preventing early/delayed recurrent CSF fistulas.
METHODS
Medical records of 17 patients undergoing surgery for intradural spinal tumors over a 30-month were reviewed. All patients underwent intradural tumor resection followed by primary dural closure, placement of Gelfoam in a non-compressive fashion, application of fibrin sealant, and utilization of a subfascial drain placed on partial suction for 48 h postoperatively. Patients are mobilized the morning following surgery. We tracked the incidence of postoperative recurrent CSF leaks, over drainage, infection, wound dehiscence, pseudo meningocele formation, and the reoperation rate.
RESULTS
For the 17 patients, our programmed average utilization of subfascial drains was 48 h. Moreover, the average drain output was 165 mL. Over the 1-year follow-up period, no patient developed a recurrent early/ delayed CSF leak, there were no wound complications, nor need for revision surgery.
CONCLUSION
Utilizing subfascial drains on partial suction following the resection of intradural spinal tumors with primary dural closure proved to be safe and effective.
PubMed: 38344094
DOI: 10.25259/SNI_934_2023 -
Journal of Medical Case Reports Nov 2023Caudal regression syndrome is a rare complex congenital anomaly with reduced penetrance and phenotypic variability characterized by osseous defects of the caudal spine,...
BACKGROUND
Caudal regression syndrome is a rare complex congenital anomaly with reduced penetrance and phenotypic variability characterized by osseous defects of the caudal spine, lower limb anomalies, and accompanying genitourinary, gastrointestinal/anorectal, and cardiac system soft tissue defects. We report a rare presentation of type 1 caudal regression syndrome in a pregnant woman with preexisting diabetes, in which early recognition of severe fetal anomalies on routine antenatal ultrasound facilitated confirmation with fetal magnetic resonance imaging to characterize extent of disease and prognosticate fetal outcome.
CASE PRESENTATION
This case of type 1 caudal regression syndrome in the setting of maternal pregestational diabetes mellitus resulted in stillbirth. The mother was a 29-year-old Caucasian primigravida female with past medical history of poorly controlled type 2 diabetes managed with metformin prior to pregnancy, prompting admission for glucose management and initiation of insulin at 13 weeks. Baseline hemoglobin A1c was high at 8.0%. Fetal ultrasound at 22 weeks was notable for severe sacral agenesis, bilateral renal pelvis dilatation, single umbilical artery, and pulmonary hypoplasia. Fetal magnetic resonance imaging at 29 weeks showed absent lower two-thirds of the spine with corresponding spinal cord abnormality compatible with type 1 caudal regression syndrome. The mother delivered a male stillborn at 39 and 3/7 weeks. Minimally invasive postmortem magnetic resonance imaging and computed tomography autopsy were performed to confirm clinical findings when family declined conventional autopsy. Etiology of sacral agenesis was attributed to poorly controlled maternal diabetes early in gestation.
CONCLUSION
Maternal preexisting diabetes is a known risk factor for development of congenital malformations. This rare case of type 1 caudal regression syndrome in a mother with preexisting diabetes with elevated hemoglobin A1c highlights the importance of preconception glycemic control in diabetic women and the utility of fetal magnetic resonance imaging for confirmation of ultrasound findings to permit accurate prognostication. Additionally, minimally invasive postmortem magnetic resonance imaging and computed tomography autopsy can facilitate diagnostic confirmation of clinical findings in perinatal death due to complex congenital anomalies while limiting the emotional burden on bereaved family members who decline conventional autopsy.
Topics: Female; Pregnancy; Male; Humans; Adult; Autopsy; Diabetes Mellitus, Type 2; Glycated Hemoglobin; Stillbirth; Tomography, X-Ray Computed; Magnetic Resonance Imaging
PubMed: 38001547
DOI: 10.1186/s13256-023-04220-5 -
International Journal of Women's Health 2024Microphthalmia is a rare autosomal recessive condition commonly known as Waardenburg anophthalmia syndrome (WAS) or oculo-acromal formation syndrome (MIM#206920).
BACKGROUND
Microphthalmia is a rare autosomal recessive condition commonly known as Waardenburg anophthalmia syndrome (WAS) or oculo-acromal formation syndrome (MIM#206920).
CASE DESCRIPTION
Here, we report the case of a woman whose fetal ultrasonography at 12 weeks of pregnancy revealed multiple fetal abnormalities. These included the absence of the left upper limb, an unclear display of the right orbit, a visible maxillary space, and a round, echoless appearance measuring 4 mm in diameter in the middle of the forehead. There was also a significant echo in the sac wall. The possibility of a frontal meningocele or a proboscis-like nose was considered. The fetus was delivered with absence of the left upper limb, absence of the right eye, a cleft lip on the right side, and a milky white sac with a diameter of 5 mm on the forehead after the pregnancy was terminated at the hospital. Pathological investigation revealed a mature cystic teratoma. The conclusion was microphthalmia with limb anomalies (MLA) after missing limbs, absence of eyes, and cleft lip were input into the Online Mendelian Inheritance in Man database. The case was diagnosed with fetal microphthalmia with limb anomalies and an interfrontal teratoma.
CONCLUSION
In this case, the entire exon analysis was not conducted, and as a result, the final diagnosis remains unclear. Based exclusively on the phenotype of the induced fetus, MLA was diagnosed. It is proposed that cases satisfying the requirements for a pathological diagnosis should undergo a pathological examination to establish a definitive diagnosis.
PubMed: 38222311
DOI: 10.2147/IJWH.S441452 -
Journal of Pediatric Urology Jun 2024Children and adolescents with neurogenic bladder often need clean intermittent catheterization (CIC) over a long period. Our study aimed to identify factors that affect...
INTRODUCTION
Children and adolescents with neurogenic bladder often need clean intermittent catheterization (CIC) over a long period. Our study aimed to identify factors that affect CIC compliance and to determine if CIC compliance affected short-term urological outcomes among patients in Malaysia.
STUDY DESIGN
50 patients aged 2-18 years who perform CIC were included in this cohort study. Patient compliance with CIC was evaluated using the validated Intermittent Catheterization Adherence Scale (ICAS). CIC difficulties were assessed using the validated Intermittent Catheterization Difficulty Questionnaire (ICDQ). Data was obtained on patients' co-morbidity, caregiver factors, socio-economic factors, CIC technique, access to catheters and facilities, urinary tract infections, incontinence, urology tests and treatment. Statistical analysis was performed.
RESULTS
Mean age was 6.68 ± 4.34 years. 32 (64%) patients commenced CIC within the first month of life. Mean daily CIC frequency was 4.70 ± 1.33.30 (60%) participants showed strong adherence to CIC. 39 (78%) participants were able to catheterize with no or minor difficulties. Pain (6, 12%), transient blocking sensation (6.12%), and urinary incontinence (3, 6%) were the predominant difficulties encountered. CIC performed by caregiver was associated with improved adherence compared to patient self-catheterization (p = 0.039). The mean age of participants who self-catheterized was 10.7 ± 3.7 years. Strong adherence was also observed among patients who purchased their own CIC catheters (p = 0.007). Participants with lower ICDQ score were more likely to be compliant with CIC (p = 0.007). CIC adherence was not affected by patient's age, gender, co-morbidity, mobility, caregiver factors, socio-economic factors, and age at initiation of CIC. There was no significant association between CIC adherence and febrile urinary tract infections, upper tract deterioration, and bladder stones at 6 months follow-up.
DISCUSSION
There is lower CIC adherence when a child begins to self-catheterize and healthcare providers should be alert during this period of transition. Though most patients with spina bifida have decreased urethral sensation, some patients do experience significant pain during CIC which may impact their compliance. These patients would need a review of their catheterization techniques to improve adherence. The limitations of our study are its modest sample size from a single center and short study period. Our study provides insights into the feasibility of instituting CIC in developing countries.
CONCLUSION
Strong CIC adherence was observed among patients who were catheterized by their caregiver, purchased their own CIC catheters, and encountered minimal difficulties during catheterization. CIC adherence had no effect on short-term urological outcomes.
Topics: Humans; Urinary Bladder, Neurogenic; Child; Male; Female; Child, Preschool; Adolescent; Intermittent Urethral Catheterization; Spinal Dysraphism; Patient Compliance; Malaysia; Meningocele; Surveys and Questionnaires; Meningomyelocele; Abnormalities, Multiple; Sacrococcygeal Region
PubMed: 38413297
DOI: 10.1016/j.jpurol.2024.02.009