Lipomyelomeningocele associated with an ulnar club hand in the spectrum of VACTERL association (costo-vertebral abnormalities; anal atresia; cardiac defects; tracheal-esophageal abnomalities, ...including atresia, stenosis, and fistula; renal and radial abnormalities; limb abnormalities; single umbilical artery) is a very rare and infrequently reported phenomenon. Within the fat mass of the lipoma, it is not common to find a well-defined cartilaginous mass with no attachments to the surrounding tissue.
The authors present the case of a 3-month-old male with low-back swelling that was off-center to the left, accompanied by a left short forearm displaying outward bowing. Echocardiography showed an atrial septal defect. This rare VACTERL association comprises lipomyelomeningocele, atrial septal defect, and ulnar longitudinal deficiency syndrome. During surgical intervention for the lipoma, a well-defined cartilaginous mass was discovered within the adipose tissue.
The manifestation of VACTERL association can be partially explained by the Shh/Gli and Wnt pathway defects. It is prudent to screen children with neural tube defects to be aware of any associated syndromes. This case is very rare, and the literature has contained no prior report on the VACTERL association of lipomyelomeningocele, atrial septal defect, and ulnar longitudinal deficiency.
Central venous catheters (CVCs) play an indispensable role in clinical practice. Catheter malposition and tip migration can lead to severe complications. The authors present a case illustrating the ...endovascular management of inadvertent marginal sinus cannulation after an internal jugular vein (IJV) catheter tip migration.
A triple-lumen CVC was inserted without complications into the right IJV of a patient undergoing a repeat sternotomy for aortic valve replacement. Two weeks postinsertion, it was discovered that the tip had migrated superiorly, terminating below the torcula in the posterior fossa. In the interventional suite, a three-dimensional venogram confirmed the inadvertent marginal sinus cannulation. The catheter was carefully retracted to the sigmoid sinus to preserve the option of catheter exchange if embolization became necessary. After a subsequent venogram, which displayed an absence of contrast extravasation, the entire catheter was safely removed. The patient tolerated the procedure well.
Clinicians must be vigilant of catheter tip migration and malposition risks. Relying solely on postinsertion radiographs is insufficient. Once identified, prompt management of the malpositioned catheter is paramount in reducing morbidity and mortality and improving patient outcomes. Removing a malpositioned catheter constitutes a critical step, best performed by a specialized team under angiographic visualization.
Early venous drainage is a critical diagnostic feature of arteriovenous malformations (AVMs) and arteriovenous fistulas (AVFs). However, other conditions associated with early venous drainage can ...mimic AVMs and AVFs and mislead the treating physician team.
The authors present a unique case of a 56-year-old man with new left hemianopsia. Workup with magnetic resonance imaging and magnetic resonance angiography had revealed cortical enhancement and early venous drainage, possibly caused by an AVF or AVM. Catheter angiography confirmed the presence of early venous drainage of specifically the right occipital lobe. The early venous drainage occurred as the result of local hyperperfusion limited to the right occipital lobe. Subsequent electroencephalography confirmed focal seizure activity in the right occipital lobe. After seizure control with multiple antiepileptic medications, the patient regained his vision. Repeat digital subtraction angiography revealed restoration of normal cerebral blood flow.
Early venous drainage observed during catheter angiography can be associated with seizure activity. Differentiation from other conditions, such as AVM, AVF, or ischemic stroke, is critical to facilitate prompt and appropriate treatment.
Radicular pain after lumbar decompression surgery can result from epidural hematoma/seroma, recurrent disc herniation, incomplete decompression, or other rare complications. A less recognized ...complication is postoperative nerve root herniation, resulting from an initially unrecognized intraoperative or, more commonly, a spontaneous postoperative durotomy. Rarely, this nerve root herniation can become entrapped within local structures, including the facet joint. The aim of this study was to illustrate our experience with three cases of lumbosacral nerve root eventration into an adjacent facet joint and to describe our diagnostic and surgical approach to this rare complication.
Three patients who had undergone lumbar decompression surgery with or without fusion experienced postoperative radiculopathy. Exploratory revision surgery revealed all three had a durotomy with nerve root eventration into the facet joint. Significant symptom improvement was achieved in all patients following liberation of the neural elements from the facet joints.
Entrapment of herniated nerve roots into the facet joint may be a previously underappreciated complication and remains quite challenging to diagnose even with the highest-quality advanced imaging. Thus, clinicians must have a high index of suspicion to diagnose this issue and a low threshold for surgical exploration.
In mechanical thrombectomy for tandem occlusions in vertebrobasilar stroke, distal emboli from the vertebral artery lesion should be prevented. However, no suitable embolic protection devices are ...currently available in the posterior circulation. Here, the authors describe the case of a vertebral artery lesion effectively treated with a closed-tip stent retriever as an embolic protection device in the posterior circulation.
A 65-year-old male underwent mechanical thrombectomy for basilar artery occlusion, with tandem occlusion of the proximal vertebral artery. After basilar artery recanalization via the nonoccluded vertebral artery, a subsequent mechanical thrombectomy was performed for the occluded proximal vertebral artery. To prevent distal embolization of the basilar artery, an EmboTrap III stent retriever was deployed as an embolic protection device within the basilar artery to successfully capture the thrombus.
A stent retriever with a closed-tip structure can effectively capture thrombi, making it a suitable distal embolic protection device in the posterior circulation.
Primary hemifacial spasm (HFS) is usually caused by arterial compression of the facial nerve at the root exit zone at the brainstem. Rarely, a purely venous compression is seen. However, arachnoid ...bands strangulating the facial nerve have not been recognized as a cause of hemifacial spasm.
The authors present a case of a 24-year-old female who had experienced HFS for 9 years. Endoscopic inspection of the root exit zone revealed no vascular compression but an arachnoid band strangulating the facial nerve. After cutting the band, the lateral spread response disappeared, and the patient was immediately spasm free after the surgery. Two years later, she was still doing well without any spasms.
This is the third patient in the authors' series of 535 patients who had no vascular conflict but rather a strangulation of the nerve by arachnoid bands. All patients have remained spasm free. To the authors' knowledge, no other group has reported arachnoid bands as an etiological factor of HFS so far.
The best surgical approach for resecting bilateral parafalcine meningioma, as well as the optimal anesthesia and airway management for craniotomy in patients with interstitial pneumonia (IP) for ...preventing postsurgical exacerbation, remains unclear.
A 66-year-old female with a history of multiple relapses of IP underwent craniotomy for resection of a 4.5-cm bilateral parafalcine meningioma located just beneath the inferior sagittal sinus. To avoid mechanical ventilation or high-concentration oxygenation, the entire procedure was performed under nonintubated spontaneous breathing conditions with a supraglottic airway/laryngeal mask airway (SGA/LMA) device. Half of the tumor was resected using the ipsilateral interhemispheric approach, while the remaining half was resected using the contralateral transfalcine approach (CTA). No brain retractors were required. Preoperative embolization contributed toward reducing blood loss and surgery duration. During most of the operation, additional oxygen administration was not required. The postoperative course was uneventful, without exacerbation of the IP.
This case demonstrated the utility and feasibility of a unilateral interhemispheric approach combined with CTA for resection of a bilateral parafalcine meningioma. Additionally, this case provides an alternative method of airway and anesthesia management with an SGA/LMA device and nonintubated spontaneous breathing for the prevention of postoperative acute exacerbation of IP.
von Hippel-Lindau disease-associated hemangioblastomas (HBs) account for 20%-30% of all HB cases, with the appearance of new lesions often observed in the natural course of the disease. By ...comparison, the development of new lesions is rare in patients with sporadic HB.
A 65-year-old man underwent clipping for an unruptured aneurysm of the anterior communicating artery. Fourteen years later, follow-up magnetic resonance imaging (MRI) revealed a strongly enhanced mass in the right cerebellar hemisphere, diagnosed as a sporadic HB. A retrospective review of MRI studies obtained over the follow-up period revealed the gradual development of peritumoral edema and vascularization before mass formation.
Newly appearing high-intensity T2 lesions in the cerebellum may represent a preliminary stage of tumorigenesis. Careful monitoring of these patients would be indicated, which could provide options for early treatment to improve patient outcomes.
Lesson study is a popular professional development approach in Japan whereby teachers collaborate to study content, instruction, and how students solve problems and reach for understanding in order ...to improve elementary mathematics instruction and learning in the classroom. This book is the first comprehensive look at the system and process of lesson study in Japan. It describes in detail the process of how teachers conducted lesson study--how they collaborated in order to develop a lesson, what they talked about during the process, and what they looked at in order to understand deeply how students were learning. Readers see the planning of a mathematics lesson, as well as how much content knowledge the teachers have. They observe students' problem solving strategies and learn how Japanese teachers prepare themselves to identify those strategies and facilitate the students' discussion. Written for mathematics teachers, educational researchers, school administrators interested in teachers' professional development, and professional developers, this landmark volume provides an in-depth understanding of lesson study that can lead to positive changes in teachers' professional development and in teaching and learning in the United States.
Aneurysmal bone cysts (ABCs) are rare, highly vascular osteolytic bone lesions that predominantly affect pediatric populations. This report evaluates the clinicopathological data of pediatric ...patients with spinal ABCs. The medical records for all patients at Children's Hospital Los Angeles with biopsy-proven ABCs of the spine between 1998 and 2018 were evaluated.
Seventeen patients, 6 males and 11 females, were identified. The mean age at surgery was 10.4 years (range, 3.5-20 years). The most common presenting complaint was pain at the lesion site 16/17 (94%), followed by lower-extremity weakness 8/17 (47%). Resection and intralesional curettage were performed in all patients. Three (18%) of 17 patients underwent selective arterial embolization prior to resection. Spinal stability was compromised in 15 of 17 patients (88%), requiring instrumented fusion. Five (29%) of the 17 patients received additional therapy including radiation, calcitonin-methylprednisolone, or phenol. Four (23.5%) of 17 patients experienced a recurrence, and the mean time to recurrence was 15 months. The postoperative follow-up ranged from 6 to 108 months (median, 28 months). Reoperation occurred after an average of 35 months. At the recent follow-up, patients were free of disease.
Gross-total resection by intralesional curettage with case-dependent instrumented spinal fusion for instability remains an effective strategy for managing pediatric spinal ABCs. Long-term follow-up is necessary to detect tumor recurrence.
