Ventriculo-inferior-venacaval shunt salvage via endovascular surgery.

INTRODUCTION: Hydrocephalus treatment can be very challenging. While some hydrocephalic patients can be treated endoscopically, many will require ventricular shunting. Frequent shunt issues over a lifetime is not uncommon. Although most shunt malfunctions are of the ventricular catheter or valve, distal failures occur as well. A subset of patients will accumulate non-functioning distal drainage sites. CASE DESCRIPTION: We present a 27-year-old male with developmental delay who was shunted perinatally for hydrocephalus from intraventricular hemorrhage of prematurity. After failure of the peritoneum, pleura, superior vena cava (SVC), gallbladder, and endoscopy, an inferior vena cava (IVC) shunt was placed minimally-invasively via the common femoral vein. We believe this is only the eighth reported ventriculo-inferior-venacaval shunt. IVC occlusion years later was successfully treated with endovascular angioplasty and stenting followed by anticoagulation. To our knowledge, a ventriculo-inferior-venacaval shunt salvaged by endovascular surgery has not been previously described in the literature. CONCLUSION: After failure of the peritoneum, pleura, SVC, gallbladder, and endoscopy, IVC shunt placement is an option. Subsequent IVC occlusion can be rescued by endovascular angioplasty and stenting. Anticoagulation after stenting (and potentially after initial IVC placement) is advised.

Impact of Low-Dose Computed Tomography and Physician Education on Referral for Head Shape.

Imaging before specialist evaluation of abnormal head shape is associated with a delay in evaluation and an increase in radiation exposure. A retrospective cohort study was performed to identify referral patterns before and after the implementation of a low-dose computed tomography (LDCT) protocol and physician education to examine the intervention's impact on time to evaluation and radiation exposure. Six hundred sixty-nine patients with an abnormal head shape diagnosis at a single academic medical center between July 1, 2014 and December 1, 2019 were reviewed. Demographics, referral information, diagnostic testing, diagnoses, and timeline of clinical evaluation were recorded. Before and after the LDCT and physician education intervention, the average ages at initial specialist appointments were 8.82 and 7.75 months, respectively ( P = 0.125). Children referred after our intervention were less likely to have prereferral imaging than children referred prior (odds ratio: 0.59, CI: 0.39-0.91, P = 0.015). Average radiation exposure per patient before referral decreased from 14.66 mGy to 8.17 mGy ( P = 0.021). Prereferral imaging, referral by a non-pediatrician, and non-Caucasian race were associated with older age at the initial specialist appointment. Widespread craniofacial center adoption of an LDCT protocol and improved clinician knowledge may lead to a reduction in late referrals and radiation exposure in pediatric patients with an abnormal head shape diagnosis.

Considerations for Choice of Cranioplasty Material for Pediatric Patients.

INTRODUCTION: Optimal material and timing of cranioplasty in the pediatric population continue to be debated. Autologous and alloplastic materials have various indications for use and risk factors for complications. METHODS: A single-center retrospective cohort study was undertaken of all pediatric patients who underwent cranioplasty with any material from 1991-2021. RESULTS: 149 cranioplasty implants were included. Younger age (6 years old or under), a diagnosis of craniosynostosis as reason for implant, use of autologous bone, and shorter times to cranioplasty were predictive of need for revision surgery. No factors studied had a statistically significant impact on rate of removal of implant at time of revision surgery. CONCLUSION: Autologous and alloplastic cranioplasty materials both have good outcomes with low rates of revision surgery in the pediatric population. Alloplastic implants may be considered in the setting of infection as reason for craniectomy given the lower rate of revision surgery and need for removal. Patients with craniosynostosis as reason for cranioplasty have a higher risk of requiring revision or additional surgeries, regardless of implant used.

The current landscape of immunotherapy for pediatric brain tumors.

Pediatric central nervous system tumors are the most common solid malignancies in childhood, and aggressive therapy often leads to long-term sequelae in survivors, making these tumors challenging to treat. Immunotherapy has revolutionized prospects for many cancer types in adults, but the intrinsic complexity of treating pediatric patients and the scarcity of clinical studies of children to inform effective approaches have hampered the development of effective immunotherapies in pediatric settings. Here, we review recent advances and ongoing challenges in pediatric brain cancer immunotherapy, as well as considerations for efficient clinical translation of efficacious immunotherapies into pediatric settings.

Pediatric craniosynostosis computed tomography: an institutional experience in reducing radiation dose while maintaining diagnostic image quality.

BACKGROUND: Children with craniosynostosis may undergo multiple computed tomography (CT) examinations for diagnosis and post-treatment follow-up, resulting in cumulative radiation exposure. OBJECTIVE: To reduce the risks associated with radiation exposure, we evaluated the compliance, radiation dose reduction and clinical image quality of a lower-dose CT protocol for pediatric craniosynostosis implemented at our institution. MATERIALS AND METHODS: The standard of care at our institution was modified to replace pediatric head CT protocols with a lower-dose CT protocol utilizing 100 kV, 5 mAs and iterative reconstruction. Study-ordered, protocol-utilized and radiation-dose indices were collected for studies performed with routine pediatric brain protocols (n=22) and with the lower-dose CT protocol (n=135). Two pediatric neuroradiologists evaluated image quality in a subset (n=50) of the lower-dose CT studies by scoring visualization of cranial structures, confidence of diagnosis and the need for more radiation dose. RESULTS: During the 30-month period, the lower-dose CT protocol had high compliance, with 2/137 studies performed with routine brain protocols. With the lower-dose CT protocol, volume CT dose index (CTDIvol) was 1.1 mGy for all patients (0-9 years old) and effective dose ranged from 0.06 to 0.22 mSv, comparable to a 4-view skull radiography examination. CTDIvol was reduced by 98% and effective dose was reduced up to 67-fold. Confidence in diagnosing craniosynostosis was high and more radiation dose was considered unnecessary in all studies (n=50) by both radiologists. CONCLUSION: Replacing the routine pediatric brain CT protocol with a lower-dose CT craniosynostosis protocol substantially reduced radiation exposure without compromising image quality or diagnostic confidence.

Comparison of Perioperative Outcomes and Parental Satisfaction Outcomes of Strip Craniectomy with Postoperative Helmet versus Spring-Mediated Remodeling in Sagittal Craniosynostosis.

INTRODUCTION: We sought to compare outcomes and parental satisfaction between 2 approaches for sagittal craniosynostosis: strip craniectomy with spring-mediated skull remodeling (SMSR) and strip craniectomy with postoperative helmet (SCH). METHODS: Perioperative and outcome data for SMSR or SCH patients between September 2010 and July 2019 were retrospectively reviewed. A telephone survey was administered to parents of children who underwent both procedures. RESULTS: A total of 62 children were treated for sagittal craniosynostosis by either SMSR (n = 45) or SCH (n = 17). The SCH group had a lower estimated blood loss (27 vs. 47.06 mL, p = 0.021) and age at surgery (13.0 vs. 19.8 weeks) than the SMSR group. Three patients underwent early springs removal due to trauma or dislodgement, all of whom converted to helmeting. Of the 62 children initially identified, 59 were determined to have an adequate follow-up time to assess long-term outcomes. The mean follow-up time was 30.1 months (n = 16) in the SCH group and 32.0 months in the SMSR group (n = 43, p = 0.39). Two patients in the SCH group and one in the SMSR group converted to open cranial vault reconstruction. Thirty parents agreed to respond to the satisfaction survey (8 SCH, 22 SMSR) based on a Likert scale of responses (0 being most dissatisfied possible, 4 most satisfied possible). Average satisfaction was 3.86/4.0 in the SCH group and 3.45/4.0 in the SMSR group. No parents in the SCH group would change to SMSR, while 3 of the 22 SMSR survey responders would have changed to SCH. CONCLUSIONS: Perioperative outcomes and average parental satisfaction were similar in both groups. Importance of helmet wear compliance and risk of spring dislodgement should be discussed with parents.

Spring-assisted minimally invasive repair of sagittal craniosynostosis.

Craniosynostosis surgery is intended to repair cranial deformity, reduce the risk of increased intracranial pressure from cephalocranial disproportion, and reduce the risk of developmental delays. In recent years, minimally invasive surgical techniques have been developed to achieve these goals with less tissue disruption, lower rates of transfusion, and shorter recovery time. The operation focuses on unlocking the fused bones, while reshaping relies on an adjunct, most commonly a postoperative cranial molding helmet. As an alternative to the care-intensive helmeting process, reshaping with implanted cranial expander springs has emerged. In this video, the authors demonstrate their technique for spring-assisted minimally invasive repair of sagittal craniosynostosis. The video can be found here: https://vimeo.com/513923721.

Pediatric pathology all grown up - An interesting case of adult tethered spinal cord.

BACKGROUND: Cervical myelopathy in an adult is typically the result of degenerative disease or trauma. Dysraphism is rarely the cause. CASE DESCRIPTION: The authors report the case of a 35-year-old male drywall installer who presented with 2 years of progressive left upper extremity weakness, numbness, and hand clumsiness. Only upon detailed questioning did he mention that he had neck surgery just after birth, but he did not know what was done. He then also reported that he routinely shaved a patch of lower back hair, but denied bowel, bladder, or lower extremity dysfunction. Magnetic resonance imaging of the cervical spine demonstrated T2 hyperintensity at C4-C5 with dorsal projection of the neural elements into the subcutaneous tissues concerning for a retethered cervical myelomeningocele. Lumbar imaging revealed a diastematomyelia at L4. He underwent surgical intervention for detethering and repaired of the cervical myelomeningocele. Four months postoperatively, he had almost complete resolution of symptoms, and imaging showed a satisfactory detethering. The diastematomyelia remained asymptomatic and is being observed. CONCLUSION: Tethered cervical cord is a rare cause for myelopathy in the adult patient. In the symptomatic patient, surgical repair with detethering is indicated to prevent disease progression and often results in clinical improvement.

Identifying the Misshapen Head: Craniosynostosis and Related Disorders.

Pediatric care providers, pediatricians, pediatric subspecialty physicians, and other health care providers should be able to recognize children with abnormal head shapes that occur as a result of both synostotic and deformational processes. The purpose of this clinical report is to review the characteristic head shape changes, as well as secondary craniofacial characteristics, that occur in the setting of the various primary craniosynostoses and deformations. As an introduction, the physiology and genetics of skull growth as well as the pathophysiology underlying craniosynostosis are reviewed. This is followed by a description of each type of primary craniosynostosis (metopic, unicoronal, bicoronal, sagittal, lambdoid, and frontosphenoidal) and their resultant head shape changes, with an emphasis on differentiating conditions that require surgical correction from those (bathrocephaly, deformational plagiocephaly/brachycephaly, and neonatal intensive care unit-associated skill deformation, known as NICUcephaly) that do not. The report ends with a brief discussion of microcephaly as it relates to craniosynostosis as well as fontanelle closure. The intent is to improve pediatric care providers' recognition and timely referral for craniosynostosis and their differentiation of synostotic from deformational and other nonoperative head shape changes.

Germinoma Involving the Retina: An Unusual Presentation of Recurrent Intracranial Mixed Germ Cell Tumor.

BACKGROUND: We report a patient with primary central nervous system mixed malignant germ cell tumor (GCT) who presented with recurrent malignant germinomatous infiltration of the retina. CASE DESCRIPTION: A 10-year-old girl initially presented with a large suprasellar mixed malignant GCT with a near-complete response after initial induction of chemotherapy and irradiation. Three and a half years after initial therapy, she presented with progressively worsening vision in her left eye. Magnetic resonance imaging showed infiltrative changes within the left optic nerve but no discrete mass. Serum and cerebrospinal fluid tumor markers were not elevated and cerebrospinal fluid cytology was negative. Left optic nerve biopsy confirmed the presence of mature teratoma and pure germinoma components. She was treated with gross-total resection of the left eye and optic nerve and chemotherapy. Histopathologic evaluation of the optic nerve showed only mature teratoma elements, but with pure germinoma cells infiltrating the inner layers of the retina. CONCLUSIONS: Loco-regional extension of suprasellar GCT to the optic nerve is not uncommon; however, to the best of our knowledge, infiltration of the tumor into the retina is not reported in the literature. Early detection of optic pathway involvement and proper delineation of the irradiation field may prevent GCT infiltration of the retina with subsequent vision loss.

The Preventable Shunt Revision Rate: A Multicenter Evaluation.

BACKGROUND: The Preventable Shunt Revision Rate (PSRR) was recently introduced as a novel quality metric. OBJECTIVE: To evaluate the PSRR across multiple centers and determine associated variables. METHODS: Nine participating centers in North America provided at least 2 years of consecutive shunt operations. Index surgery was defined as new shunt implantation, or revision of an existing shunt. For any index surgery that resulted in a reoperation within 90-days, index surgery information (demographic, clinical, and procedural) was collected and a decision made whether the failure was potentially preventable. The 90-day shunt failure rate and PSRR were calculated per institution and combined. Bivariate analyses were performed to evaluate individual effects of each independent variable on preventable shunt failure followed by a final multivariable model using a backward model selection approach. RESULTS: A total of 5092 shunt operations were performed; 861 failed within 90 days of index operation, resulting in a 16.9% combined 90-day shunt failure rate and 17.6% median failure rate (range, 8.7%-26.9%). Of the failures, 307 were potentially preventable (overall and median 90-day PSRR, 35.7% and 33.9%, respectively; range, 16.1%-55.4%). The most common etiologies of avoidable failure were infection (n = 134, 44%) and proximal catheter malposition (n = 83, 27%). Independent predictors of preventable failure (P < .05) were lack of endoscopy (odds ratio [OR] = 2.26), recent shunt infection (OR = 3.65), shunt type (OR = 2.06) and center. CONCLUSION: PSRR is variable across institutions, but can be 50% or higher. While the PSRR may never reach zero, this study demonstrates that overall about a third of early failures are potentially preventable.

A Retrospective Study of Cervical Spine MRI Findings in Children with Abusive Head Trauma.

BACKGROUND/AIMS: Increasing attention has been given to the possible association of cervical spine (c-spine) injuries with abusive head trauma (AHT). The aims of this study were to describe c-spine MRI findings in hospitalized AHT patients. METHODS: This is a retrospective study of children under the age of 5 years with AHT admitted to hospital in 2004-2013. Those with c-spine MRI were identified, and the images were reviewed. RESULTS: 250 AHT cases were identified, with 34 (14%) undergoing c-spine MRI. Eleven patients (32%) had 25 findings, including hematoma in 2, occiput-C1-C2 edema in 3, prevertebral edema in 6, facet edema in 2, and interspinous and/or muscular edema in 10. No patients had a clinically evident c-spine injury, a clinically unstable c-spine, or required c-spine surgery. CONCLUSIONS: C-spine MRI may identify abnormalities not apparent upon physical examination and the procedure should therefore be considered in cases of suspected AHT.

Minimally invasive pediatric neurosurgery.

Advances in technology have facilitated the development of minimally invasive neurosurgical options for the treatment of pediatric neurological disease. This review seeks to familiarize pediatric neurologists with some of the techniques of minimally invasive pediatric neurosurgery, focusing on treatments for hydrocephalus, arachnoid cysts, intracranial mass lesions, and craniosynostosis.

Recurrence after gross-total resection of low-grade pediatric brain tumors: the frequency and timing of postoperative imaging.

OBJECT: Low-grade glial and glioneuronal brain tumors are frequently encountered in the pediatric population and can be effectively treated by resection. The authors aimed to use imaging to evaluate how often tumors recurred and to determine if recurrences were associated with any clinical symptoms, along with the financial costs of imaging, in patients with radiographically proven gross-total resection (GTR) at Boston Children's Hospital. These data were assessed to propose guidelines regarding postoperative surveillance. METHODS: The authors performed a retrospective cohort analysis of the Pediatric Brain Tumor Program database from 1993 to 2003 to identify patients with glial or glioneuronal tumors initially evaluated at Boston Children's Hospital. Among the 888 patients evaluated for any type of brain tumor during this period, 67 patients had WHO Grade I glial or glioneuronal lesions with radiographically proven GTR and available follow-up data. The frequency and timing of postoperative imaging was compared with the institutional protocol. Recurrence-free survival was calculated using the Kaplan-Meier method. Financial costs of imaging were available from 2001 to 2009 and were averaged to extrapolate the postoperative surveillance costs. RESULTS: Among the 67 patients with GTR, 13 recurrences were detected radiographically with a mean time to recurrence of 32.4 months (range 2.9-128.5 months). The mean duration of follow-up after surgery was 6.6 years. The recurrence-free survival at 2 and 5 years after GTR for all low-grade glial and glioneuronal tumors was 0.90 (95% CI 0.82-0.97) and 0.82 (95% CI 0.73-0.92), respectively. No clinical symptoms were associated with any of the recurrences, and no deaths occurred. Under the institutional protocol of surveillance imaging, the estimated cost per recurrence at 5 years was $104,094 per patient. The proposed protocol would reduce the number of MR scans in the first 5 years from 10 to 5, providing a potential cost savings of $52,047 per recurrence. CONCLUSIONS: Given the slow-growing, clinically asymptomatic nature of low-grade glial and glioneuronal tumors coupled with the financial and psychological costs of repeated imaging, the authors propose a postoperative surveillance MRI schedule that is less intensive than current institutional practice.

Synovial chondromatosis of the cervical spine: a case report and review of the literature.

Synovial chondromatosis is a benign condition characterized by metaplastic changes of the synovial membrane typically affecting large joints. Cervical spine involvement is rare and has not been reported in a teenager. The authors report a case of cervical synovial chondromatosis in a 19-year-old male presenting with left-sided weakness and numbness from spinal cord compression. After gross total resection was accomplished via laminoplasty, the patient's presenting symptoms improved and continued to do so over the follow-up period. The likely cause of the synovial chondromatosis in this patient was repetitive neck trauma as a child from a motor vehicle accident and football. This case demonstrates that the pathophysiology of this rare entity can initiate in the pediatric population. Although rare, synovial chondromatosis should be considered in the differential diagnosis of calcified extradural masses in the teenage population.

Surgical treatment for primary spinal aneurysmal bone cysts: experience from Children's Hospital Boston.

OBJECT: Spinal aneurysmal bone cysts (ABCs) constitute a rare and clinically challenging disease, primarily affecting the pediatric population. Information regarding the management of spinal ABCs remains sparse. In this study the authors review their experience with spinal ABCs at Children's Hospital Boston. METHODS: The medical records of all patients treated surgically for primary spinal ABCs between January 1998 and July 2010 were retrospectively reviewed. RESULTS: Fourteen cases were identified (6 males and 8 females, ages 5-19 years old). The ABCs were located throughout the spine, with an equal number in the thoracic and lumbar spine, and rarely in the cervical spine. The majority of patients presented with back pain, but neurological deficits and spinal deformity were common. A variety of radiographic techniques were used to establish the diagnosis, including needle biopsy. Preoperative selective arterial embolization was performed in 7 cases (50%), and the majority of cases required spinal instrumentation along with resection. Mean follow-up was 55.9 months (range 15-154 months) after initial intervention. Two ABCs recurred (14%), at 9 months and 8 years after incomplete initial resection, and the patients underwent reoperation. Complete resection was ultimately achieved in all cases. All patients were asymptomatic and neurologically intact at their last follow-up evaluation, and showed no evidence of deformity or recurrence on imaging. CONCLUSIONS: Computed tomography and MR imaging are adequate for an initial evaluation of spinal ABCs, although solid variants can present a diagnostic challenge. Given the high rates of recurrence with residual disease, complete obliteration of the lesion should be the goal of treatment. Preoperative embolization is often performed, although in the authors' opinion the degree of bleeding tends not to support its routine use. Long-term follow-up is warranted as recurrences can occur years after initial intervention. However, gross-total excision in conjunction with spinal stabilization, as needed, usually provides cure of the ABC and excellent long-term spinal alignment.

Idiopathic syrinx in the pediatric population: a combined center experience.

OBJECT: Discovery of a syrinx in a child, without a readily identifiable proximate cause such as a Chiari malformation, tumor, or site of tethering, is often a cause of concern for families and a source of consternation for clinicians. There is a paucity of data describing the natural history of an idiopathic syrinx in the pediatric population. The authors present the combined data of 2 major pediatric neurosurgical centers to describe their experience with this condition. METHODS: Data were collected at Children's Hospital Boston and St. Louis Children's Hospital according to institutional review board-approved protocols and captured visits over a 2.5-year interval (October 2006-March 2009), with records reviewed if the patient had a preexisting diagnosis of syrinx. Patients were identified by ICD-9 codes derived from departmental databases. All pediatric patients (age < 19 years) in whom idiopathic syrinx had been diagnosed, as defined by MR imaging findings (dilated central canal in the spinal cord of ≥ 1 mm in axial dimension and extending over at least 2 vertebral levels), were included. RESULTS: Forty-eight patients met the criteria for idiopathic syrinx during this period, and in 32 of them detailed follow-up imaging was available. Discovery of a syrinx was incidental in 6 patients, whereas the others were referred for imaging because of the presence of pain, neurological symptoms, scoliosis, or skin markings. The average age at the first MR imaging session was 9.7 years, with a mean syrinx size of 4 mm (range 1.2-9.4 mm). The majority (52%) of patients had a thoracic syrinx, with the average lesion spanning 7.1 vertebral levels. The average follow-up was 23.8 months (range 2-64 months), and subgroups of patients with < 3 years and ≥ 3 years of follow-up were independently reviewed. Overall, symptoms improved in 34% and worsened in 9%; 57% of the patients remained asymptomatic or stable. Radiographically (in the subgroup of 32 patients with detailed follow-up imaging), syrinx size decreased in 25% of patients, increased in 12.5%, and remained unchanged in 62.5%, with no apparent correlation between change in syrinx size and clinical symptoms. CONCLUSIONS: Clinically, children with an idiopathic syrinx remained asymptomatic, stable, or improved in 91% of cases. The majority of syringes (87.5%) remained stable or shrank over time, with no apparent correlation between changes in size and changes in symptoms. Although longer follow-up is needed, these data suggest that the natural history of an idiopathic syrinx in children is benign, and that repeated imaging may not be necessary.

Midline filum of the sellar dura: a useful landmark during endoscopic transsphenoidal pituitary surgery.

BACKGROUND: During endoscopic transsphenoidal pituitary surgery, identification and constant awareness of the midline is imperative to prevent injury to critical lateral structures, such as the internal carotid arteries. OBJECTIVE: To describe the relevance of a midline filum of the sellar dura which, when present, can serve as a useful intraoperative anatomic marker. METHODS: Intraoperative video recordings of twenty patients undergoing endoscopic transsphenoidal surgery were retrospectively reviewed to assess for the presence and location of a midline dural filum or apparent central dural vascular structure. Prospective intraoperative data were gathered on an additional 16 patients. RESULTS: A midline dural filum was identified in 18 of 36 patients (50%) undergoing endoscopic transsphenoidal surgery. This structure was identified on the midline in all cases, as confirmed by intraoperative neuronavigation and comparison with the vomer. The midline dural filum was identified as a strand-like dural extension (13 patients) or as a small vascular dural structure usually exhibiting low pressure venous bleeding (5 patients). Samples of the midline dural filum were obtained from 2 patients for histopathological analysis, which demonstrated dense collagenous connective tissue without evidence of vessel wall or ductal epithelium. CONCLUSION: In addition to anatomic structures such as the vomer and midline sphenoid sinus septations, a midline dural filum serves as a useful marker during the sellar phase of endoscopic transsphenoidal surgery. Along with intraoperative neuronavigation and Doppler ultrasonography of the cavernous carotid arteries, identification of this structure may further aid in safeguarding against injury to critical paramedian structures.