Utility of ventriculogallbladder shunts in complex cases of hydrocephalus related to extreme prematurity.

OBJECTIVE: The management of hydrocephalus resulting from intraventricular hemorrhage related to extreme prematurity remains demanding. Given the complexities of controlling hydrocephalus in this population, less commonly used procedures may be required. The authors examined the utility of ventriculogallbladder (VGB) shunts in a series of such children. METHODS: The authors retrospectively reviewed the medical records of all children who underwent surgery for hydrocephalus in the period from 2011 through 2019 at Children's Healthcare of Atlanta. Six patients who underwent VGB shunt placement were identified among a larger cohort of 609 patients who had either a new shunt or a newly changed distal terminus site. The authors present an analysis of this series, including a case of laparoscopy-assisted distal VGB shunt revision. RESULTS: The mean age at initial shunt placement was 5.1 months (range 3.0-9.4 months), with patients undergoing a mean of 11.8 shunt procedures (range 5-17) prior to the initial VGB shunt placement at a mean age of 5.3 years (range 7.9 months-12.8 years). All 6 patients with VGB shunt placement had hydrocephalus related to extreme prematurity (gestational age < 28 weeks). At the time of VGB shunt placement, all had complex medical and surgical histories, including poor venous access due to congenital or iatrogenic thrombosis or thrombophlebitis and a peritoneum hostile to distal shunt placement related to severe necrotizing enterocolitis. VGB complications included 1 case of shunt infection, identified at postoperative day 6, and 2 cases of distal shunt failure due to retraction of the distal end of the VGB shunt. In all, there were 3 conversions back to ventriculoperitoneal or ventriculoatrial shunts due to the 2 previously mentioned complications, plus 1 patient who outgrew their initial VGB shunt. Three of 6 patients remain with a VGB shunt, including 1 who underwent laparoscopy-assisted distal shunt revision 110.5 months after initial VGB shunt insertion. CONCLUSIONS: Placement of VGB shunts should be considered in the armamentarium of procedures that may be used in the particularly difficult cohort of children with hydrocephalus related to extreme prematurity. VGB shunts show utility as both a definitive treatment and as a "bridge" procedure until the patient is larger and comorbid abdominal and/or vascular issues have resolved sufficiently to allow conversion back to ventriculoperitoneal or ventriculoatrial shunts, if needed.

Posterior cervical spinal fusion in a 3-week-old infant with a severe subaxial distraction injury.

Unstable spinal injuries in the neonate pose particular challenges in the clinical and radiographic assessment as well as the surgical stabilization of the spine. In this report, the authors present the unfortunate case of a 3-week-old infant who suffered a severe subaxial cervical fracture dislocation with spinal cord injury that occurred as a result of nonaccidental trauma. Imaging demonstrated severe distraction at C5-6 and near-complete spinal cord transection resulting in quadri-paresis. Open surgical reduction was performed with noninstrumented posterior fusion augmented with split rib autograft and recombinant human bone morphogenetic protein-2. Postoperative imaging demonstrated progressive bony fusion at 2 months, and clinical examination findings progressed to a motor examination classification of ASIA C. At 2 years, the fusion mass is stable and cervical alignment is maintained. The patient remains flaccid in the bilateral lower extremities, but has movement with some dexterity in both hands. Follow-up MRI shows severe spinal cord injury with evidence of bilateral C-5 nerve root avulsions. This case represents the first report of spinal fusion in an infant of less than 1 month of age. Given the extreme young age of the patient, the diagnostic challenges as well as the mechanical and technical considerations of surgical fusion are discussed.

Opioid overdose in a child: case report and discussion with emphasis on neurosurgical implications.

In environments in which opioids are increasingly abused for recreation, children are becoming more at risk for both accidental and nonaccidental intoxication. In toxic doses, opioids can cause potentially lethal acute leukoencephalopathy, which has a predilection for the cerebellum in young children. The authors present the case of a 2-year-old girl who suffered an accidental opioid overdose, presenting with altered mental status requiring cardiorespiratory support. She required emergency posterior fossa decompression, partial cerebellectomy, and CSF drainage due to cerebellar edema compressing the fourth ventricle. To the authors' knowledge, this is the first report of surgical decompression used to treat cerebellar edema associated with opioid overdose in a child.

A Novel Technique for Distal Shunt Revision: Retrospective Analysis of Guidewire-Assisted Distal Catheter Replacement.

BACKGROUND: Ventriculoperitoneal shunt revision is a common procedure. Disconnection and fracture of the distal catheter remain a common cause of ventriculoperitoneal shunt malfunction. OBJECTIVE: To describe a novel procedure for peritoneal replacement of the distal catheter by using a guidewire and a modified Seldinger technique (guidewire-assisted distal catheter replacement) and retrospectively evaluate the results of the surgical procedure. METHODS: Between September 2005 and December 2013, 68 patients were treated by a single surgeon (DMW) with distal catheter replacement using our technique. In brief, the previously placed distal catheter was exposed at its entry site into the abdomen. A soft guidewire with hydrophilic coating was inserted down the distal catheter into the peritoneum. The distal catheter was then removed over the guidewire, leaving the guidewire in place. A peel-away sheath and dilator were then inserted over the guidewire, and the dilator and guidewire were removed. The new distal catheter was then passed from the valve to the abdomen and was then fed through the peel-away sheath into the peritoneum. Charts were retrospectively reviewed for preoperative presentation, operative technique, and postoperative outcome. Records were specifically examined for any early or late complications. RESULTS: The mean patient age at surgery was 13 years. No immediate acute complications were noted. Of the 68 total patients, 45 patients had more than 6 months of follow-up. Of the 68 patients, 7 patients required another distal revision after guidewire-assisted distal catheter replacement. CONCLUSION: Distal shunt malfunction due to a mechanical failure is a common reason for shunt revision. We describe a technique for guidewire-assisted distal catheter replacement.

Utility of surveillance imaging after minor blunt head trauma.

OBJECT: Nonoperative blunt head trauma is a common reason for admission in a pediatric hospital. Adverse events, such as growing skull fracture, are rare, and the incidence of such morbidity is not known. As a result, optimal follow-up care is not clear. METHODS: Patients admitted after minor blunt head trauma between May 1, 2009, and April 30, 2013, were identified at a single institution. Demographic, socioeconomic, and clinical characteristics were retrieved from administrative and outpatient databases. Clinical events within the 180-day period following discharge were reviewed and analyzed. These events included emergency department (ED) visits, need for surgical procedures, clinic visits, and surveillance imaging utilization. Associations among these clinical events and potential contributing factors were analyzed using appropriate statistical methods. RESULTS: There were 937 admissions for minor blunt head trauma in the 4-year period. Patients who required surgical interventions during the index admission were excluded. The average age of the admitted patients was 5.53 years, and the average length of stay was 1.7 days; 15.7% of patients were admitted for concussion symptoms with negative imaging findings, and 26.4% of patients suffered a skull fracture without intracranial injury. Patients presented with subdural, subarachnoid, or intraventricular hemorrhage in 11.6%, 9.19%, and 0.53% of cases, respectively. After discharge, 672 patients returned for at least 1 follow-up clinic visit (71.7%), and surveillance imaging was obtained at the time of the visit in 343 instances. The number of adverse events was small and consisted of 34 ED visits and 3 surgeries. Some of the ED visits could have been prevented with better discharge instructions, but none of the surgery was preventable. Furthermore, the pattern of postinjury surveillance imaging utilization correlated with physician identity but not with injury severity. Because the number of adverse events was small, surveillance imaging could not be shown to positively influence outcomes. CONCLUSIONS: Adverse events after nonoperative mild traumatic injury are rare. The routine use of postinjury surveillance imaging remains controversial, but these data suggest that such imaging does not effectively identify those who require operative intervention.

Return to system within 30 days of pediatric neurosurgery.

OBJECT: Quality assessment measures have not been well developed for pediatric neurosurgical patients. This report documents the authors' experience in extracting information from an administrative database to establish the rate of return to system within 30 days of pediatric neurosurgical procedures. METHODS: Demographic, socioeconomic, and clinical characteristics were prospectively collected in administrative, business, and operating room databases. The primary end point was an unexpected return to the hospital system within 30 days from the date of a pediatric neurosurgical procedure. Statistical methods were used to identify clinical and demographic factors associated with the primary end point. RESULTS: There were 1358 pediatric neurosurgical procedures performed in the Children's Healthcare of Atlanta operating rooms in 2012, with 37.4% of these surgeries being preceded by admissions through the emergency department. Medicare or Medicaid was the payor for 54.9% of surgeries, and 37.6% of surgeries were shunt related. There were 148 unexpected returns to the system within 30 days after surgery, and in 109 of these cases, the patient had a presenting complaint that was attributable to the index surgery (related returns). The most common complaints were headache, nausea, vomiting, or seizure after shunt revision or cranial procedures (n = 62). The next most common reason for re-presentation was for wound concerns (n = 30). Thirty-seven of the 109 related returns resulted in a reoperation. The monthly rate of related returns was 8.1% ± 2.5% over the 12-month study period. When using related returns as the dependent variable, the authors found that patients who underwent a shunt-related surgery were both more likely to unexpectedly return to the system (OR 1.86, p = 0.008) and to require surgery upon readmission (OR 3.28, p = 0.004). Because an extended hospitalization shortened the window of time for readmission after surgery, extended length of stay was protective against return to system within 30 days of surgery. Importantly, if related and unrelated returns were analyzed together as the dependent variable (n = 148), no independent clinical and demographic risk factor could be identified. CONCLUSIONS: Quality assessment measures need to be clearly and carefully defined, as the definition itself will impact the analytical results. Clinicians must play a leading role in the development of these measures to ensure their clinical meaningfulness.

Epstein-Barr virus associated smooth muscle tumors in post transplant pediatric patients two cases of rare locations, and review of the literature.

Epstein-Barr virus (EBV) may present few or no symptoms in immunocompetent individuals; however, in immunocompromised patients as in the case of AIDS and post-transplant patients, the virus occasionally stimulates neoplastic transformations. Epstein-Barr virus may play a role in the development of smooth muscle tumors (SMT). In the case of Epstein-Barr associated smooth muscle tumors (EBV+SMT), the virus is thought to be the leading factor to the tumorigenic pathway. We report two pediatric patients (6 and 13 years old) who underwent liver transplantation and developed EBV+SMT in the colon and orbit. These two cases represent rare locations for this kind of lesion.

The use of inside-outside screws for occipitocervical fusion in pediatric patients.

OBJECT: The authors describe the use of inside-outside occipital screws in 21 children with occipitocervical instability requiring occipitocervical fusion. METHODS: The ages of the patients were from 2 to 15 years, and patients presented with a variety of causes of occipitocervical instability, including congenital disorders, posttraumatic instability, idiopathic degeneration, and postoperative instability. Surgeries frequently included foramen magnum decompression, duraplasty, and laminectomy, but all patients required occipitocervical instrumentation and arthrodesis. Postoperative orthosis included the use of either a cervical collar or halo device. In all but one case, patients were followed postoperatively for at least 12 months. RESULTS: The mean age of patients was 9.93 years. Inside-outside screws were used in all reported cases. Rib autograft was used in all patients. In addition, demineralized bone matrix was used in 2 cases, and bone morphogenetic protein was used in 2 patients. Two patients required halo placement, and the other 19 were placed in cervical collars. The average time postoperative orthotics were used was 2.82 months. Arthrodesis was determined radiographically and was noted in all patients. No operative complications were noted; however, postoperative complications included 1 wound infection, 2 cases of hardware loosening, and the need for tracheostomy in 2 patients. CONCLUSIONS: Inside-outside screws were found to be a useful component of occipitocervical instrumentation in pediatric patients ranging from 2 to 15 years of age. Arthrodesis was demonstrated in all cases.

Transient enlargement of craniopharyngioma after radiation therapy: pattern of magnetic resonance imaging response following radiation.

Clinical experience suggests that craniopharyngiomas may temporarily increase in size after radiation therapy (RT). The study goal is to determine the incidence and natural history of this response in a cohort of patients managed at Children's Healthcare of Atlanta (CHOA) or Emory Healthcare (EHC). Between 08/1998 and 06/2009, 41 children and young adults were diagnosed with craniopharyngioma at CHOA and/or EHC. Of these, 21 received external-beam radiation and were included in our analysis. Serial magnetic resonance imaging (MRI) studies were evaluated volumetrically to assess response to RT. Median age at diagnosis was 8.2 years (range 3.2-23.5 years). Median radiation dose was 54.0 Gy using standard fractionation (1.8-2.0 Gy/day). With median follow-up of 41.3 months (range 7.2-121.8 months), actuarial local control and overall survival rates at 5 years were 78.7 % and 100 %, respectively. Of subjects, 52.4 % of subjects (11 of 21) were noted on serial MRI evaluation to have tumor enlargement (mostly cystic component) after radiation before eventual shrinkage without further intervention. For tumors that expanded, the median volume increase was 33.9 % (range 15.6-224.4 %). Median time to maximal tumor/cyst expansion was 1.5 months (range 1.0-5.0 months). Finally, nearly all patients (20 of 21) showed a measurable objective response to therapy by MRI regardless of ultimate disease control. Median time to maximal response post-radiation, as defined by MRI, was 9.5 months (range 3.5-39.9 months). In summary, RT is effective for managing craniopharyngioma. However, despite good ultimate responses, approximately 50 % of the patients show tumor/cyst expansion on MRI over the first few months post-radiation. Caution should be taken not to subject these patients to "salvage surgery" or cyst aspiration during this early time unless there are other overriding surgical indications. Understanding the natural history of this phenomenon could potentially help guide the management of these craniopharyngioma patients.

Cutis aplasia of the scalp: three cases in eighteen months.

Three patients with cutis aplasia of the scalp presented to Arkansas Children's Hospital over a period of eighteen months. Two of the patients required operative intervention for closure of the defects and the third patient healed with dressing changes. Surgery required the combined efforts of both neurosurgery and plastic surgery.