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Amir Ahmadian, Jotham Manwaring, Devon Truong, Jeane McCarthy, Luis F. Rodriguez, Carolyn M. Carey, and Gerald F. Tuite

Vascular access in the neonate can be challenging, especially in preterm infants. When other access is not available, superficial scalp veins can be safely used for vascular access. However, rare and potentially catastrophic complications can occur due to unique features of the neonatal skull and soft-tissue anatomy. The authors report a rare complication of vascular access in a preterm infant, which led to the direct infusion of parenteral nutrition into the intracranial space. The child had an excellent outcome after open drainage and irrigation of bilateral intracranial spaces and the spinal thecal sac. Relevant anatomy is illustrated, and an outcome-based literature review is presented on this rarely reported condition. Surgical and conservative management strategies are discussed, along with clinical and radiographic follow-up. Drainage and irrigation is advocated in patients with mass effect, viscous effusions, or declining neurological examination findings.

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Stephen L. Reintjes, Ernest K. Amankwah, Luis F. Rodriguez, Carolyn C. Carey, and Gerald F. Tuite

OBJECT

Fusion rates are high for children undergoing posterior cervical fusion (PCF) and occipito-cervical fusion (OCF). Autologous bone has been widely used as the graft material of choice, despite the risk of donor-site morbidity associated with harvesting the bone, possibly because very low fusion rates were reported with posterior allograft cervical fusions in children several decades ago. Higher overall fusion rates using allograft in adults, associated with improvements in internal fixation techniques and the availability of osteoinductive substances such as bone morphogenetic protein (BMP), have led to heightened enthusiasm for the use of bank bone during pediatric PCF. A systematic review was performed to study factors associated with successful bone fusion, including the type of bone graft used.

METHODS

The authors performed a comprehensive PubMed search of English-language articles pertaining to PCF and OCF in patients less than 18 years old. Of the 561 abstracts selected, 148 articles were reviewed, resulting in 60 articles that had sufficient detail to be included in the analysis. A meta-regression analysis was performed to determine if and how age, fusion technique, levels fused, fusion substrate, BMP use, postoperative bracing, and radiographic fusion criteria were related to the pooled prevalence estimates. A systematic review of the literature was performed according to the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) statement.

RESULTS

A total of 604 patients met the specific inclusion and exclusion criteria. The overall fusion rate was 93%, with a mean age of 9.3 years and mean follow-up of 38.7 months. A total of 539 patients had fusion with autograft (94% fusion rate) and 65 patients with allograft (80% fusion rate). Multivariate meta-regression analysis showed that higher fusion rates were associated with OCF compared with fusions that excluded the occiput (p < 0.001), with the use of autograft instead of allograft (p < 0.001), and with the use of CT to define fusion instead of plain radiography alone. The type of internal fixation, the use of BMP, patient age, and the duration of follow-up were not found to be associated with fusion rates in the multivariate analysis.

CONCLUSIONS

Fusion rates for PCF are high, with higher rates of fusion seen when autograft is used as the bone substrate and when the occiput is included in the fusion construct. Further study of the use of allograft as a viable alternative to autograft bone fusion is warranted because limited data are available regarding the use of allograft in combination with more rigid internal fixation techniques and osteoinductive substances, both of which may enhance fusion rates with allograft.

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Amir Ahmadian, Ali A. Baa j, Michael Garcia, Carolyn Carey, Luis Rodriguez, Bruce Storrs, and Gerald F. Tuite

The authors present a case of extreme brain herniation encountered during decompressive craniectomy in a 21-month-old boy who suffered a trauma event that necessitated temporary scalp closure in which a sterile silicone sheet was placed. Although the clinical situation is usually expected to lead to brain death or severe disability, the patient's 3-year follow-up examination revealed a highly functional child with a good quality of life. The authors discuss the feasibility and advantages of temporary scalp expansion as a treatment option when extreme brain herniation is encountered during craniotomy.

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Andrew C. Vivas, Nir Shimony, Eric M. Jackson, Risheng Xu, George I. Jallo, Luis Rodriguez, Gerald F. Tuite, and Carolyn M. Carey

OBJECTIVE

Hydrocephalus associated with subdural hygromas is a rare complication after decompression of Chiari malformation type I (CM-I). There is no consensus for management of this complication. The authors present a series of 5 pediatric patients who underwent CM-I decompression with placement of a dural graft complicated by posterior fossa hygromas and hydrocephalus that were successfully managed nonoperatively.

METHODS

A retrospective review over the last 5 years of patients who presented with hydrocephalus and subdural hygromas following foramen magnum decompression with placement of a dural graft for CM-I was conducted at 2 pediatric institutions. Their preoperative presentation, perioperative hospital course, and postoperative re-presentation are discussed with attention to their treatment regimen and ultimate outcome. In addition to reporting these cases, the authors discuss all similar cases found in their literature review.

RESULTS

Over the last 5 years, the authors have encountered 194 pediatric cases of CM-I decompression with duraplasty equally distributed at the 2 institutions. Of those cases, 5 pediatric patients with a delayed postoperative complication involving hydrocephalus and subdural hygromas were identified. The 5 patients were managed nonoperatively with acetazolamide and high-dose dexamethasone; dosages of both drugs were adjusted to the age and weight of each patient. All patients were symptom free at follow-up and exhibited resolution of their pathology on imaging. Thirteen similar pediatric cases and 17 adult cases were identified in the literature review. Most reported cases were treated with CSF diversion or reoperation. There were a total of 4 cases previously reported with successful nonoperative management. Of these cases, only 1 case was reported in the pediatric population.

CONCLUSIONS

De novo hydrocephalus, in association with subdural hygromas following CM-I decompression, is rare. This presentation suggests that these complications after posterior fossa decompression with duraplasty can be treated with nonoperative medical management, therefore obviating the need for CSF diversion or reoperation.

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Samuel Browd, Lindsay J. Healy, Ginger Dobie, J. Thomas Johnson III, Greg M. Jones, Luis F. Rodriguez, and Douglas L. Brockmeyer

Object

Congenital occipitocervical (OC) instability is uncommon in healthy children but can occur in many children with Down syndrome. A simple morphometric method of evaluating the OC joint in children with OC instability is presented, supported by a qualitative image analysis based on computed tomography (CT).

Methods

Thin-cut CT scans of the OC joint were obtained in eight patients with Down syndrome and one patient with congenital OC instability. These patients’ CT scans were compared with those of 15 healthy age-matched control individuals. Morphometric analysis was performed by measuring the depth and length of the superior articular surface (SAS) of C-1, and these values were normalized for a comparison between groups. Qualitative data were acquired using a surface-rendering technique for a visual comparison of the C-1 SAS.

Morphometric analysis demonstrated an absence of the concave C-1 SAS anatomy in patients with congenital OC instability compared with age-matched control individuals (0.083 compared with 0.202, p < 0.001). Three-dimensional (3D) image analysis of the C-1 SAS supported this finding.

Conclusions

Congenital differences in the shape of the OC joint are highly associated with atraumatic OC instability in children with Down syndrome. High-resolution CT imaging combined with 3D rendering techniques and surface mapping provides support for this assessment. It appears that abnormal OC joint shape is a contributing factor to congenital OC instability, especially in patients with Down syndrome.

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Jotham C. Manwaring, Devon Truong, Armen R. Deukmedjian, Carolyn M. Carey, Bruce B. Storrs, Luis F. Rodriguez, Lisa Tetreault, and Gerald F. Tuite

The management of newborns with extreme macrocephaly related to hydrocephalus can be difficult; balancing the treatment of severe cranial deformity with optimal hydrocephalus management can be complicated. Excessive CSF drainage can result in significant suture overlap that leads to difficulties in patient positioning, secondary synostosis, and long-term aesthetic complications. Delayed cranial reduction and remodeling procedures carry significant risk, and the aesthetic outcomes have sometimes been poor.

The authors describe a newborn with severe macrocephaly who underwent shunt placement followed by a limited cranial reduction and fixation procedure using an absorbable plate within the 1st week of life. The procedure produced an immediate intracranial volume reduction of 49%. This novel management strategy facilitated patient positioning, simplified hydrocephalus management, and provided an excellent aesthetic outcome.

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Gerald F. Tuite, Bruce B. Storrs, Yves L. Homsy, Sarah J. Gaskill, Ethan G. Polsky, Margaret A. Reilly, Ignacio Gonzalez-Gomez, S. Parrish Winesett, Luis F. Rodriguez, Carolyn M. Carey, Sharon A. Perlman, and Lisa Tetreault

An intradural somatic-to-autonomic anastomosis, or Xiao procedure, has been described to create a “skin-CNS-bladder” reflex that improves bladder and bowel function in patients with neurogenic bladder and bowel dysfunction. The authors present their experience with a 10-year-old boy with chronic neurogenic bladder and bowel dysfunction related to spinal cord injury who underwent the Xiao procedure. After undergoing a left L-5 ventral root to left S2–3 intradural anastomosis, the patient reported that his bladder and bowel dysfunction improved between 6 and 12 months. Two years after the procedure, however, he reported that there was no change in his bladder or bowel dysfunction as compared with his condition prior to the procedure. Frequent, systematic multidisciplinary evaluations produced conflicting data.

Electrophysiological and histological evaluation of the previously performed anastomosis during surgical reexploration 3 years after the Xiao procedure revealed that the anastomosis was in anatomical continuity but neuroma formation had prevented reinnervation. Nerve action potentials were not demonstrable across the anastomosis, and stimulation of the nerve above and below the anastomosis created no bladder or perineal contractions.

This is the first clinical report on the outcome of the Xiao procedure in a child with spinal cord injury outside of China. It is impossible to draw broad conclusions about the efficacy of the procedure based on a single patient with no demonstrable benefit. However, future studies should carefully interpret transient improvements in bladder function, urodynamic findings, and the patient's ability to void in response to scratching after the Xiao procedure. The authors' experience with the featured patient, in whom reinnervation could not be demonstrated, suggests that such changes could be related to factors other than the establishment of a skin-CNS-bladder reflex as a result of a somatic-to-autonomic anastomosis.

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Nir Shimony, Travis Dailey, David Barrow, Anh Bui, Mohammad Hassan A. Noureldine, Meleine Martínez-Sosa, Luis F. Rodriguez, Carolyn M. Carey, Gerald F. Tuite, and George I. Jallo

OBJECTIVE

Pediatric traumatic brain injury (TBI) is the leading cause of death among children and is a significant cause of morbidity. However, the majority of injuries are mild (Glasgow Coma Scale score 13–15) without any need for neurosurgical intervention, and clinically significant neurological decline rarely occurs. Although the question of repeat imaging within the first 24 hours has been discussed in the past, the yield of short-term follow-up imaging has never been thoroughly described. In this paper, the authors focus on the yield of routine repeat imaging for pediatric mild TBI (mTBI) at the first clinic visit following hospital discharge.

METHODS

The authors conducted a retrospective review of patients with pediatric brain trauma who had been admitted to Johns Hopkins All Children’s Hospital (JHACH). Patients with mTBI were identified, and their presentation, hospital course, and imaging results were reviewed. Those pediatric patients with mTBI who had undergone no procedure during their initial admission (only conservative treatment) were eligible for inclusion in the study. Two distinct groups were identified: patients who underwent repeated imaging at their follow-up clinic visit and those who underwent only clinical evaluation. Each case was assessed on whether the follow-up imaging had changed the follow-up course.

RESULTS

Between 2010 and 2015, 725 patients with TBI were admitted to JHACH. Of those, 548 patients qualified for analysis (i.e., those with mTBI who received conservative treatment without any procedure and were seen in the clinic for follow-up evaluation within 8 weeks after the trauma). A total of 392 patients had only clinic follow-up, without any diagnostic imaging study conducted as part of their clinic visit, whereas the other 156 patients underwent repeat MRI. Only 1 patient had a symptomatic change and was admitted after undergoing imaging. For 30 patients (19.2%), it was decided after imaging to continue the neurosurgical follow-up, which is a change from the institutional paradigm after mTBI. None of these patients had a change in neurological status, and all had a good functional status. All of these patients had one more follow-up in the clinic with new MRI, and none of them required further follow-up.

CONCLUSIONS

Children with mTBI are commonly followed up in the ambulatory clinic setting. The authors believe that for children with mTBI, normal clinical examination, and no new symptoms, there is no need for routine ambulatory imaging since the clinical yield of such is relatively low.

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Douglas L. Brockmeyer, W. Jerry Oakes, Curtis Rozzelle, James Johnston, Brandon G. Rocque, Richard C. E. Anderson, Neil Feldstein, Jonathan Martin, Gerald F. Tuite, Luis Rodriguez, Nicholas Wetjen, Phillip Aldana, Dave Pincus, Phillip Storm, Mark R. Proctor, and Sean Lew

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Gerald F. Tuite, Ethan G. Polsky, Yves Homsy, Margaret A. Reilly, Carolyn M. Carey, S. Parrish Winesett, Luis F. Rodriguez, Bruce B. Storrs, Sarah J. Gaskill, Lisa L. Tetreault, Denise G. Martinez, and Ernest K. Amankwah