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.
Amir Ahmadian, Ali A. Baa j, Michael Garcia, Carolyn Carey, Luis Rodriguez, Bruce Storrs, and Gerald F. Tuite
Andrew C. Vivas, Nir Shimony, Eric M. Jackson, Risheng Xu, George I. Jallo, Luis Rodriguez, Gerald F. Tuite, and Carolyn M. Carey
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.
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.
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.
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.
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.
Rajiv R. Iyer, Carolyn M. Carey, S. Alex Rottgers, Lisa Tetreault, Nir Shimony, Jennifer Katzenstein, Ernesto Ruas, and Gerald F. Tuite
Infants with severe hydrocephalus and extreme macrocephaly typically undergo CSF diversion early in life, which can result in significant cranial deformity due to CSF overdrainage. In this scenario, overlap of the cranial plates can precede the development of secondary synostosis and/or severe, permanent cranial deformity. As a result, extensive cranial vault remodeling is sometimes undertaken later in life, which is often challenging and has been associated with mortality and a high morbidity rate. The authors have previously described a technique for early postnatal cranial vault reduction and fixation (CVRF), in which the calvarial bones are stabilized using absorbable fixation plates in the neonatal period, in an attempt to facilitate patient positioning, simplify hydrocephalus management, and improve cosmesis. Here, the authors describe their institutional experience managing patients with extreme neonatal hydrocephalus with CSF diversion, with and without CVRF, over the past 12 years.
The authors retrospectively reviewed the charts of infants with extreme hydrocephalus (head circumference > 49 cm) treated at their children’s hospital with ventriculoperitoneal shunting, with or without CVRF, between 2005 and 2017. Data collected included age, sex, etiology of hydrocephalus, type of CVRF performed (anterior, posterior, or combined), follow-up duration, orbitofrontal circumference, craniometric measurements, intraoperative blood loss, operative duration, and postoperative complications. Developmental data were collected using the third edition of the Ages and Stages Questionnaire. Photographic imaging was used to demonstrate esthetic outcomes, and family questionnaires were used to evaluate satisfaction with the esthetic outcome.
Eleven patients with extreme neonatal hydrocephalus underwent CSF shunting; 5 underwent shunting alone and 6 patients underwent shunting and CVRF. For patients who underwent shunting and CVRF, the median age at CVRF was 6 days and the median interval between shunt placement and CVRF was 2.5 days. The mean extent of calvarial vault volume reduction was 44.5% (± 3.9%). The mean duration of the CVRF procedure was 108 minutes, and 5 of 6 patients required intraoperative transfusion. Of the 5 patients who underwent shunting alone, 3 developed severe cranial deformities. Of 6 patients who underwent shunting and CVRF, 1 had a poor cosmetic outcome. In the shunting-alone group, 2 patients died and 1 required extensive cranial vault correction at 10 years of age. One patient in the shunting and CVRF group also died.
CVRF in combination with CSF shunting in the neonatal period can simplify the treatment of the rare case of severe hydrocephalic macrocephaly and leads to cosmetic outcomes that are considered good by their families.
Gerald F. Tuite, Carolyn M. Carey, William W. Nelson, Scott J. Raffa, and S. Parrish Winesett
Profuse bleeding originating from an injured cerebral sinus can be a harrowing experience for any surgeon, particularly during an operation on a young child. Common surgical remedies include sinus ligation, primary repair, placement of a hemostatic plug, and patch or venous grafting that may require temporary stenting. In this paper the authors describe the use of a contoured bioresorbable plate to hold a hemostatic plug in place along a tear in the inferomedial portion of a relatively inaccessible part of the posterior segment of the superior sagittal sinus in an 11-kg infant undergoing hemispherotomy for epilepsy. This variation on previously described hemostatic techniques proved to be easy, effective, and ultimately lifesaving. Surgeons may find this technique useful in similar dire circumstances when previously described techniques are ineffective or impractical.
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.
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.
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
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.
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.
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.
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.