Patrick Graupman

New resorbable plate and screw system in pediatric craniofacial surgery.

Abstract Early forms of resorbable fixation induced foreign body reactions requiring surgical removal of the fixation material. Most current plating systems attempt to circumvent this phenomenon by altering the composition of the &agr; esters involved. ResorbX (KLS Martin, Jacksonville, FL), a plating system derived from 50:50 poly(D,L)lactide, boasts short resorption times, minimal foreign body reaction, and adequate strength for bony fixation. We present our experience with 134 patients undergoing correction of primary craniosynostosis, using ResorbX. One hundred thirty-four consecutive craniosynostosis patients underwent correction with calvarial remodeling by the senior author between April 2002 and March 2008. Five patients had 2-stage repairs. Postoperative analysis included plate visibility or palpability, head contour, and the incidence of complications. The mean age at repair was 17.0 months, with the median age being 9.0 months (range, 2.5–137 months). Postoperative follow-up visits were recorded at 3-month intervals from 0 to 24 months. Synostosis diagnoses were as follows: 43 metopic, 37 sagittal, 27 coronal, 5 lambdoid, and 27 multisutural synostosis. There were 3 complications requiring operative intervention. Three plates became exposed through the coronal excision, requiring removal, for an overall complication rate of 2%. Forty-four patients (31.7%) had a visible mass at the site of fixation at some point postoperatively; all of these resolved through observation only. Overall postoperative head aesthetics were deemed satisfactory to excellent, and no instances of contour regression occurred. This study documents the safety and efficacy of ResorbX in pediatric cranial vault remodeling. The system is easy to use, and with the advent of an ultrasonic pin, more abbreviated operating and anesthesia times are achievable.

Craniofacial abnormalities in a murine knock-out model of mucopolysaccharidosis I H: a computed tomography and anatomic study.

The genetic mucopolysaccharidoses are a group of lysosomal storage diseases in which mucopolysaccharides (glycosaminoglycans) accumulate as the result of a malfunction or lack of a lysosomal degradation enzyme. There are currently seven known forms of mucopolysaccharidoses. Type I results from an enzymatic deficiency of alpha-l-iduronidase. There are three subtypes of mucopolysaccharidoses I that are commonly recognized: Hurler syndrome, Hurler-Scheie syndrome, and Scheie syndrome. Of the three subtypes, Hurler syndrome has the most severe clinical picture. Craniofacial anomalies and cognitive impairment are some of the more pronounced features of Hurler syndrome. Hurler syndrome has been described in cats, dogs, mice, and human beings and is inherited as an autosomal recessive trait. The biochemical nature of the disease is preserved across species lines. Clinically, the disease has similar effects in human beings and animals. It has been difficult to reverse the phenotype of the disease even with replacement of the defective alpha-l-iduronidase enzyme. The purpose of this study is to characterize the cranio-facial differences in the murine knock-out model of Hurler syndrome objectively. Twenty-three measurements were taken from computed tomographic scans in a coronal and sagittal plane on 24 black C57/B6 knock-out Hurler syndrome mice. The seven statistically significant measurements are width of the cervical canal, height of the foramen magnum, width between the external auditory canals, width of the skull base at the mandibular condyles, midocular distance, spread of the mandibular condyles, and width of the zygoma at the maxilla. This information now provides researchers with objective data from living Hurler syndrome-affected mice that will allow them to follow therapies directed at improving craniofacial outcomes for any therapy over time.

Pediatric intracranial complications of central venous catheter placement.

Central venous catheters are essential in the medical management of critically ill pediatric patients. Medical practitioners should be aware of the potential for misplacement of these devices as such complications may result in serious injury and possible death. Catheter malpositioning is not limited to any particular site and may have far-reaching consequences that affect a single or multiple organ systems. We present two cases where central venous catheter positioning led to complications that resulted in intracranial pathology which was fatal in one case.

Meckel-gruber syndrome in association with an occipital meningocele

and acute and chronic infl ammation of the wall. The large cyst was partially fi lled with necrotic debris and no neural elements could be identifi ed. He was discharged home postoperatively to the care of his parents. He died 10 days later. A prenatal ultrasound at 18 weeks’ gestation demonstrated a male fetus with an occipital meningocele, a Dandy-Walker malformation, and enlarged cystic kidneys. The pregnancy was otherwise uncomplicated. The patient was born at 34 weeks’ gestation by cesarean section. Based on the triad of fi ndings on ultrasound he was diagnosed with Meckel-Gruber syndrome. He was discharged home shortly after birth for comfort care due to the universally poor prognosis associated with Meckel-Gruber syndrome. In spite of his poor prognosis, he was noted to be sucking, feeding, and gaining weight. He was referred for neurosurgical evaluation due to the large size of his meningocele, which caused considerable diffi culty for the parents in caring for the neonate ( fi g. 1, 2 ). Upon presentation at 10 days of age, he was noted on examination to have a large fl uctuant bilobed occipital mass. His fontanelle was fl at and soft with slightly splayed sutures. He had normal facial features and a well-formed palate. He had normal fi ngers and toes, both in number and in appearance. He demonstrated an abnormal breathing pattern, consisting of intermittent episodes of labored respirations at an increased rate, only to return to normal again. MRI confi rmed the prenatal ultrasound fi ndings ( fi g. 3, 4 ). The occipital meningocele was removed and the post-operative course was unremarkable. Pathology demonstrated a cystic structure with vascular proliferation

Air gun orbitocranial penetrating injury: emergency endovascular treatment and surgical bypass following pellet migration to middle cerebral artery: case report

The authors describe a 14-year-old boy presenting with an orbitocranial penetrating injury (OPI) from a metallic air gun pellet to the left eye who developed hemiparesis and speech difficulty due to migration of the pellet to the left middle cerebral artery. They highlight the potential complications associated with both OPIs and intravascular foreign body migration and occlusion by describing the patients presentation, results of imaging evaluation, and the combined endovascular treatment and extracranial-intracranial bypass, which resulted in rapid restoration of blood flow and full neurological recovery with intact vision. Based on this case and a review of the literature on intracranial foreign body migration with resultant vascular occlusion, the authors recommend that complex OPIs be treated at centers that offer both neuroendovascular and neurovascular surgical capabilities on an urgent basis to manage both the primary injury and potential secondary vascular compromise.