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David F. Jimenez and Constance M. Barone

Object

The objective of this study was to present the authors' 16-year experience treating coronal craniosynostosis in infants using endoscopy-assisted techniques and postoperative cranial orthoses.

Methods

A total of 128 synostosed coronal sutures in 115 patients were treated between 1996 and 2012 by endoscopically resecting a strip of bone containing the stenosed suture via a 2–3 cm incision made at the ipsilateral stephanion. Data were obtained from a prospective database. Following surgery, patients were fitted with custom cranial orthoses to help correct preoperative craniofacial deformities. All patients were followed closely with cranial anthropometric measurements and photographs.

Results

The estimated mean blood loss was 20 ml (range 5–120 ml) and the estimated mean strip size was 0.6 cm × 10.7 cm. The mean surgical duration was 55 minutes (range 22–150 minutes). One patient underwent an intraoperative blood transfusion and 1 had a postoperative blood transfusion, for a total transfusion rate of 1.7%. Ninety-seven percent of patients were discharged on the first postoperative day. There were no deaths. Vertical dystopia correction of more than 80% from baseline was obtained in almost two-thirds of patients, with 51% achieving 100% correction. Nasal and sagittal craniofacial deviation (vertex-nasion-gnathion) correction greater than 80% was achieved in 80% of patients, with 77% achieving 100% correction. Supraorbital rim advancement of the ipsilateral eye was obtained in 98% of cases, with correction of frontal plagiocephaly the last deformity to achieve correction.

Conclusions

Early treatment of coronal synostosis with endoscopy-assisted craniectomy and postoperative molding helmets leads to significant correction of craniofacial abnormalities, including vertical dystopia, nasal deviation, sagittal misalignment, and ipsilateral proptosis. This treatment method is associated with minimal trauma, blood loss, and transfusion rates, and typically only requires 1 overnight stay. This surgical approach is safe, effective, and associated with excellent results.

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David F. Jimenez and Constance M. Barone

Object

The authors present the results of treating infants with multiple-suture nonsyndromic craniosynostosis in whom the authors used minimally invasive endoscopy-assisted techniques and postoperative cranial molding over an 11-year period.

Methods

A total of 21 patients who presented with multiple-suture (nonsyndromic) craniosynostosis were treated using minimally invasive endoscopy-assisted craniectomies. Surgery was followed by treatment with custommade cranial orthoses for up to 12 months. A total of 48 sutures were treated. The most common was the coronal suture (38 cases) and this was followed by the sagittal (11 cases), metopic (6 cases), and lambdoid (3 cases) sutures. There were 13 male and 8 female pediatric patients. Their ages ranged between 3 weeks and 9 months (mean 3.2 months, median 2.5 months). The sagittal suture was treated with a wide vertex craniotomy via 2 incisions located behind the anterior fontanel and in front of the lambda. The metopic suture underwent a suturectomy as did the coronal and lambdoid sutures.

Results

The mean follow-up duration was 61 months (range 3–135 months). There were no deaths. In patients with bicoronal synostosis, brachycephaly was corrected. Patients presenting with vertical dystopia or nasal deviation had these deformities corrected as well. The mean blood loss was 42 ml (range 10–120 ml). The mean hospital length of stay was 1 day. The intraoperative transfusion rate was 0%. The results indicate that nonsyndromic multiple-suture synostosis can be safely and effectively treated using endoscopic techniques.

Conclusions

Early treatment of complex multiple-suture synostosis with endoscopic techniques provides an excellent surgical alternative. The results of the present study indicate marked correction of skull base and craniofacial deformities. Endoscopy provides a safe and effective way to treat these patients.

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David F. Jimenez and Constance M. Barone

Object. The authors sought to minimize scalp incisions, blood loss, and operative time by using endoscopically assisted strip craniectomies and barrel-stave osteotomies to treat infants with sagittal suture synostosis.

Methods. Four patients, aged 2, 4, 9, and 12 weeks, who presented with scaphocephaly underwent endoscopic midline craniectomies through small midline scalp incisions. The mean operative time for the procedure was 1.68 hours (range 1.15–2.8 hours); the mean blood loss was 54.2 ml (range 12–150 ml). Three patients did not require blood transfusions and were discharged within 24 hours. Postoperatively, all patients were fitted with custom cranial molding helmets. Follow-up evaluation ranged between 8 and 15 months. All patients had successful correction of their scaphocephaly with no mortalities, morbidities, or complications.

Conclusions. The use of endoscopic techniques for early correction of sagittal synostosis is safe; decreases blood loss, operative time, and hospitalization costs; and provides excellent early surgical results.

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David F. Jimenez and Constance M. Barone

Object

The purpose of this study was to assess the efficacy, safety, associated complications, and outcome in patients with sagittal suture craniosynostosis in whom endoscopy-assisted wide-vertex craniotomy and “barrel-stave” osteotomy were performed.

Methods

During a 4-year period, 59 patients with sagittal suture synostosis underwent endoscopy-assisted wide-vertex craniectomies, barrel stave–like osteotomies, and postoperatively were fitted with custom-made molding helmets. Data on operative time, blood loss, transfusion rates, hospital length of stay, complications, and hospital charges were collected prospectively. The mean patient age at the time of surgery was 3.7 months. The average blood loss was 31.8 ml; and only one patient required an intraoperative blood transfusion. Nine patients received transfusions of donor blood postoperatively. The mean operative time was 50 minutes, and all but three patients were discharged from the hospital the morning following surgery. There were no intraoperative complications. Normocephaly as well as normal cephalic indices were observed at latest follow up.

Conclusions

The authors conclude that early treatment of infants with sagittal suture craniosynostosis by using minimally invasive, endoscopy-assisted wide-vertex craniectomies provides excellent results and a significantly lower morbidity rate than traditional calvarial vault reconstructive procedures.

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David F. Jimenez and Constance M. Barone

Patients with Apert syndrome commonly present with ocular proptosis due to bilateral coronal craniosynostosis and midfacial hypoplasia. Severe proptosis can cause visual compromise and damage, which is most commonly treated with bilateral orbital frontal advancement. The authors present the case of a patient who was treated at 8 weeks of age with endoscope-assisted bilateral coronal craniectomies followed by treatment with a custom-made postoperative cranial orthosis. The patient underwent the procedure without any complications. Over the ensuing months, the patient's proptosis corrected, the forehead and orbital rims advanced without the need for an orbital frontal advancement and craniotomies. This approach may provide an alternative treatment modality for these patients.

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John David Morenski, Joseph D. Tobias and David F. Jimenez

✓ Brain injury remains one of the leading causes of death and disability in children. Appropriate therapy involves aggressive management of intracranial pressure (ICP) and cerebral perfusion pressure, which often requires placement of an intraparenchymal ICP monitor or intraventricular catheter. These potentially life-saving interventions require normal coagulation function; however, several factors may lead to coagulopathy in the head-injured patient. Standard therapies, which often include multiple doses of fresh frozen plasma (FFP), have a number of drawbacks when used in the pediatric population. The use of FFP requires time to type and crossmatch, thaw, and administer. It imposes a significant volume load on a child in whom cerebral edema remains a problem. Success in using recombinant activated factor VII (rFVIIa) in the hemophiliac population suggests an alternative therapy.

Three patients suffered severe coagulopathy after cerebral injury. One patient received rFVIIa after repeated doses of FFP had failed to correct the coagulopathy; the other two patients received rFVIIa as the initial therapy. Treatment with rFVIIa consisted of a bolus of 90 µg/kg. Recombinant activated factor VII rapidly corrected the patients' coagulopathies, which allowed placement of intraparenchymal fiberoptic lines and intraventricular catheters to monitor ICP. The patients suffered no complication from the placement of ICP monitoring devices, as demonstrated on computerized tomography scans obtained within 24 hours after placement.

Brain injury—induced coagulopathy may lead to significant secondary injury and delays the invasive monitoring necessary for the aggressive management of intracranial hypertension. Fresh frozen plasma takes time to administer, may require repeated doses of significant volume for the pediatric patient, and may ultimately fail. Preliminary data indicated that rFVIIa provides a rapid and successful correction of coagulopathy in the head-injured patient.

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David F. Jimenez, Michael J. McGinity and Constance M. Barone

OBJECTIVE

The objective of this study was to present the authors’ 19-year experience treating metopic craniosynostosis by using an endoscopy-assisted technique and postoperative cranial orthotic therapy. The authors also aimed to provide a comprehensive, comparative statistical analysis of minimally invasive surgery (MIS) versus open surgery in reports previously published in the literature (through 2014) regarding only patients with metopic synostosis.

METHODS

A total of 141 patients with single-suture metopic nonsyndromic craniosynostosis sutures were treated between 1998 and 2017 by endoscopically resecting the synostosed bone followed by postoperative custom cranial orthosis use. All data used in the case series were collected prospectively and stored in a secure database. A comprehensive literature review was performed that included all previous case series reporting common surgical performance measures. A statistical comparison of traditional open methods versus MIS techniques was performed with regard to age, length of hospital stay (LOS), surgical time, estimated blood loss (EBL), and transfusion rate.

RESULTS

The mean age at the time of surgery in the current series was 4.1 months. The mean EBL was 33 ml (range 5–250 ml). One patient underwent an intraoperative blood transfusion and 5 underwent postoperative blood transfusion for a total transfusion rate of 4.3%. The mean operating time was 56 minutes. Ninety-eight percent of patients were discharged on the 1st postoperative day. The median size of the removed synostosed bone was 0.6 cm × 10 cm. The primary goal of achieving correction of the forehead deformity was obtained in 94% of the patients. One hundred eight patients presented with hypotelorism (76.6%). Those with a minimum 1-year follow-up achieved 99% correction (n = 97). Six patients younger than 1 year had not achieved correction at the time of follow-up (6%). There were no intra- or postoperative deaths. One patient had a temporary contact dermatitis to the helmet materials and 2 patients developed pseudomeningoceles, which were successfully treated with a lumbar drain and/or spinal tap. No patient required nor underwent a second surgical procedure. Regarding the previously published literature through 2014, the reported EBL in patients who underwent MIS versus traditional open methods was 54.7 ml versus 224 ml, respectively. The reported average age for patients undergoing MIS versus traditional open methods was 3.8 months versus 11.5 months. The average LOS for patients undergoing MIS versus traditional open methods was 1.7 days versus 3.7 days. The average reported surgical time for those undergoing MIS versus traditional open methods was 66.7 minutes versus 223.7 minutes. The transfusion rate for patients undergoing MIS versus traditional open methods was 22% versus 77%. All of the above differences demonstrated statistical significance.

CONCLUSIONS

The authors’ team has safely and effectively performed 141 metopic craniosynostosis corrections over the past 19 years, with excellent outcomes. Literature review comparing metrics such as LOS, EBL, operating time, and transfusion rate demonstrates a statistically significant improvement in all commonly reported measurements. MIS techniques are safe and effective and should be offered to parents and patients as an option at craniofacial centers treating this condition.

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Viktor Bartanusz, Mateo Ziu, David F. Jimenez and James M. Henry

Calcifying pseudoneoplasm of the spine is a rare nonneoplastic lesion of unknown origin described in adolescents and adults. Its clinical manifestations include axial pain, myelopathy, or radiculopathy. Surgery is the preferred method of treatment. The authors report the occurrence of calcifying pseudoneoplasm at the C1–2 cervical segment in a 22-month-old child who became completely asymptomatic 2 months after open biopsy. A review of the literature is presented, emphasizing the uniqueness of the presented case in comparison with the previously published cases. The 22-month-old healthy girl presented with sudden onset of neck pain. Due to persistence of the symptoms 2 weeks after onset, imaging studies were performed that revealed an inhomogeneous calcified mass extending from the transverse ligament to the C1–2 interlaminar space and facet joint on the left side. Open biopsy of the mass at the C1–2 lamina was performed. The histological features were consistent with calcifying pseudoneoplasm. The child's neck pain progressively improved and she remained asymptomatic at the 1-year follow-up. The postoperative MRI at 8 months did not reveal any progression of the lesion. Contrary to reported cases, calcifying pseudoneoplasm of the spine may occur as early as 2 years of age and should be included in the differential diagnosis of calcified lesions in this age group. Complete resection is not a prerequisite to clinical improvement when there is no compromise of neural structures; conservative management is appropriate.

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James Tait Goodrich