Algorithm to the treatment of Crouzon syndrome.

Clinical reports regarding the entire surgical sequence in Crouzon syndrome from the neonatal period to the adult age are rare. The purpose of this study is to trace an operative algorithm with a long term follow up in a homogenous group of patients affected by Crouzon syndrome. A retrospective review was conducted for all patients affected by Crouzon syndrome who completed the entire surgical sequence. 7 Crouzon patients (4 females, 3 males) completed the entire surgical sequence at different ages: fronto-orbital advancement (0.9 years), LF III distraction osteogenesis (11.5 years) and orthognathic surgery (18 years). The mean age at the last follow up was 19.3 years; normalization of the face was obtained in all cases with improvement of the respiratory problems. After orthognathic surgery, all patients had stable occlusion. A one-year postoperative CBCT scan revealed almost complete ossification of all osteotomy sites. Frontoorbital advancement and modified Le Fort III distraction osteogenesis are reliable surgical procedures. SARME and conventional orthognathic surgery with reductive genioplasty and fat grafting are performed at the end of the surgical sequence to enhance facial aesthetics.

Management of orbital and brain complications of sinusitis: A practical algorithm.

The aim of this work was to present a practical management algorithm for orbital and brain complications of sinusitis. According to the inclusion criteria, a sample of 68 patients was collected between 2008 and 2018 (39 males and 29 females). Among them, 44 were adults, with a mean age of 50.46 years, and 24 were pediatric patients, with a mean age of 10.33 years. Oral or intravenous antibiotic therapy was administered to all patients. Pharmacological resolution was observed in 14 cases. Early surgical treatment within 48 hours was necessary in 10 cases. Surgery consisted of abscess drainage, associated or not with functional endoscopic sinus surgery. Delayed surgery within 15-30 days was performed in 44 patients. The core procedure was functional endoscopic sinus surgery. Subsidiary procedures were abscess drainage, tooth extraction or cranial base repair. The combination of two or more of these procedures was case selected. Median follow-up was of 46.36 months. Sinusitis complications necessitate rapid diagnosis and prompt treatment. Antibiotic therapy alone is enough for mistreated rhinosinusitis with no anatomical predisposing factor. Surgery is mandatory for altered nasal and paranasal sinus anatomy or odontogenic infections.

Respiratory and volumetric changes of the upper airways in craniofacial synostosis patients.

To assess postoperative changes of the upper airway in a pediatric syndromic cranial-synostosis (SCS) population who underwent Le Fort III (LFIII) or frontofacial advancement by distraction osteogenesis (DO). Charts' review of 25 SCS infants presented at our tertiary-care children's hospital between January 2005 and December 2016 was performed. Preoperative (T0) and postoperative (T1) three-dimensional computed tomography (3D-CT) and polysomnography (PSG) were recorded. Differences between T0 and T1 airway volumes and changes in PSG data were analyzed. 18 patients were included. The mean T0 and T1 volumes were calculated as 15.963 mm3 ± 7.181 SD and 24.550 mm3 ± 12.946 SD, respectively. Airway areas increased significantly (p < 0.05) in the total study-group by a median value of 8.004 mm3, together with a global 72.22% improvement in respiratory parameters. A statistically significant gain of the upper airway after LF III and DO in SCS patients has been demonstrated. Given the absence of a direct relationship between post-operative upper airway volume increase and OSAS degree improvement, further insights should consider performing T0 and T1 sleep endoscopy analysis to complete the diagnostic workup and to better assess the level of residual or recurrent upper airway obstruction in patients who experience unsuccessful surgical treatment.

Intracavitary chemotherapy (Gliadel) and oral low-dose etoposide for recurrent anaplastic ependymoma.

Anaplastic ependymoma is associated with a higher incidence of tumor recurrence and its prognosis still remains unsatisfactory. Consolidated therapy for ependymoma includes surgery followed by focal radiotherapy when resection is incomplete. In the case of relapse treatment, options are limited especially for patients who have already received radiotherapy. We sought to establish the feasibility of administering low-dose oral etoposide (50 mg/m(2)/day for 21 days) in combination with the implantation of intracavitary carmustine (BCNU) wafers (Gliadel) at the gross total resection for achieving synergistic treatment in three children affected by recurrent anaplastic ependymoma. All patients had Karnofsky performance scale (KPS) scores >80%. The therapy was tolerated safely and well in all patients without any post-surgery complications. After BCNU wafer implantation, all patients achieved radiological and clinical stabilization for an average period of 3 months. Two patients relapsed after 4 months as shown in brain MRIs. The other patient went to progression two months after the Gliadel implantation. This multimodal approach was not effective for the treatment of refractory anaplastic ependymoma and further studies are required in order to define the role of the combination of multidrug systemic chemotherapy with BCNU wafer implantation in children with high-risk brain tumors.

Transvenous embolization: a report of 4 pediatric cases.

The treatment of brain arteriovenous malformations (AVMs) in children has always been a challenge for interventionalists, neurosurgeons, and radiosurgeons. Endovascular embolization is usually performed through transarterial access, but in selected cases the transvenous approach can be considered. The authors of this report aimed to evaluate the efficacy of transvenous embolization in very selected pediatric cases. They describe 4 children treated using transvenous embolization for AVMs that were small, had a single drainage vein, and were deeply located or had a difficult arterial access. The 6-month angiographic and clinical follow-ups are reported as well. In all cases, complete occlusion of the AVM was achieved with no side effects for the patient. Transvenous embolization may represent a promising alternative therapeutic option in very selected cases.

Complex craniofacial advancement in paediatric patients: Piezoelectric and traditional technique evaluation.

PURPOSE: The piezoelectric device allows bone cutting without damaging the surrounding soft tissues. The purpose of this study was to assess the role of this surgical instrument in paediatric craniofacial surgery in terms of safety and surgical outcomes. METHODS: Thirteen consecutive paediatric patients underwent craniofacial Le Fort osteotomies type III and IV. The saw was used on the right side in seven patients and on the left side in six patients; the piezoelectric instrument was used on the right side in six patients and on the left side in seven patients. Intraoperative blood loss, surgical procedure length, incision precision, postoperative haematoma and swelling, and nerve impairment were evaluated to compare the outcomes of both procedures. RESULTS: A longer surgical procedure was observed in 28% of the patients when using the piezoelectric device (p = 0.032), with an intraoperative blood loss reduction of 18% (p = 0.156). Greater precision in bone cutting was reported, together with a reduction in the requirement to protect and incise adjacent soft tissues during piezoelectric osteotomies. There was a lower incidence of postoperative haematoma and swelling following piezo-osteotomy, and a significant reduction in postoperative nerve impairment (p = 0.002). CONCLUSIONS: The ultrasonic surgical device guaranteed a clean bone cut, preserving the integrity of the adjacent soft tissues beneath the bone. Although the time required for a piezoelectric osteotomy was longer, the total operation time remained approximately the same. In conclusion, the device's lack of power appears to be a minor problem compared with the advantages, and an ultrasonic device could be considered a valuable instrument for paediatric craniofacial advancement.

Maximizing results in craniofacial surgery with bioresorbable fixation devices.

The resorbable plating system allows the infant's skull to grow once the system is resorbed, thus not inhibiting the necessary developmental growth seen with the titanium system. Despite marked improvements in long-term outcomes, there are still technical points that can be followed to maximize outcome while reducing and possibly eliminating minor complications such as plate palpability and visibility through the skin as well as skin breakdown over the plate. A retrospective electronic chart review was performed on the pediatric patient population who underwent craniofacial surgery with the use of resorbable fixation devices by the senior author (LG). Fifty-two patients underwent surgical correction for craniosynostosis with resorbable material (Craniosorb, Lactosorb, or Biosorb PDX). This series included patients with brachycephaly (17), anterior plagiocephaly (unilateral coronal synostosis; 16), trigonocephaly (11), multisuture craniosynostosis (7), and Cohen's craniotelencephalic dysplasia (1). The mean age at the time of the operation was 8 months and the mean follow up was 17 months. Eight patients experienced complications related to the resorbable material. Seven of the eight had complete resolution of symptoms after conservative treatment and one patient had complete resolution of the skin infection after plate removal. The purpose of this study was to evaluate the risks and complications with the use of resorbable material to establish guidelines for avoidance of surgical pitfalls that lead to increased risk of morbidity with the use of this material, particularly as it relates to plate visibility under the skin, plate palpability, skin breakdown, and skin infections over the plating system.