Background: Sturge-Weber syndrome (SWS) is a rare neurocutaneous syndrome characterized by port-wine stain, leptomeningeal angiomatosis, and glaucoma. Due to the involvement of the nervous system, patients are often accompanied with epilepsy. It reported that 75% of patients with SWS did not respond to standard antiepileptic drugs. Although hemispherectomy is effective in treating these patients, the application of it has been limited due to high risk and huge trauma. Recent studies have shown that sirolimus has a positive on complex vascular malformations and seizures, so the authors attempted to treat them by using sirolimus. Methods: The authors retrospectively analyzed 6 patients with SWS who were refractory to antiepileptic drugs and accepted oral sirolimus in their department between 2017 and 2020. Results: All 6 patients were responsive to oral sirolimus treatment. Epilepsy was controlled in all patients, no epilepsy relapsed in 6 patients during the follow-up period. The facial port-wine stain of the patients were all lightened and the hypertrophy of pathological tissue was improved. Only minor adverse reactions occurred during the treatment. Conclusions: Oral sirolimus could control the occurrence of epilepsy and improve the appearance, with minor and tolerable adverse reactions. Sirolimus is especially suitable for patients with severe epilepsy, failure, or contraindications of antiepileptic drugs; it could be an alternative method for patients who are unwilling to accept the risks of neurosurgery.

This procedure not only raises cranial height and extends it upward but also singularizes the occipital carina by prolonging it in a downward direction to form a occipitalia. The separated cranium is not easy to retract after fixation for 3 months so management of unilateral lambdoid synostosis with cranium distraction osteogenesis with a Z-shaped osteotomy is effective.

Background: The cervicofacial lymphatic malformations (LMs) often have poor outcomes due to their microcystic component and diffuse infiltration. Mostly, traditional treatments are inadequate for these refractory cases. Recent researches have shown that sirolimus is effective in the treatment of complicated LMs, however, there is still no standard strategy. Objective: To evaluate the efficacy and safety of intermittent oral sirolimus in treating refractory cervicofacial LMs as a second-line treatment. Methods: Fifteen pediatric patients of refractory cervicofacial LMs were retrospectively analyzed in this study. All the cases had received traditional therapy before, but could not completely control the symptoms and eliminate lesions. As a remedy, sirolimus was then proceeded with an intermittent administration regimen, that is 3 continuous months as a course and started the next course after 1 month interval. The clinical characteristics, imaging data of patients, the changes in the signs and symptoms observed, and associated adverse effects were collected and analyzed. Results: The patients initiated sirolimus therapy at the average age of 2.3 years (range 28 days-8 years 9 months). At the end point of the study, 2 patients remained on sirolimus in continuous courses of treatment. Of 13 patients who withdrawn therapy, 4 had restarted due to recurrence of symptoms and re-expansion of LMs. All patients demonstrated reduction in residual LMs and complete disappearance of symptoms during treatment, and 2 patients with complete resolution on imaging. Toxicity was tolerant in this series. There was no patient develop opportunistic or systemic bacterial infection. Conclusions: Sirolimus is commended as a second-line treatment to treat intractable cervicofacial LMs after failure of traditional therapy. The intermittent administration regimen is efficacious to completely control symptoms and partially reduce residual lesions with good tolerance and limited side effects.

Purpose: One of the most characteristic features in premature craniosynostosis is fronto-orbital retrusion. The standardized surgical technique of fronto-orbital advancement (FOA) can treat this (some) deformity, such as bilateral coronal synostosis. The purpose of the study is to investigate an available method to assess the postoperative outcome of the craniofacial surgery. Methods: From 2010 to 2015, 6 pediatric patients were taken the FOA in the Department of Burn and Plastic Surgery in the Children‘s Hospital of Nanjing Medical University. All the patients were performed the computed tomography (CT) scan preoperatively and postoperatively. The CT databases were processed by DICOM files into MIMICS 16.0 software, which were automatically calculated into orbital volume and orbital roof and base surface area. T -test was used to compare measured values before and after surgery. P < 0.05 was considered statistically significant. Results: The average preoperative orbital volume was 13930.70 mm 3 , and the postoperative was 18578.67917 mm 3 . After operation, the volume of orbital was significantly increased ( P < 0.05). The mean area of the orbital roof surface was 753.989025 mm preoperatively, and the postoperative was 1122.074583 mm. The difference was statistically significant ( P < 0.05). The average area of the orbital base ( S 2) was 334.94 ± 91.76 mm 2 . After the FOA, the orbital base was 356.99 ± 114.21 mm 2 . P ( S 2) = 0.6072 > 0.05, there was no significant statistical difference. Conclusions: Fronto-orbital advancement can successfully improve morphological orbital deformities in children with premature craniosynostosis, but much less for maxillary. The computer-assisted technique can present a measurement of FOA preoperatively and postoperatively, which make the evaluation intuitive.

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