Treatment of Syndromic Craniosynostosis by Anterior and Posterior Vault Distraction Osteogenesis (A-PVDO).

OBJECTIVE: To explore the feasibility and therapeutic effect of anterior and posterior vault distraction osteogenesis (A-PVDO) in the treatment of infantile syndromic craniosynostosis. METHODS: Between January 2017 and December 2019, 7 children with syndromic craniosynostosis underwent osteotomy with Piezo-surgery at our hospital. The first step was to harvest the frontal bone flap and the orbitofrontal bone flap. The second step was to separate the scalp and expose the posterior occipital. Osteotomy was performed on the occipital tubercle. Thereafter, 2 distractors were horizontally installed on the upper edge of the anterior cranial orbit, 2 distractors of 3 cm were installed on the posterior cranial bone. Meanwhile, lambdoidal sutures were fixed by titanium plates. Bone distraction was initiated on postoperative day 5 at the rate of 0.4 to 0.6 mm/day, twice per-day, for a total of 10 to 15 days. After 6 months, the distractors and the titanium plates were removed by secondary surgery. RESULTS: The intracranial volume and posterior cranial morphology were recorded during the follow-up of 6 to 14 months (average = 12 months). The posterior craniums of 7 cases with lambdoidal sutures fixation were completely extended. The anterior cranial morphology was normal. All the cranial deformities were significantly improved. There were no severe complications, such as death, cranial necrosis, and intracranial infection. CONCLUSIONS: A-PVDO is an ideal method for the treatment of severe syndromic craniosynostosis, which can achieve more natural appearance than anterior vault distraction osteogenesis or posterior vault distraction osteogenesis. Moreover, A-PVDO causes no severe complications and is suitable for the treatment of severe syndromic craniosynostosis.

Intermittent Administration Regimen of Sirolimus for Refractory Cervicofacial Lymphatic Malformation.

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.

Sirolimus as a Potential Treatment for Sturge-Weber Syndrome.

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.

Sirolimus for Kaposiform Hemangioendothelioma With Kasabach-Merritt Phenomenon in Two Infants.

Kaposiform hemangioendothelioma is an aggressive vascular tumor with infiltrative growth that commonly occurs in infancy and is associated with a life-threatening consumptive coagulopathy, as well as Kasabach-Merritt phenomenon. Recently, promising results have shown that sirolimus had been successfully used to treat Kasabach-Merritt phenomenon without significant toxicity. However, the situation the authors encountered in treating infants was not so satisfactory. Here, the authors present 2 patients younger than 3 months with refractory Kaposiform hemangioendothelioma treated with sirolimus and experienced severe pneumonia. The outcomes suggest that it is necessary to keep an eye on any symptoms indicate the infection of respiratory tract and use the antibiotics in time. The 2 cases also remind us of the potential sign that indicate the recurrence of KMP, which refers to firmer lesion with deepen color, especially when it comes with complications.

Celecoxib induces adipogenic differentiation of hemangioma-derived mesenchymal stem cells through the PPAR-γ pathway in vitro and in vivo.

Infantile hemangioma (IH) is a benign tumor that produces a permanent scar or a mass of fibro-fatty tissue after involution in 40-80% of cases. Celecoxib is an inhibitor of cyclooxygenase-2 (COX-2), and can inhibit angiogenesis and fibrosis. The present study aimed to clarify whether celecoxib is able to induce tumor regression with minimal side effects. For that purpose, the regulation of celecoxib in the involution of IH was investigated in an IH model. Hemangioma-derived mesenchymal stem cells (Hem-MSCs) were isolated from proliferating specimens, and an IH model was established by injecting these cells into nude mice. Celecoxib was administered in vitro and in vivo. Oil Red O staining and reverse transcription-quantitative-PCR were used to detect the adipogenic differentiation of Hem-MSCs. Histologic analysis and immunohistochemical staining of the tumor xenografts were performed to investigate the pathological evolution of the tumor. The results showed that celecoxib inhibited the proliferation and induced the adipogenic differentiation of Hem-MSCs in vitro. In vivo, adipocytes were only present in the celecoxib group at week 4, while a larger number of fibroblasts and collagenous fibers could be observed in the basic fibroblast growth factor group. Therefore, celecoxib may be a potential agent used for IH treatment by inducing adipogenesis and inhibiting fibroblast formation.

The Role of ICG Angiography in Decision Making About Skin-Sparing in Pediatric Acute Trauma.

Background: Indocyanine green (ICG) angiography has proven useful in assessing skin flap perfusion in plastic and reconstructive surgeries. This research aimed to explore its role in decision making about skin-sparing in children's acute trauma. Methods: A total of 19 patients suffering with acute trauma from January 2019 to September 2021 were retrospectively assessed. Both ICG angiography and clinical judgment were performed to evaluate skin tissue viability. The intraoperative decisions for each case depended on the specific condition of the traumatic wound, including tissue perfusion, skin defect area, and location of the wound. Postoperative vascular imaging software was used to quantify the tissue perfusion, and the duration of postoperative follow-up was from 6 to 18 months. Results: Among them, 18 (94.7%) patients experienced treatments according to ICG angiography and did not develop postoperative necrosis. One case with right forearm trauma suffered from partial necrosis. Hypertrophic scar and local infection were the independent complications, which were managed by symptomatic treatment. Conclusion: ICG angiography may reduce the risk of postoperative necrosis and renders a promising adjunctive technique for surgeons to make reasonable decisions in skin sparing in acute pediatric trauma.