Impact of Anterior and Posterior Vault Distraction Osteogenesis (A-PVDO) and 3D-Printed Positioning/Shaping Templates in Apert Syndrome: A Case Series Study.

OBJECTIVE: This study seeks to examine the impact of anterior and posterior vault distraction osteogenesis (A-PVDO) in conjunction with 3D-printed positioning and shaping templates for the management of Apert syndrome. METHODS: From January 2018 to February 2022, a retrospective analysis was conducted on 6 cases of Apert syndrome employing fronto-orbital 3D-printed positioning and molding templates. The cranium underwent surgical modification in accordance with the template's configuration and was affixed with absorbable plates. Subsequently, distraction devices were applied, encompassing both anterior and posterior craniotomies. The evaluation encompassed clinical outcomes, complications (including cerebrospinal fluid leakage and infection), safety, and the feasibility of the distraction osteogenesis procedure. RESULTS: Six patients diagnosed with Apert syndrome underwent treatment involving the integration of fronto-orbital 3D-printed positioning and shaping templates in conjunction with anterior and posterior cranial distraction osteoplasty. Follow-up durations ranged from 18 to 32 months (average: 22 mo). No instances of fronto-orbital retraction, cerebrospinal fluid leakage, or intracranial infection were noted during the follow-up period. The sole reported complication entailed an infection at the extension rod site in 1 case. All patients conveyed satisfaction with the treatment outcomes. CONCLUSIONS: The application of 3D-printed positioning and shaping templates in tandem with anterior and posterior cranial distraction osteogenesis demonstrates efficacy in addressing Apert syndrome. Notably, significant enhancements in head shape and orbit were observed, and the incidence of postoperative complications such as cerebrospinal fluid leakage and infection remained minimal. Moreover, long-term follow-up affirmed stability.

Repair of cervicothoracic skin defects with extra-long transverse cervical flaps by stepwise pressure packing in children: a technical innovation.

Objective: To investigate the clinical effect of prolonging predilated transverse cervical flap with stepwise pressure packing for neck and chest lesions in children. Methods: A retrospective review of children with large cervicothoracic lesions admitted to our department from January 2011 to June 2021 was conducted to compare stepwise pressure packing with normal dressing in the surgical method of transverse cervical pedicled flaps after expansion. Among 58 included children, 22 (14 males and 8 females) were allocated to the extended and expanded transverse cervical flap with stepwise compression dressing group, and 36 (19 males and 17 females) to the transverse cervical flap group. The causes of skin defects were: scars (37 cases) and giant nevus (21 cases). The course of the disease ranged from 0.5 to 8 years. The two groups were compared in terms of child satisfaction, the occurrence of infection, recurrence of the contracture, secondary operation, and repaired area. Results: In 22 cases of extended transverse cervical flaps, 8 cases were embedded with two expanders, resulting in a total of 30 expanded flaps, which were successfully transferred to the neck and chest without necrosis at the distal end of compression, with good effect. Comparison of pedicled transverse cervical flaps with stepwise pressure packing and pedicled transverse cervical flaps alone revealed no significant difference in child satisfaction, the occurrence of infection, recurrence of the contracture, and secondary surgery (all P > 0.05). Yet, there was a significant difference in the repair area between two groups (P < 0.05). Conclusion: Prolongation of pedicled cervical flaps after expansion with stepwise pressure packing resulted in an effective method for repairing the large skin defect of children's face and neck caused by various diseases. In terms of increasing neck repair area, the operation with stepwise pressure dressing was significantly superior to the simple packing.

Cellular senescence contributes to mechanical ventilation-induced diaphragm dysfunction by upregulating p53 signalling pathways.

BACKGROUND: Mechanical ventilation can cause acute atrophy and injury in the diaphragm, which are related to adverse clinical results. However, the underlying mechanisms of ventilation-induced diaphragm dysfunction (VIDD) have not been well elucidated. The current study aimed to explore the role of cellular senescence in VIDD. METHODS: A total of twelve New Zealand rabbits were randomly divided into 2 groups: (1) spontaneously breathing anaesthetized animals (the CON group) and (2) mechanically ventilated animals (for 48 h) in V-ACV mode (the MV group). Respiratory parameters were collected during ventilation. Diaphragm were collected for further analyses. RESULTS: Compared to those in the CON group, the percentage and density of sarcomere disruption in the MV group were much higher (p < 0.001, both). The mRNA expression of MAFbx and MuRF1 was upregulated in the MV group (p = 0.003 and p = 0.006, respectively). Compared to that in the CON group, the expression of MAFbx and MuRF1 detected by western blotting was also upregulated (p = 0.02 and p = 0.03, respectively). Moreover, RNA-seq showed that genes associated with senescence were remarkably enriched in the MV group. The mRNA expression of related genes was further verified by q-PCR (Pai1: p = 0.009; MMP9: p = 0.008). Transverse cross-sections of diaphragm myofibrils in the MV group showed more intensive positive staining of SA-ßGal than those in the CON group. p53-p21 axis signalling was elevated in the MV group. The mRNA expression of p53 and p21 was significantly upregulated (p = 0.02 and p = 0.05, respectively). The western blot results also showed upregulation of p53 and p21 protein expression (p = 0.03 and p = 0.05, respectively). Moreover, the p21-positive staining in immunofluorescence and immunohistochemistry in the MV group was much more intense than that in the CON group (p < 0.001, both). CONCLUSIONS: In a rabbit model, we demonstrated that mechanical ventilation in A/C mode for 48 h can still significantly induce ultrastructural damage and atrophy of the diaphragm. Moreover, p53-dependent senescence might play a role in mechanical ventilation-induced dysfunction. These findings might provide novel therapeutic targets for VIDD.

Closure of Giant Dorsal Lumbosacral Myelomeningocele in Children Using Double Expanded Flaps: A Case Report and Literature Review.

INTRODUCTION: Myelomeningocele (MMC) is the most common neural tube defect that can occur due to neural tube's failure to fuse properly during embryonic life. To prevent this, expanded flaps can be used for closure of large MMCs. PRESENTATION OF CASE: A 4-year and 6-month girl was diagnosed with multiple congenital anomalies including hydrocephalus, dorsal lumbosacral MMC, and congenital tethered cord syndrome. Preoperative evaluation showed no lower limb movements and the size of the sacrococcygeal region was about 10 cm × 8 cm × 15 cm mass, prominent thoracic kyphosis, and no obvious urogenital or limb anomalies. The large dorsal lumbosacral MMC was treated with a double expanded flap to reconstruct, the soft tissue defect following the neurosurgical reconstruction. DISCUSSION: The expanded flap was deemed as viable as all wounds were healed without any complications, such as dehiscence, leakage of cerebrospinal fluid, or infection. The technique described in the case report offers an effective method of closure. CONCLUSION: This flap can be an effective method for reconstruction of large dorsal lumbosacral MMC defects that might improve outcomes and minimize complications. It also ensures minimal wound tension and breakdown.

Optimizing surgical management of iatrogenic bile duct injury: transhepatic percutaneous cholangial drainage combined with end-to-end biliary anastomosis.

Iatrogenic bile duct injury remains the most severe complication of gallbladder surgeries. To reduce post-operation complication, we introduce an improved approach for bile duct injury repairment, named transhepatic percutaneous cholangial drainage (TPCD) which combined with end-to-end biliary anastomosis. Clinical data obtained from 12 patients between February 2012 and May 2022 were retrospectively analyzed. Patient demographic, clinical, operative, and follow-up data were analyzed using descriptive statistics. All injuries were repaired successfully and no fatal cases occurred. The mean operative time and hospital stay duration were 367.5 ± 103.2 min and 11.3 ± 3.5 days, respectively. In two cases (16.7%), bile leakage occurred at the bile duct anastomosis site. Three patients (25.0%) developed low-grade fever and one patient (8.3%) developed a postoperative infection of the incision site. No postoperative bleeding or bile duct strictures occurred in any of the cases. The patients were followed up from 12 to 122 months (median, 70.5 months). No cholangitis or bile duct restenosis was observed after biliary drainage tube removal. There were no long-term bile duct-related complications seen in the follow-up time. It is safe and feasible for TPCD combined with end-to-end biliary anastomosis using in bile duct injury.

Accuracy and safety of robotic navigation-assisted distraction osteogenesis for hemifacial microsomia.

Introduction: This study aimed to verify the accuracy and safety of distraction osteogenesis for hemifacial microsomia assisted by a robotic navigation system based on artificial intelligence. Methods: The small sample early-phase single-arm clinical study, available at http://www.chictr.org.cn/index.aspx, included children aged three years and older diagnosed with unilateral hemifacial microsomia (Pruzansky-Kaban type II). A preoperative design was performed, and an intelligent robotic navigation system assisted in the intraoperative osteotomy. The primary outcome was the accuracy of distraction osteogenesis, including the positional and angular errors of the osteotomy plane and the distractor, by comparing the preoperative design plan with the actual images one week postoperatively. Perioperative indicators, pain scales, satisfaction scales, and complications at one week were also analyzed. Results: Four cases (mean 6.5 years, 3 type IIa and 1 type IIb deformity) were included. According to the craniofacial images one week after surgery, the osteotomy plane positional error was 1.77 ± 0.12 mm, and the angular error was 8.94 ± 4.13°. The positional error of the distractor was 3.67 ± 0.23 mm, and the angular error was 8.13 ± 2.73°. Postoperative patient satisfaction was high, and no adverse events occurred. Discussion: The robotic navigation-assisted distraction osteogenesis in hemifacial microsomia is safe, and the operational precision meets clinical requirements. Its clinical application potential is to be further explored and validated.

Occurrence of Rhabdomyosarcoma After Surgery Combined with oral Sirolimus for Mixed Vascular Malformation of the Tongue.

Vascular malformation is the general term of a kind of lesions originated from lymphatic vessels and vascular tissues, which contains a variety of components called mixed vascular malformation. Rhabdomyosarcoma (RMS) is a kind of soft tissue sarcoma, originating from striated muscle cells or mesenchymal cells. RMS and vascular malformation mostly occur in children, and common in the head and neck, but their simultaneous occurrence is rare. A 9-year-old boy who was hospitalized for a second attack of combined vascular malformation: hemolymphangioma. The child experienced severe upper airway obstruction and tongue bleeding. Postoperative pathology demonstrated hemolymphangioma combined with RMS. Subsequently, he was transferred to the oncology department for chemotherapy and lately died of RMS with lung metastasis. The secondary RMS may be related to the usage of sirolimus. Because of its uncertain border, vascular malformation in the oral and maxillofacial region is difficult to completely remove by surgical resection, and local recurrence could be often observed. Due to its rapid progress and continuous bleeding, the possibility of malignant tumor should be considered and multidisciplinary comprehensive treatment should be actively taken. Besides, family history of related malignant tumors and immune function should be investigated in detail before choosing the application of oral sirolimus.

Posterior Cranial Retraction Combined With Bilateral Parietal Distraction for Children With Nonsyndromic Craniosynostosis.

BACKGROUND: The nonsyndromic craniosynostosis is the most common of craniosynostoses in childhood. There are many treatments. We aim to treat 12 cases of nonsyndromic craniosynostosis via posterior cranial vault distraction osteogenesis combined with bilateral parietal distraction. METHODS: Data of a total of 12 patients (7 boys and 5 girls) with nonsyndromic sagittal synostosis who underwent distraction osteogenesis between January 2015 and August 2020 were retrospectively analyzed. Bilateral parietal bone flaps and posterior occipital flaps were designed and cut. Then, distraction device was placed, which was distracted at 5 days after surgery (twice per day, 0.4-0.6 mm/d, and lasting for 10-15 days). After 6 months of fixation, the secondary surgery was performed to remove the device. RESULTS: The scaphocephaly was corrected, and the appearance was satisfactory. Postoperative follow-up time was 6 to 14 months, with an average of 10 months, and the mean CI was 63.2 and 78.25 before and after surgery, respectively; the mean anterior-posterior skull diameter was shortened (12.63 ± 3.47) mm, the transverse diameter of both temporal regions was lengthened (15.4 ± 4.18) mm, and the scaphocephalic deformity was significantly improved. There was no detachment or rupture of the extender postoperatively. No severe complications, such as radiation necrosis or intracranial infection, were observed. CONCLUSION: Posterior cranial retraction combined with bilateral parietal distraction in children with nonsyndromic craniosynostosis, in which the proposed technique did not exhibit severe complications, and it is worthy of further promotion and application in clinical practice.

KNTC1 as a putative tumor oncogene in pancreatic cancer.

PURPOSE: Recent studies have demonstrated that kinetochore-associated protein 1 (KNTC1) plays a significant role in the carcinogenesis of numerous types of cancer. This study aimed to explore the role and possible mechanisms of KNTC1 in the development of pancreatic cancer. METHODS AND RESULTS: We analyzed differentially expressed genes by RNA sequencing in three paired pancreatic cancer and para-cancerous tissue samples and found that the expression of KNTC1 was significantly upregulated in pancreatic cancer. A Cancer and Tumor Gene Map pan-analysis showed that high expression of KNTC1 was related to poor prognosis in 9499 tumor samples. With immunohistochemical staining, we found that the high expression of KNTC1 in pancreatic cancer was related to pathological grade and clinical prognosis. Similarly, RT-PCR results indicated that the expression of KNTC1 was higher in three groups of pancreatic cancer cell lines (BxPC-3, PANC-1, and SW1990) than in normal pancreatic ductal cells. We introduced lentivirus-mediated shRNA targeting KNTC1 into PANC-1 and SW1990 cells and found that KNTC1 knockdown significantly decreased cell growth and increased cell apoptosis compared to the control group cells. Bioinformatic analysis of the cell expression profile revealed that differential genes were mainly enriched in the cell cycle, mitosis, and STAT3 signaling pathways, and co-immunoprecipitation confirmed an interaction between KNTC1 and cell division cycle associated 8. CONCLUSIONS: KNTC1 could be linked to the pathophysiology of pancreatic cancer and may be an early diagnostic marker of cervical precancerous lesions.

[Corrigendum] HIF-1α contributes to tube malformation of human lymphatic endothelial cells by upregulating VEGFR-3.

Subsequently to the publication of the above article, the authors have realized that Fig. 4 on p. 145 (showing the results from the colony formation assay) was published containing erroneous images in Fig. 4C. Essentially, incorrect images were selected to represent the negative control (NC) experiments owing to an altered shooting angle and incorrect naming of the files. The corrected version of Fig. 4, now showing the correct data for the NC experiments in Fig. 4C, is shown on the next page. The authors confirm that the error made in the presentation of Fig. 4 did not adversely affect either the results or the conclusions reported in this paper, and they are grateful to the Editor of International Journal of Oncology for granting them this opportunity to publish a Corrigendum. They also apologise to the readership for any inconvenience caused. [International Journal of Oncology 54: 139-151, 2019; DOI: 10.3892/ijo.2018.4623].

Circ-FOXM1 promotes the proliferation, migration and EMT process of osteosarcoma cells through FOXM1-mediated Wnt pathway activation.

BACKGROUND: Osteosarcoma (OS) is a malignant bone tumor that commonly occurs in adolescents with a high mortality rate and frequent pulmonary metastasis. Emerging evidence has suggested that circular RNAs (circRNAs) are important regulators in multiple biological activities of carcinomas. Nevertheless, the role of circRNAs derived from forkhead box M1 (FOXM1), a well-accepted modulator of OS progression, has not been discussed in OS. METHODS: Quantitative real-time polymerase chain reaction (qRT-PCR) was utilized to test circ-FOXM1 (hsa_circ_0025033) expression in OS cell lines. Cell counting kit-8 (CCK-8), 5-ethynyl-2'-deoxyuridine (EdU), terminal deoxynucleotidyl transferase dUTP nick end labeling (TUNEL), transwell assays and western blot analysis of epithelial-mesenchymal transition (EMT) markers were conducted to evaluate cell proliferation, apoptosis, migration, and EMT process. Luciferase reporter assay and RNA-binding protein immunoprecipitation (RIP) assay were utilized to detect the interaction of circ-FOXM1 and RNAs. RESULTS: High expression of circ-FOXM1 was detected in OS cell lines. Functionally, circ-FOXM1 knockdown inhibited the proliferation, migration and EMT process, whereas induced the apoptosis of OS cells. From the aspect of molecular mechanism, circ-FOXM1 was discovered to upregulate FOXM1 expression via sponging miR-320a and miR-320b, therefore activating Wnt signaling pathway. Besides, rescue experiments elucidated that circ-FOXM1 regulated cellular activities of OS cells via FOXM1. Further, in vivo assays supported that loss of circ-FOXM1 restrained OS tumor growth. CONCLUSION: Circ-FOXM1 facilitated the malignant phenotypes of OS cells through FOXM1-mediated Wnt pathway activation, revealing circ-FOXM1 as a potential biomarker for OS treatment.

Treated Pierre Robin Sequence Using Placed Allogenic Acellular Bone Matrix and Mandibular Distraction Osteogenesis in the Neonate.

Objective: The aim of the study was to report our experience with placed allogenic acellular bone matrix and mandibular distraction osteogenesis in Pierre Robin sequence (PRS), and explore the role of distraction in the osteogenesis of acellular bone. Materials and Methods: A total of 428 neonates with severe PRS managed with placing allogenic acellular bone and bilateral mandibular distraction osteogenesis were included in the study. The procedure included using oblique-shaped osteotomy, fixing bilateral mandibular distractor, instantly extending a 4-6 mm gap, and placing allogenic acellular bone into the gap. The length of allogenic acellular bone was 4-5 mm. Although the surgical techniques, distraction, and consolidation periods were similar, the allogenic acellular bone matrix we placed was quite different from the traditional distraction. With the technology we used, tracheal intubation could be immediately removed, thus quickly improving breathing conditions compared to traditional methods after the surgery. The jaw extending and oral feeding could begin on the 5th day. The jaw was extended 0.6 mm twice a day until the mandible was overcorrected by 20%. Results: All 428 cases included in this study were successfully extubated after the operation, and the difficulty in breathing was instantly relieved. Total mandibular distraction was 15-20 mm. Oral feeding was started at 6 h to 6 days postoperatively, while hospital stay ranged from 18 to 20 days postoperatively. No major complications were reported. Medium to long-term results was good. Mandibular distractors were removed after 3 months. Conclusions: Bilateral mandibular distraction osteogenesis combined with placing allogenic acellular bone in the neonate are safe and accurate procedures, which are the primary treatment options for cases of severe PRS. It can be considered that the tension of distraction can promote osteogenesis in acellular bone and thus improve distractive effect of the mandible.

Clinical Effectiveness of Laparoscopic Fiberoptic Choledochoscopy versus Conventional Open Surgery for Gallbladder Stones Complicated with Common Bile Duct Stones.

Objective: To assess the clinical effectiveness of laparoscopic fiberoptic choledochoscopy versus conventional open surgery for gallbladder stones complicated with common bile duct stones. Methods: In this retrospective study, 110 patients with gallbladder stones complicated with common bile duct stones treated in our institution between May 2018 and April 2020 were recruited and assigned to receive either open surgery (conventional group) or laparoscopic fiberoptic choledochoscopy (experimental group). Outcome measures included intraoperative indices, postoperative indices, postoperative complications, and changes in postoperative blood indices. Results: Laparoscopic fiberoptic choledochoscopy was associated with less intraoperative bleeding volume and a shorter surgical incision length versus open surgery (P < 0.05). All eligible patients showed similar operative time (P > 0.05). Laparoscopic fiberoptic choledochoscopy resulted in shorter postoperative exhaust time and mean length of stay and a higher mean hospitalization cost versus open surgery (P < 0.05). There was no significant difference in the number of patients with intensive care units (ICU) monitoring or primary suture of the common bile duct between the two groups (P > 0.05). The eligible patients after laparoscopic fiberoptic choledochoscopy experienced fewer complications versus those after open surgery (P < 0.05). Laparoscopic fiberoptic choledochoscopy had a milder impact on postoperative albumin decrease versus open surgery (P < 0.05). No significant difference was found in the postoperative leukocyte changes and total bilirubin decrease between the two groups (P > 0.05). Conclusion: Laparoscopic fiberoptic choledochoscopy has better perioperative indices outcomes, lower incidence of postoperative complications, smaller postoperative albumin changes, and superior overall performance versus conventional open surgery for gallbladder stones complicated with common bile duct stones.

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.

Selective radical resection for unresectable pancreatic cancer.

OBJECTIVE: The objective of the study was to evaluate safety and value of radical resection for unresectable pancreatic cancer (UPC). MATERIALS AND METHODS: Clinical data were analyzed retrospectively. In unresectable group, 360° resection of the involved artery sheath, resection and reconstruction of the involved artery, resection and reconstruction of the involved vein as well as resection and reconstruction of combined organs were, respectively, performed. Operation time, intraoperative blood loss, intensive care unit (ICU) transitional treatment, pancreatic fistula, bleeding, reoperation, and survival time were analyzed for two groups. RESULTS: Operation time and intraoperative blood loss were greatly increased in the unresectable group. The incidence of intractable diarrhea and abdominal hemorrhage in the unresectable group was higher. However, the rate of ICU transitional therapy, delayed gastric emptying, and reoperation was lower. Grade C pancreatic fistula occurred in neither group. CONCLUSIONS: Surgical treatment through strict selection for patient with UPC is safe and their median survival time is similar to patient with resectable pancreatic cancer.

OBJETIVO: evaluar la seguridad y el valor de la resección radical para el cáncer de páncreas irresecable (CPU). MATERIAL Y MÉTODOS: Los datos clínicos se analizaron de forma retrospectiva. En el grupo irresecable, se realizó resección de 360° de la vaina de la arteria afectada, resección y reconstrucción de la arteria afectada, resección y reconstrucción de la vena afectada, así como resección y reconstrucción de órganos combinados, respectivamente. Se analizaron el tiempo operatorio, la pérdida de sangre intraoperatoria, el tratamiento transitorio en la UCI, la fístula pancreática, el sangrado, la reintervención y el tiempo de supervivencia para dos grupos. RESULTADOS: El tiempo de operación y la pérdida de sangre intraoperatoria aumentaron considerablemente en el grupo irresecable. La incidencia de diarrea intratable y hemorragia abdominal en el grupo irresecable fue mayor. Sin embargo, la tasa de terapia de transición en la UCI, el retraso del vaciamiento gástrico y la reintervención fueron menores. La fístula pancreática de grado C ocurrió en ninguno de los grupos. CONCLUSIONES: el tratamiento quirúrgico mediante selección estricta del paciente con CP irresecable es seguro y su mediana de supervivencia es similar a la del paciente con CPR.

Management of Macrocystic Lymphatic Malformation in the Cervicofacial Region: Ultrasound-Guided Iodine Tincture Cauterization Combined With Intralesional Negative Pressure.

PURPOSE: The purpose of our study was to evaluate the efficacy and safety of ultrasound-guided iodine tincture cauterization combined with postoperative intralesional negative pressure in the management of cervicofacial cystic lymphatic malformation (cLM). METHOD: From January 2019 to July 2021, indocyanine green lymphography was performed preoperatively to confirm the lymph inflow, and this treatment was administered in 71 patients with cervicofacial cLM in our center. All cases were evaluated by curative effects, treatment frequency, and adverse events. The duration of posttreatment follow-up was from 12 to 14 months. RESULTS: Indocyanine green lymphography indicated at least one lymphatic inflow in each cLM lesion. Excellent resolution was observed in 87.3% of cases, and good improvement of the treated cLM occurred in 9.9% of cases, and 2 cases with fair outcomes required subsequent treatment. It is noteworthy that no case was treated more than 3 times. Some minor adverse effects, including localized itch and scar, were managed by symptomatic treatment. CONCLUSIONS: Because of satisfactory outcomes and low treatment frequency, ultrasound-guided iodine tincture cauterization combined with intralesional negative pressure represents an efficacious, safe, and feasible method for the management of macro-cLM in the cervicofacial region.

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.