Surgery for Port-Wine Stains: A Systematic Review.

BACKGROUND: Port-wine stains (PWS) are congenital low-flow vascular malformations of the skin. PWS tend to become thicker and darker with time. Laser therapy is the gold standard and the first-line therapy for treating PWS. However, some resistant PWS, or PWS that have tissue hypertrophy, do not respond to this therapy. Our aim is to evaluate the role of surgery in the treatment of PWS birthmarks. METHODS: A literature search was performed in PubMed, Scopus, Web of Science (WOS) and Google Scholar for all papers dealing with surgery for port-wine stains, from January 2010 to December 2020 using the search strings: (capillary vascular malformation OR port-wine stains OR Sturge Weber Syndrome OR sws OR pws) AND (surgical OR surgery). RESULTS: Ten articles were identified and used for analysis. They were almost all case series with a short follow up period and lacked an objective-systematic score of evaluation. CONCLUSIONS: Delay in treatment of port wine stains may result in soft tissue and bone hypertrophy or nodules with disfiguring or destructive characteristics. The correction of PWS-related facial asymmetry often requires bone surgery followed by soft tissue corrections to achieve a more harmonious, predictable result.

Multiple General Anesthesia in Children: A Systematic Review of Its Effect on Neurodevelopment.

The effect of multiple general anesthesia (mGA) procedures administered in early life is a critical theme and has led the Food and Drug Administration (FDA) to issue an alert. This systematic review seeks to explore the potential effects on neurodevelopment of mGA on patients under 4 years. The Medline, Embase and Web of Science databases were searched for publications up to 31 March 2021. The databases were searched for publications regarding "children multiple general anesthesia OR pediatric multiple general anesthesia". Case reports, animal studies and expert opinions were excluded. Systematic reviews were not included, but they were screened to identify any possible additional information. A total of 3156 studies were identified. After removing the duplicates, screening the remaining records and analyzing the systematic reviews' bibliography, 10 studies were considered suitable for inclusion. Comprehensively, a total cohort of 264.759 unexposed children and 11.027 exposed children were assessed for neurodevelopmental outcomes. Only one paper did not find any statistically significant difference between exposed and unexposed children in terms of neurodevelopmental alterations. Controlled studies on mGA administered before 4 years of age support that there might be a greater risk of neurodevelopmental delay in children receiving mGA, warranting the need for careful risk/benefit considerations.

Tongue cancer following hematopoietic cell transplantation for Fanconi anemia.

OBJECTIVES: The aim of this retrospective study was to determine the incidence and the clinical outcome of tongue cancer (TC) in patients affected by Fanconi anemia (FA) who received an allogeneic hematopoietic cell transplantation (HCT). MATERIALS AND METHODS: The patient database from the Bone Marrow Transplant Center of Pescara was reviewed to enroll FA patients. Patients', donors', HCT's, and screening's data were collected as well to look for the incidence and the treatment of TC. RESULTS: Twelve patients affected by FA were identified. Three patients died for transplant-related causes. Five of nine surviving patients were diagnosed with TC at a median of 21.7 years since transplantation and at a median age of 32.10 years. Interestingly, no patient manifested graft-versus-host-disease (GvHD). The 28-year cumulative incidence function of TC was 46.9% (95% CI, 36.9-56.9%). Two patients were treated with chemotherapy alone, two patients were treated with surgery alone, and one with surgery followed by chemotherapy. Overall, 4 patients with TC showed a clinical course characterized by a marked aggressiveness of the tumor disease which led to death due to cancer progression between 2 and 13 months. One patient is surviving 8 months after diagnosis of TC. CONCLUSIONS: Our study confirms the high incidence of tumors and in particular tongue tumors in allotransplanted FA patients. A careful screening has to be life-long maintained. CLINICAL RELEVANCE: Considering the rarity of FA and the frailty of FA patients, this study may add important information for the cancer management of these patients.

Emerging challenges and possible strategies in maxillo-facial and oral surgery during the COVID-19 pandemic.

The Coronavirus disease 2019 (COVID-19) pandemic suddenly took the world by storm and Italy was one of the hardest hit countries. Maxillo-facial surgery and dentistry procedures had to be significantly reorganized, since they are considered high-risk procedures. Protocols had to be changed and interdepartmental cooperation was put in place to plan surgical interventions and maintain high standards. Various improvements have been made to prevent and reduce the risks of spreading the infection. Even if the situation seems to have improved, being unprepared is not an option. In this paper the experience gained during these months has been shared and possible future challenges has been highlighted, suggesting practical adjustments based also on new guidelines and recommendations.

Clinical Management of Acinic Cell Carcinoma of the Lacrimal Gland.

To report a case of acinic cell carcinoma occurred in the lacrimal gland. A 59-year-old man was admitted because of sudden blurring of vision, progressive proptosis of the left eye, and mild double vision in left and down directions of the gaze (Hess-Lancaster test). His medical history detailed controlled bilateral keratoconus and open angle glaucoma. On examination, the best corrected visual acuity decreased from 8/20 till 1/50 in one week. There was a swelling of the left upper eyelid. A hard and tender mass was palpated in the superior temporal left orbit. Ultrasound scan showed an extraconal solid mass, situated in the superior lateral corner of the orbit. Computed tomography and magnetic resonance imaging (MRI) revealed a mass of two centimeters in diameter, with round well-defined outline, within the lacrimal gland. We performed an enucleoresection of the mass, via a coronal approach and a lateral orbitotomy by a piezosurgical device. The lesion appeared nodular, brownish, measuring about 2 × 1.5 cm. Histopathological findings were consistent with acinic cell carcinoma with a microcystic, focally papillary-cystic growth of pattern. Follow-up MRI outcomes led to removal of the residual lacrimal gland for suspicion of recurrence. No tumor recurrences where detected at 7-year follow-up.