Sirolimus treatment for paediatric head and neck lymphatic malformations: a systematic review.

PURPOSE: This PRISMA-compliant systematic review aimed to assess risks and benefits of sirolimus treatment for paediatric lymphatic malformations by focusing not only on treatment efficacy but also on possible treatment-related adverse events, and treatment combinations with other techniques. METHODS: Search criteria were applied to MEDLINE, Embase, Web of Science, Scopus, Cochrane Library, and ClinicalTrials.gov databases and included all studies published up to March 2022 reporting paediatric lymphatic malformations treated with sirolimus. We selected all original studies that included treatment outcomes. After the removal of duplicates, selection of abstracts and full-text articles, and quality assessment, we reviewed eligible articles for patient demographics, lymphatic malformation type, size or stage, site, clinical response rates, sirolimus administration route and dose, related adverse events, follow-up time, and concurrent treatments. RESULTS: Among 153 unique citations, 19 studies were considered eligible, with reported treatment data for 97 paediatric patients. Most studies (n = 9) were case reports. Clinical response was described for 89 patients, in whom 94 mild-to-moderate adverse events were reported. The most frequently administered treatment regimen was oral sirolimus 0.8 mg/m2 twice a day, with the aim of achieving a blood concentration of 10-15 ng/mL. CONCLUSION: Despite promising results for sirolimus treatment in lymphatic malformation, the efficacy and safety profile of remains unclear due to the lack of high-quality studies. Systematic reporting of known side effects, especially in younger children, should assist clinicians in minimising treatment-associated risks. At the same time, we advocate for prospective multicentre studies with minimum reporting standards to facilitate improved candidate selection.

Basal Cell Carcinoma Arising over a Venous Malformation of the Forehead Communicating with the Endocranial Veins.

INTRODUCTION: An increasing number of skin cancer arising over vascular anomaly has been reported in literature. In such cases, the oncologic radicality required to threat skin malignancies may be in contrast with the safety needed when dealing with vascular malformation. As a result, treatment of this association may be insidious and treacherous and imposes a sound knowledge and carefulness. MATERIALS AND METHODS: The authors report on a case of a 77-years-old woman affected by a basal cell carcinoma (BCC) arising over a vascular malformation of forehead. Preoperative radiological imaging revealed an underlying venous malformation (VM) communicating with intracranial district. Patient underwent sclerotherapy of the VM with gelified ethanol in order to reduce potentially fatal bleeding during surgery and, on the other hand, any leakage of the sclerosant in the intracranial veins. Excision of the BCC was then performed without complications. RESULTS: Neither intra-operative nor post-operative complications were observed. Current 3-years follow-up shows no recurrence of BCC whilst the residual VM is stable and clinically silent. CONCLUSIONS: Mechanisms leading to the onset of skin cancers over venous malformations are still unclear. However, association between these 2 conditions may be underestimated with possible catastrophic consequences. Thorough knowledge of vascular malformations and a multidisciplinary approach is of the uttermost importance when dealing with such clinical challenges.

SECg Staging System: A New Approach to the Management of Arteriovenous Malformations of the Head and Neck.

OBJECTIVES: Arteriovenous malformations (AVM) are the most troublesome vascular malformations to deal with. They tend to behave like low-grade malignancies with infiltrative and disruptive growth. Crucially, the clinical course of an AVM that has been improperly managed is usually characterized by a recurrence that is much more aggressive than the original disease. As in oncology, a comprehensive staging system is highly desirable and is to date lacking in the literature. The authors present a new comprehensive staging system. METHODS: A multicentric multidisciplinary team of experts in the field of vascular anomalies has created this new staging system. The SECg staging system defines the local extension of the disease (S1-S4), the vascular architecture of the malformation (E1, E2, E3), the severity of the symptoms (C0-C3) and the presence or absence of growth of the AVM (g+, g-). RESULTS: This staging system allows to address all the aspects of AVMs and, more importantly, to help building an appropriate, individualized treatment plan for affected patients. After being staged an AVM can be defined as (a) healable, (b) healable with predicted sequelae, or (c) unhealable. Then, the SECg system allows to outline (a) absolute indications, (b) relative indications, and (c) no indications for treatment. The purpose of the treatment (radical, palliative) is furthermore taken into consideration. CONCLUSIONS: This multicentric, the SECg staging system that this multidisciplinary group of Authors has defined allows for a comprehensive staging of the disease which in turn has enabled to outline an algorithm to properly manage AVMs.

Objective Assessment of Lingual Nerve Microsurgical Reconstruction.

Lingual nerve (LN) injury is one of the most serious consequences of oral surgery. Prompt microsurgical reconstruction of the nerve can alleviate most of those symptoms leading to satisfactory functional recovery.Thirty-five patients with partial to complete LN injury underwent surgery in the period between January 2006 and May 2015. All patients underwent a preoperative clinical and neurological evaluation with the assessment of lingual tactile and pain sensory thresholds and masseteric inhibitory reflex.All patients underwent explorative surgery and direct microneurorrhaphy of distal and proximal stumps in case of complete lesion, while the removal of traumatic neuroma and the following microneurorrhaphy of distal and proximal stumps of the injured nerve was performed in case of incomplete lesion. Nerve grafting has always been avoided because of distal stump mobilization obtained by severing the submandibular branch of the LN.All patients but 1 exhibited good recovery of tongue sensation, never complete, both clinically and electrophysiologically: recovery of the excitability of masseteric inhibitory reflex suppression components SP1 and SP2 was observed, often with increased latencies but consistent with a functional recovery.All patients feeling pain preoperatively experienced complete relief of algic symptoms.The early microsurgical approach is the most suitable choice for the treatment of LN injuries.

Complications After Treatment of Head and Neck Venous Malformations With Sodium Tetradecyl Sulfate Foam.

PURPOSE: The aim of this study was to evaluate complications in patients with head and neck venous malformations (VMs) treated with foam sclerotherapy using sodium tetradecyl sulfate (STS). METHODS: The authors retrospectively evaluated the complications, pain. and degree of satisfaction in 69 consecutive patients affected by cervicofacial VM managed with STS using the Tessari method in a single institution. RESULTS: The average number of procedures for each patient was 2.1. The most frequent complication was blistering. We observed 1 patient of temporary weakness of a facial nerve branch, 1 paradoxical embolism, and 1 orbital compartment syndrome.The average pain score was 0 (no pain at all) (51.5%). There was no statistically significant correlation between patient satisfaction and the presence of complications or the degree of pain. CONCLUSIONS: Sclerotherapy with STS is an effective treatment that yields to very high patient satisfaction. This procedure has an overall low complication rate and is usually effective within a few sessions. However, severe complications may occur; these must be pointed out in the informed consent and the surgeon must be aware of and ready to quickly treat them to prevent long-term sequelae.

A Shift in the Orbit: Immediate Endoscopic Reconstruction After Transnasal Orbital Tumors Resection.

Endoscopic endonasal resection of orbital lesions is a safe and common approach. Nevertheless, medial orbital wall defects following the procedure are not routinely addressed, potentially leading to diplopia and enophthalmos. In this article, the authors propose a new technique for purely endoscopic endonasal reconstruction of orbital wall defects following endoscopic endonasal resection of orbital lesions.The patient, a 43-year-old male, suffering from right exophthalmos and diplopia due to a venous malformation of the right orbit underwent endoscopic endonasal resection of the mass. Excision was followed by immediate transnasal endoscopic reconstruction with a commercially available porous polyethylene mesh (Medpor).The postoperative course was uneventful. The patient did not report any residual orbital asymmetry or diplopia. No recurrence of the venous malformation, mesh infection, or reconstruction instability was reported during the follow-up.The authors believe that this new technique could spur head and neck surgeons in strategically rethinking their approach to orbital tumors, proposing reconstruction to patients on a routine basis, and developing even more reliable and manageable solutions.

Stereophotogrammetric Evaluation of Labial Symmetry After Surgical Treatment of a Lymphatic Malformation.

Lymphatic malformations (LMs) are rare, nonmalignant masses, frequently involving the head and neck, potentially causing impairment to the surrounding anatomical structures. Major LMs frequently cause facial disfigurement with obvious consequences on self-esteem and social functioning. The attempt to restore symmetry is thus one of the main goals of treatment. In this study, the authors present a not-invasive method to objectively quantify the symmetry of the labial area before and after surgical treatment of a LM, affecting a 16-year-old woman. This was done with sequential three-dimensional stereophotogrammetric imaging and morphometric measurements. The method showed a high reproducibility and supplied quantitative indicators of the local degree of symmetry, helping clinicians in its objective assessment, and facilitating treatment planning and evaluation. A quantitative appraisal of the results can additionally improve patient adherence to a usually multistage therapy.

Regional flaps in head and neck reconstruction: a reappraisal.

PURPOSE: Starting from our experience with 45 consecutive cases of regional pedicled flaps, we have underlined the effectiveness and reliability of a variety of flaps. The marketing laws as applied to surgical innovations are reviewed to help in the understanding of why regional flaps are regaining wide popularity in head and neck reconstruction. MATERIALS AND METHODS: From January 2009 to January 2014, 45 regional flaps were harvested at San Paolo Hospital to reconstruct head and neck defects. These included 35 pectoralis major muscular and myocutaneous flaps, 4 lower trapezius island or pedicled flaps, 3 supraclavicular flaps, 2 latissimus dorsi pedicled flaps, and 1 fasciocutaneous temporal flap. The basic literature of marketing regarding the diffusion of new products was also reviewed. RESULTS: Two myocutaneous pectoralis major flaps were complicated by necrosis of the cutaneous paddle (one complete and one partial). No complete loss of any of the 45 flaps was observed. At 6 months of follow-up, 2 patients had died of multiple organ failure after prolonged sepsis. The 43 remaining patients had acceptable morphologic and functional results. CONCLUSIONS: Regional and free flaps appear to compete in many cases for the same indications. From the results of the present case series, regional flaps can be considered reliable reconstructive choices that are less expensive than their free flap alternatives. The "resurrection" of regional flaps can be partially justified by the changes in the global economy and the required adaptation of developed and developing countries.

Retrobulbar hemorrhage during or after endonasal or periorbital surgery: what to do, when and how to do it.

Retrobulbar hemorrhage (RBH) is an uncommon complication of endoscopic sinus surgery or periorbital surgery consisting in an accumulation of blood within the orbit posteriorly to the eyeball. It must be treated within 90-100 minutes to avoid irreversible visual loss. The present paper tries to pinpoint the key steps in diagnosis and treatment of RBM.On the wake of a new case, the authors review and update their clinical experience and propose a step-by-step protocol to diagnose and to treat RBH developing under different circumstances: during endoscopic sinus surgery, during periorbital surgery, immediately after the surgery, in the awakening room, or postoperatively in the ward.A therapeutic ladder is proposed that starts with lateral canthotomy and inferior cantholysis and progresses to a lower lid transconjunctival incision with retrocaruncular extension.Based on our experience, the proposed guidelines are effective in diagnosing and treating RBH. They allow to preserve vision and minimize reliquates of this rare and dangerous surgical emergency.

Contemporary management of vascular malformations.

PURPOSE: To review the literature on vascular malformations and to clarify their diagnosis, clinical presentation, and treatment options. MATERIAL AND METHODS: The authors reviewed the current literature on vascular malformations looking for more innovative and credited diagnostic criteria and treatment protocols. RESULTS: The review is divided in 4 sections (capillary, venous, arteriovenous, and lymphatic malformations). In each section, the clinical presentation, radiologic features, and treatment options for each kind of vascular malformation are described. The experience and results of the authors also are presented. CONCLUSIONS: Vascular malformations are a heterogeneous group of diseases. Each type of malformation has unique features that make it largely different from the others. Only a clear and correct diagnosis can lead to optimal results.

Comprehensive surgical management of cocaine-induced midline destructive lesions.

PURPOSE: This article presents a review of the literature and proposes a protocol for managing acute and chronic midfacial cocaine-induced injuries. MATERIALS AND METHODS: This report describes a series of 4 patients affected by cocaine-induced midline destructive lesions. Three patients came to the authors' attention after 18 months of drug withdrawal and underwent surgical treatments to restore nasal and palatal morphology and function, and the fourth patient was referred because of acute cocaine-induced destructive lesions and was treated by aggressive debridement. An 18-month drug-free period is planned before beginning any reconstructive procedures in this latter patient. RESULTS: Long-term follow-up showed stable results without relapse of palatal fistulas and good esthetic nasal appearance in all 3 patients undergoing reconstruction. The fourth patient did not show any disease progression and will be monitored for drug withdrawal. CONCLUSION: Chronic cocaine consumption may cause multiple types of damage to the soft and hard tissues of the midface. Acute lesions must be addressed with aggressive debridement. As a result of chronic injury, the palate and nose are deformed in a very complex way and the vascularity of the remaining local tissues may be compromised or inadequate for flap harvesting. Palatal and nasal reconstructions are very delicate operations and should be addressed separately to maximize the predictability of the result.

Surgical treatment of vascular malformations of the facial bones.

PURPOSE: To characterize intraosseous vascular malformations and describe the most appropriate approach for treatment according to clinical experience and a review of the published data. MATERIALS AND METHODS: We performed a retrospective review of 11 vascular malformations (7 venous and 4 arteriovenous) of the facial bones treated during a 10-year period using en bloc resection or intraoral aggressive curettage alone or preceded by endovascular embolization. Corrective surgery was planned to address any residual bone deformities. The cases were reviewed at a mean follow-up point of 6 years. RESULTS: Facial symmetry was restored in the cases requiring reconstruction. Tooth sparing was possible in the case of jaw and/or maxillary localization. Recanalization occurred in 14% of the venous and 33% of the arteriovenous malformations. CONCLUSIONS: Facial intraosseous venous malformations can be successfully treated using surgery alone. Facial intraosseous arteriovenous malformations will be better addressed using combined approaches. Aggressive curettage will obviate the need for extensive surgical resection in selected cases.

Repair of cocaine-related oronasal fistula with forearm radial free flap.

BACKGROUND: Cocaine snorting may cause significant local ischemic necrosis and the destruction of nasal and midfacial bones and soft tissues, leading to the development of a syndrome called cocaine-induced midline destructive lesion. A review of the English-language literature reveals only a few articles describing the treatment of hard and/or soft palatal perforation related to cocaine inhalation. Described here are 4 patients with a history of cocaine abuse showing palatal lesions. MATERIALS AND METHODS: From 2010 to 2013, a total of 4 patients affected by cocaine-related midline destructive lesions were referred to our department. They all presented signs of a cocaine-induced midline destructive lesion. They showed wide midfacial destruction involving the nasal septum as well as the hard and soft palates causing an ample oronasal communication. RESULTS: In 3 patients, oronasal communication has been treated successfully using a personal technique based on a partially de-epithelialized forearm free flap. The fourth patient had been treated only with local debridement because, when she came to our attention, her abusive habits were still unsolved. DISCUSSION: Different surgical options have been reported such as local, regional, and free flaps for hard and soft palate reconstruction. However, because of an unpredictable vascularization of the palatal tissues and owing to the scarceness of the local soft tissues, local flaps are at high risk for partial and complete failure. The transfer of free vascularized tissue, however, seems to be the most reliable and logical solution for medium- to large-sized fistulas. Among the various free flaps, we choose the radial forearm type because of the pedicle length and the flap thickness.

The reconstruction of the spheno-orbital region using latissimus dorsi flap and costal graft.

To minimize complications in skull base surgery, it is necessary to separate intracranial structures from the upper aerodigestive tract with well-vascularized tissue. The majority of defects can be reconstructed using local flaps using pericranium, galea, or temporalis muscle. However, there are conditions that affect the suitability of the previously mentioned flaps, for example, previous surgical procedures or radiotherapy. Local flaps may also be inadequate to reconstruct particularly large defects. Extensive bony demolitions produce aesthetic deformities that need accurate reconstructions. Orbital wall defects have to be reconstructed to avoid complications such as the transmission of cerebral pulsation, bulbar dystopias, diplopia, and ophthalmoplegia. The microvascular latissimus dorsi flap is ideal in all these cases, and the use of a costal graft allows simultaneous reconstruction of bony defects. From January 2000 to January 2008, 17 patients have undergone surgical ablation of the spheno-orbital region and reconstruction with latissimus dorsi flap and costal grafts. The flap survival rate was 100%. One patient required revision of the venous anastomosis. No cerebrospinal fluid leak or intracranial infections have been detected. The only complications registered were dystopias in 6 patients and diplopia in 4; all of these spontaneously resolved within 2 months. The microvascular latissimus dorsi flap with costal graft is an effective method for the reconstruction of the spheno-orbital region when local flaps are not indicated. It has a negligible donor-site morbidity, an ideal vascular pedicle, and an easy harvesting technique. The costal graft allows the simultaneous reconstruction of the orbital walls, giving good results.

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.