OBJECTIVE: This retrospective systematic literature review aimed to summarize available data regarding epidemiology, etiology, presentation, investigations, differentials, treatment, prevention, monitoring, complications, and prognosis for radiation-induced cavernous malformations (RICMs) in pediatric patients.
METHODS: Review conducted per PRISMA guidelines. Google Scholar, PubMed, Trip Medical Database, and Cochrane Library searched utilizing a keyphrase, articles filtered per inclusion/exclusion criteria, duplicates excluded. Based on criteria, 25 articles identified, 7 further excluded from the systematic data but included in discussion (5 × insufficient data, 2 × other systematic reviews).
RESULTS: Many studies did not contain all explored data. 2487 patients reviewed, 325 later found to have RICM (143 male, 92 female). Mean age at irradiation 7.6 years (range 1.5-19). Mean total radiation dose 56 Gy (12-112). Most common indications for radiation-medulloblastoma 133x, astrocytoma 23x, ependymoma 21x, germinoma 19x. Mean age at RICM diagnosis 18 years (3.6-57). Mean latency to RICM 9.9 years (0.25-41). Most common anatomic locations-temporal 36, frontal 36, parietal 13, basal ganglia 16, infratentorial 20. Clinical presentation-incidental 270, seizures 19, headache 11, focal neurological deficit 7, other 13. 264 patients observed, 34 undergone surgery. RICM bled in 28 patients. Mean follow-up 11.7 years (0.5-50.3). Prognostic reporting highly variable.
CONCLUSIONS: From our data, pediatric RICMs appear to display slight male predominance, present about 10 years after initial irradiation in late teen years, and present incidentally in majority of cases. They are mostly operated on when they bleed, with incidental lesions mostly being observed over time. Further prospective detailed studies needed to draw stronger conclusions.

Isolated unilateral absence of lunate is an extremely rare condition with only one such case reported in English literature so far. The rarity of this condition can lead to diagnostic dilemma, especially if patient has an old history of trauma or surgery around the wrist, leading to unnecessary intervention and difficulty in ensuring a good surgical outcome. We present such a case and discuss the clinical pointers to the diagnosis of this condition. Level of Evidence: Level V (Therapeutic).

OBJECTIVE: Vascular anomalies are classified as either vascular tumors or vascular malformations. Vascular malformations can be difficult to diagnose and treat in the pediatric population and can masquerade as malignant processes. Understanding the genetics behind vascular malformations can lead to identification of specific mutations which can be treated with targeted immunotherapy.
METHODS: Our case presents a pediatric patient with progressively enlarging vascular malformation despite multiple surgical resections and systemic medical treatments who underwent genetic evaluation and was found to have PIK3CA mutation.
RESULTS: After identification of PIK3CA mutation, our patient was successfully treated with the p110ɑ-specific inhibitor, alpelisib, with both shrinkage of malformation on follow-up imaging as well as gains in her developmental milestones.
CONCLUSIONS: Progressive vascular malformations in the pediatric population can be hard to diagnose and treat and are thought to arise from somatic mutations. Our case highlights a patient with progressive malformation despite multiple surgical resections who was successfully treated with targeted immunotherapy after proper identification of genetic mutation.

BACKGROUND: Cervicofacial arteriovenous malformations (AVMs) are a significant source of morbidity. Endovascular embolization has emerged as a promising treatment technique for these lesions. However, current literature on cervicofacial AVM embolization mostly consists of single-agent oriented case series, and to date, no comprehensive study has compared the outcomes of available embolic agents.
OBJECTIVE: To investigate the performance of different embolic agents in the management of cervicofacial AVMs.
METHODS: We systematically searched Ovid MEDLINE, Ovid EMBASE, Ovid Cochrane Central Register of Controlled Trials Scopus, and Web of Science. Studies providing data for the endovascular treatment of cervicofacial AVMs were included. The data regarding complication and cure rates were collected for each embolic agent. Pooled event rates were presented as descriptive statistics.
RESULTS: Eleven studies comprising 204 patients were included in the review. The overall complete and partial cure rates were 62.2% (127/204) and 36.2% (74/204), respectively. Embolization failed to achieve significant improvement in only 1.6% (3/204) of the patients. The complete cure rates were 87.5% (75-100%) for n-Butyl cyanoacrylate (NBCA, glue), 80.5% (61-100%) for Onyx (Medtronic, MN, USA), and 51.5% (18-85%) for ethanol. The overall complication rate was 30% (61/204). The complication rates were 33% (12.5-53%) for ethanol, 14% (0-28%) for Onyx, and 0% for NBCA.
CONCLUSIONS: Our systematic review supports that endovascular embolization is an effective treatment option for cervicofacial AVMs. In our review, the use of Onyx and NBCA was associated with consistently high complete cure rates and a promising safety profile. However, more research is needed to investigate the use of different embolic agents in the treatment of cervicofacial AVMs.

Arthrogryposis multiplex congenita (AMC) is a heterogeneous group of disorders associated with decreased fetal movement, with a prevalence between 1/3000 and 1/5200 live births. Typical features of AMC include multiple joint contractures present at birth, and can affect all joints of the body, from the jaw, and involving the upper limbs, lower limbs and spine. The jaws may be affected in 25 % of individuals with AMC, with limited jaw movement and mouth opening. Other oral and maxillofacial deformities may be present in AMC, including cleft palate, micrognathia, periodontitis and delayed teething. To our knowledge, oral and maxillofacial abnormalities have not been systematically assessed in individuals with AMC. Therefore, this scoping review was conducted to identify, collect, and describe a comprehensive map of the existing knowledge on dental and maxillofacial involvement in individuals with AMC.
A scoping review was conducted in accordance with the Preferred Reporting Items for Systematic reviews and Meta-Analyses extension for Scoping Reviews guidelines. The PRISMA guidelines for scoping reviews were followed and databases were searched for empirical articles in English and French published until October 2022. We searched MEDLINE, Embase, Web of Science and ERIC databases. Two authors independently reviewed the articles and extracted the data.
Of a total of 997 studies that were identified, 96 met the inclusion criteria and were subsequently included in this scoping review. These 96 studies collectively provided insights into 167 patients who exhibited some form of oral and/or maxillofacial involvement. Notably, 25 % of these patients were within the age range of 0-6 months. It is worth highlighting that only 22 out of the 96 studies (22.9 %), had the primary objective of evaluating dental and/or maxillofacial deformities. Among the patients studied, a prevalent pattern emerged, revealing that severe anomalies such as micrognathia (56 %), high-arched palate (29 %), cleft palate (40 %), limited mouth opening (31 %), and dental anomalies (28 %) were frequently observed. Importantly, many of these patients were found to have more than one of these anomalies. Even though these maxillofacial impairments are known to be associated with dental problems (e.g., cleft palate is associated with oligodontia, hypodontia, and malocclusion), their secondary effects on the dental phenotype were not reported in the studies.
Our findings have uncovered a notable deficiency in existing literature concerning dental and maxillofacial manifestations in AMC. This underscores the need for interdisciplinary collaboration and the undertaking of extensive prospective cohort studies focused on AMC. These studies should assess the oral and maxillofacial abnormalities that can impact daily functioning and overall quality of life.

UNASSIGNED: The intracranial lipomas are rare congenital malformations accounting for approximately 0.1-1.3% of all intracranial tumors, of which Sylvian fissure lipomas account for <5%. These lesions are frequently associated with dysgenesis of neuronal brain tissues and vascular malformations and in the majority are asymptomatic. Intracranial lipomas on magnetic resonance imaging (MRI) may mimic late subacute hemorrhage due to similar radiological features. Due to the tight adhesion of the lipoma to the surrounding nerve structures and vessels, complete removal is difficult and does not guarantee the disappearance of symptoms.
UNASSIGNED: We present the case of a 42-year-old woman with chronic headaches and short-term memory impairment who was admitted to the emergency room after an out-of-hospital brain MRI with suspected ruptured right middle cerebral artery (MCA) aneurysm and late subacute intracranial hemorrhage. In the hospital, after clinical evaluation, emergency computed tomography (CT) angiography was performed, which revealed an unruptured fusiform aneurysm located in the right MCA trifurcation surrounded by an extremely hypodense lesion corresponding to fat in the right Sylvian fissure. No features of intracranial hemorrhage were present. The diagnosis of intracranial lipoma was finally confirmed after the MRI of the brain with a fat suppression sequence. Surgical treatment was not attempted, and the patient was treated conservatively with a satisfactory general outcome.
UNASSIGNED: A Sylvian fissure lipoma may be associated with a fusiform aneurysm in the MCA trifurcation. By modifying the standard MRI protocol and performing a CT scan, an intracranial lipoma can be detected and a late subacute intracranial hemorrhage can be excluded.

OBJECTIVE: To review hearing and surgical outcomes after reconstructive middle ear surgery in class 4 congenital middle ear anomalies (CMEA), e.g., patients with oval- or round window atresia of dysplasia.
METHODS: Pubmed/Medline, Embase and Cochrane library.
METHODS: Articles containing data on hearing outcomes and complications after reconstructive ear surgery in class 4 anomalies were analyzed and critically appraised. The following data were included and reviewed: patient demographics, audiometric testing, surgical techniques, complications, revision surgeries and their outcomes. Risk of bias was determined, and GRADE certainty of evidence was assessed. Primary outcomes were postoperative air conduction thresholds (AC), change in AC, and success rates (closure of the ABG to within 20 dB), the occurrence of complications (most importantly sensorineural hearing loss) and the long-term stability of hearing results (> 6-month follow-up) and occurrence of recurrence of preoperative hearing loss.
RESULTS: Success rates varied from 12.5 to 75% at long-term follow-up with larger cohorts reporting success rates around 50%, mean postoperative gain in AC varied from 4.7 to 30 dB and - 8.6 to 23.6 dB at, respectively, short- and long-term follow-up. No postoperative change in hearing occurred in 0-33.3% of ears, and recurrence of hearing loss occurred in 0-66.7% of ears. SNHL occurred in a total of seven ears across all studies of which three experienced complete hearing loss.
CONCLUSIONS: Reconstructive surgery can be an effective treatment option which should be considered in patients with very favorable baseline parameters, while also considering the substantial risk of recurrence of hearing loss, the possibility of unchanged hearing despite surgery and the rare occurrence of SNHL.
METHODS: 2c.

BACKGROUND: Intraoperative DSA is used to confirm complete obliteration of neurovascular pathologies. For spinal neurovascular lesions, femoral access can be challenging given the need to flip the patient after sheath placement. Similarly, radial access can be complicated by arch navigation difficulties. Vascular access via the popliteal artery represents an appealing alternative option; however, data regarding its utility and efficacy in these cases are limited.
METHODS: A retrospective series of four consecutive patients between July 2016 and August 2022 who underwent intraoperative spinal DSA via the popliteal artery was analyzed. Additionally, a systematic review was conducted to collect previously reported such cases. Collective patient demographics and operative details are presented to consolidate the available evidence supporting popliteal access.
RESULTS: Four patients met the inclusion criteria from our institution. The systematic review yielded six previously published studies reporting 16 additional transpopliteal access cases. Of the 20 total cases (mean±SD age 60.8±17.2 years), 60% were men. Most treated lesions were dural arteriovenous fistulas (80%) located in the thoracic spine (55%) or cervical spine (25%). The left popliteal artery was most accessed and the highest visualized level was the craniocervical junction. All outcomes were either stable or improving after surgery, and no complications were observed.
CONCLUSIONS: We report the safety and feasibility of transpopliteal access for intraoperative DSA in the prone position in four cases in addition to 16 previously reported cases in the literature. Our case series highlights popliteal artery access as an alternative to transfemoral or transradial access in this setting.

This study aimed to describe the characteristics of fetal demise after SARS-CoV-2 infections and clarify whether it is associated with clinical severity, placental lesions, or malformations or due to actual fetal infections.
PubMed and Web of Science databases were searched between December 1, 2019, and April 30, 2022.
Cohort, cross-sectional, and case-control studies and case series or case reports describing stillbirths or late miscarriages (ie, pregnancy loss occurring between 14 and 22 weeks of gestation, before and after the onset of labor) from mothers with SARS-CoV-2 infection during pregnancy (demonstrated by at least 1 positive real-time reverse transcription-polymerase chain reaction from nasopharyngeal swabs and/or SARS-CoV-2 placental infection). No language restriction was applied; cases with other causes possibly explaining the fetal demise were excluded.
The Preferred Reporting Items for Systematic Reviews and Meta-Analyses and Meta-analysis Of Observational Studies in Epidemiology guidelines were followed. The quality of the case series and case reports was evaluated using the specific Mayo Clinic Evidence-Based Practice Center tool. Maternal and clinical fetal data and placental and fetal virology and histology findings were collected. Data were summarized with descriptive statistics using the World Health Organization criteria to classify disease severity and fetal-neonatal infections.
Data from 184 mothers and 190 fetuses were analyzed. No clear link to maternal clinical severity or fetal malformation was evident. Approximately 78% of fetal demise cases occurred during the second and third trimesters of pregnancy, approximately 6 to 13 days after the diagnosis of SARS-CoV-2 infection or the onset of symptoms. Most placentas (88%) were positive for SARS-CoV-2 or presented the histologic features of placentitis (massive fibrin deposition and chronic intervillositis) previously observed in transplacentally transmitted infections (85%-91%). Of note, 11 fetuses (5.8%) had a confirmed in utero transmitted SARS-CoV-2 infection, and 114 fetuses (60%) had a possible in utero transmitted SARS-CoV-2 infection.
The synthesis of available data showed that fetal demise generally occurs a few days after the infection with histologic placental inflammatory lesions associated with transplacental SARS-CoV-2 transmission and eventually causing placental insufficiency.

BACKGROUND: Rbert\'s uterus, also known as asymmetric septate uterus, is a rare genital malformation first reported by Dr. Robert in 1970. Robert\'s uterus is characterized by a septate uterus with a blind hemicavity and an intact external fundus. According to some reports, Robert\'s uterus was typically managed by laparoscopic uterine resection of a hemicavity, laparoscopic endometrectomy, and even hysterectomy. Considering that fertility preservation is important in young patients, we recommend ultrasound-guided hysteroscopic septum resection as an optimum treatment for Robert\'s uterus.
METHODS: Herein is described a clinical case of Robert\'s uterus in a 15-year-old girl who was misdiagnosed for primary dysmenorrhea in the beginning. Magnetic resonance imaging (MRI) and 3-dimensional (3D) ultrasound identified an asymmetrical uterine septum. The patient was treated using ultrasound-guided hysteroscopic treatment without laparoscopy. The surgical procedure lasted less than an hour, and the symptoms of dysmenorrhea were relieved during a six months follow-up.
CONCLUSIONS: Ultrasound-guided hysteroscopic septum resection is the preferred treatment for Robert\'s uterus.