UNASSIGNED: To investigate the prevalence of palatogingival groove (PGG) affecting maxillary anterior teeth, bilateral occurrence, and distribution among sex in the Saudi subpopulation and to review the literature on the prevalence of PGG.
UNASSIGNED: Palatogingival groove (PGG) primarily affects maxillary lateral incisors and, when present, may contribute to the pathogenesis of periodontal and endodontic lesions.
UNASSIGNED: A total of 509 CBCT scans of Saudi patients with 2747 maxillary anterior teeth were included in the study. Patients\' information, the tooth type, the presence/absence, the unilateral/bilateral distribution, and the type of PGG according to Gu\'s classification (type I, II, or III) were recorded.
UNASSIGNED: The prevalence of the PGG in maxillary anterior teeth was 1.3%, affecting 32 (6.3%) patients. The PGGs were mostly detected in lateral incisors 25 (2.77%). The PGG was found to be unilateral in most patients (96.9%), with higher frequency in males than in females without significance for sex.
UNASSIGNED: PGG is not a rare anomaly in the Saudi population and is most frequently found in maxillary lateral incisors. Type I Gu\'s classification was mostly detected.

UNASSIGNED: Dandy-Walker malformation or syndrome is the most common posterior fossa malformation. It is commonly associated with other congenital anomalies such as cardiac defects; however, association with a giant occipital meningocele is extremely rare, as only around 34 cases have been described.
UNASSIGNED: We report a case of a 2-month-old female infant who presented with a midline, gigantic mass in the back of the head. It was first discovered on a prenatal ultrasound. The mass measured about 15 × 5 cm, extending to the midback, not changing in size with crying, not attached to the back, and with a positive transillumination test. The diagnosis was confirmed after doing a brain computed tomography, which revealed hypoplasia of the vermis with an enlarged posterior fossa as well as cystic dilation of both ventricles with herniation through a bone defect.
UNASSIGNED: Our case highlights a rare association between giant occipital meningocele and Dandy-Walker syndrome that is rarely discussed or reported in the medical literature. By reporting this extremely rare case of Dandy-Walker syndrome associated with a giant occipital meningocele, we hope to contribute to the creation of a database for future research so that a management protocol can be established for use by clinicians and neurosurgeons for better management of the condition.

Ultrasound examination is used for the assessment of abnormal findings on prenatal screening. Radial ray defect can be screened by using ultrasonography. Abnormal findings can be detected quickly by having the understanding of the etiology, pathophysiology and embryology. It is a rare congenital defect that may be isolated or associated with other anomalies including Fanconi\'s syndrome and Holt-Oram syndrome. We report the case of a 28-year-old woman (G2P1L1) who presented for routine antenatal ultrasound at 25 weeks 0 days according to the last menstrual period. The patient did not have any level-II antenatal anomaly scan done. An ultrasound was performed, and the gestational age according to the ultrasound scan was 24 weeks and 3 days. In this paper, we present a brief review of embryology and critical practical points, and report a rare case of radial ray syndrome with associated ventricular septal defect.

This study aimed to determine the incremental yield of prenatal exome sequencing over chromosomal microarray or G-banding karyotype in fetuses with: (1) intrauterine growth restriction related to placental insufficiency or (2) short long bones, in isolated and nonisolated instances for both scenarios.
Data were collected via electronic searches for relevant citations from January 2010 to April 10, 2022 in MEDLINE, Embase, Web of Science, and Cochrane, and using relevant bibliographies and data generated in-house.
Included were prospective or retrospective cohort studies and/or case series with: (1) n>5 cases of short long bones and/or intrauterine growth restriction undergoing prenatal sequencing with a clearly defined phenotype including assessment of placental function; (2) testing based on prenatal phenotype only; (3) a nondiagnostic chromosomal microarray/karyotype; and (4) known results of genetic testing.
Incremental yield was calculated for each study and as a pooled value for the aforementioned groups using a random-effects model. Results were displayed in forest plots with 95% confidence intervals. Heterogeneity was assessed statistically using Higgins\' I2. Publication bias was assessed graphically using funnel plots. Quality assessment was performed using modified Standards for Reporting of Diagnostic Accuracy criteria (International Prospective Register of Systematic Reviews registration number CRD42022324680).
Nineteen studies were included (n=452 cases). The apparent incremental yields with prenatal sequencing were: (1) 4% (95% confidence interval, -5.0 to 12; I2=0%) in isolated intrauterine growth restriction with evidence of placental insufficiency, (2) 30% (95% confidence interval, 13-47; I2=1%) in intrauterine growth restriction with additional structural anomalies, (3) 48% (95% confidence interval, 26-70; I2=73%) in isolated short long bones, and (4) 68% (95% confidence interval, 58-77; I2=51%) in short long bones with additional skeletal anomalies. Of the 37 short long bone cases with a diagnosis, 32 had a skeletal dysplasia, with thanatophoric dysplasia and osteogenesis imperfecta being the most common (both 21.6% [n=8/37]). In fetuses with short long bones and additional skeletal features, osteogenesis imperfecta was the most common diagnosis (28% [n=57/204]). Where documented, the inheritance patterns were de novo in 75.4% (n=150) of cases.
Prenatal sequencing adds substantially to incremental yield over chromosomal microarray in fetuses with short long bones or multisystem intrauterine growth restriction. Robust studies are required to assess the utility of fetal sequencing in isolated intrauterine growth restriction with evidence of placental insufficiency, which cannot be recommended on the basis of current evidence.

BACKGROUND: The hepatic artery (HA) is one of the most threatened vascular structures during hepatopancreatobiliary (HPB) surgeries and interventional procedures. It can be affected by many clinical pictures, especially tumors, due to its anatomical position and neighborhood.
OBJECTIVE: To reveal the evolution and recent developments in the management of HA traumas in the light of the literature.
METHODS: In this article, 100 years of MEDLINE (PubMed) literature and articles including cases and series of HA injuries were reviewed, and the types of injury occurrence, treatment, and related complications and their management were compiled.
RESULTS: The risk of HA injury increases during cholecystectomies and pancreatoduodenectomies, among the most common operations. HA anatomy shows anomalies in approximately 15%-25% of the cases, further increasing this risk. The incidence of HA injury is not precisely known. Approaches that have evolved in recent years in managing patients with HA injury (laceration, transection, ligation, resection) with severe morbidity and mortality risk are reviewed in light of the current literature.
CONCLUSIONS: In conclusion, complications and deaths due to HA injury are less common today. The risk of complications increases in patients with hemodynamic instability, jaundice, cholangitis, and sepsis. Revealing the variations in the preoperative radiological evaluation will reduce the risks. In cases where HA injury is detected, arterial flow continuity should be tried to maintain with primary anastomosis, arterial transpositions, or grafts. In cases where bile duct injury develops, patients should be directed to HPB surgery centers, considering the possibility of accompanying HA injury. Large-scale and multicentric studies are needed to understand better the early and long-term results of HA ligation and determine preventive procedures.

BACKGROUND: Splenic torsion is a rare condition but one that many surgeons will encounter once in their career. Management options are varied but due to the rarity of the condition there are no contemporary evidence-based summaries to inform a treating clinician. We aim to describe patterns of presentation and provide an evidence-based guide to the management.
METHODS: A PRISMA structured meta-analysis was conducted of all published cases of splenic torsion and a recent case added from our institution.
RESULTS: 408 cases were identified between 1888 and 2021 and a single case added from our institution, 312 cases were sourced from case reports and 96 from 40 case series. 8% of patients had a co-existing congenital anomaly and 28% an identified risk factor for splenic torsion. 82% required emergency surgery. A preoperative diagnosis is becoming more common, reaching 80% in 2020\'s. While spleen conserving surgery is feasible using a variety of techniques. splenectomy was the definitive management for the majority (82%). On histopatholy no occult disease was identified and a significant number of resected spleens were potentially viable; 32% were reported to be normal or congested and 14% demonstrated only partial or focal necrosis.
CONCLUSIONS: Despite the significant publication bias implied by the methodology this is a large dataset in a rare condition. Splenic torsion frequently occurs in a premorbid population. The presence of a palpable mass in the context of abdominal pain should increase suspicion and trigger cross sectional imaging. Conservation of the spleen, using the techniques discussed, should be seriously considered.

UNASSIGNED: As the largest and most complex cerebral artery, the middle cerebral artery (MCA) patterns and anomalies are not fully reported. At present, there is confusion about the criteria for the different subtypes. The study of MCA patterns and anomalies is important because variants such as accessories or duplicates represent a high risk of failure during endovascular embolization or navigation during treatment for ischemic stroke. This study conducted a systematic review of studies on the neuroangiography patterns and anomalies of MCA.
UNASSIGNED: We conducted a systematic review of four articles online databases and included English articles from PubMed, the Cochrane Library, Directory of Open Access Journals, and EBSCOhost.
UNASSIGNED: The proportion of the MCA branching pattern was 1.9% (range from 0% to 6.3%) for monofurcation, 1.0% (range from 0% to 1.4%) for tetrafurcation, 69.9% (range from 58.1% to 92.7%) for bifurcation, and 27% (ranging from 7.3% to 40.4%) for trifurcation. The proportion of MCA anomalies for accessory is 0.03% (range from 0% to 1%), duplication is 0.17% (range from 0% to 3%), and fenestration is 0.15% (range from 0% to 2%).
UNASSIGNED: The proportions of the branching pattern and anomalies of MCA based on the systematic review are described in this study. This study is the first to systematically review the neuroangiography pattern of MCA and neuroangiography variations/anomalies of MCA in the literature.

BACKGROUND: Tracheal trifurcation is an uncommon and often unknown type of anomalous tracheobronchial arborization, characterized by three main bronchi originating at the level of the carina. Diagnosis is important due to its clinical implications.
OBJECTIVE: To highlight the anatomical, clinical and diagnostic aspects of tracheal trifurcation by reporting our experience and reviewing the literature.
METHODS: We retrospectively evaluated pediatric patients referred to our institution from January 2018 to May 2020 with a diagnosis of tracheal trifurcation. All patients underwent chest radiographs, computed tomography (CT) (with/without dynamic airway scanning) and bronchoscopy. Clinical and anatomical data were collected.
RESULTS: Three patients with tracheal trifurcation were identified (1 female, median age: 7.6±0.4 months). All had associated anomalies: two had tetralogy of Fallot, one with esophageal atresia/tracheoesophageal fistula and one with an atrioventricular septal defect, and the other had skeletal ciliopathy. Chest radiographs were not diagnostic for tracheal trifurcation. Bronchoscopy could not fully evaluate the trachea and main bronchi in two cases. CT detected tracheal trifurcation in all of the patients and also demonstrated other lung parenchymal and congenital anomalies. Two of the three main bronchi were directed to the right lung in all cases. Ostial stenosis of either the central (two patients) or right upper bronchus (one patient) was present. No signs of tracheobronchomalacia were found.
CONCLUSIONS: Tracheal trifurcation is rare and often associated with additional disorders, such as other tracheobronchial anomalies, cardiovascular defects or esophageal malformations, responsible for clinical manifestations and earlier detection. Bronchoscopy is often used for diagnosis, but is invasive and may be incomplete or inconclusive, while CT allows for a noninvasive and correct diagnosis, while also highlighting additional findings in the thorax.

Cantrell\'s pentalogy is a congenital multiple malformation consisting of midline supraumbilical thoraco-abdominal wall defects, anterior and pericardial diaphragm defects, lower sternum defects, ectopia cordis and various intracardiac anomalies. Complete pentalogy is very rare. Some additional anomalies may accompany pentalogy and there are cases in the literature where chromosomal anomalies and pentalogy are seen. Cases of Cantrell\'s pentalogy in twin pregnancies are rare. Twin pregnancies with Cantrell\'s pentalogy in both fetuses are one of the rarest cases in the literature. In this study, we presented a twin pregnancy case with Cantrell\'s pentalogy in both fetuses and we reviewed twin pregnancy cases in which Cantrell\'s pentalogy was seen in the literature. In our case, anomalies were found in both fetuses in the evaluation performed on a 32-year-old, gravida 2, para 1 woman with 10 weeks + 5 days monochorionic-monoamniotic twin pregnancy. The first fetus had ectopia cordis, cystic hygroma and increased nuchal thickness (4.6 mm), and the anterior abdominal wall was in contact with the amniotic band. The second fetus had ectopia cordis omphalocele, cystic hygroma, holoprosencephaly and a single lower extremity deficiency. Both fetuses were pre-diagnosed with Cantrell\'s pentalogy and the parents were informed about the adverse course of the fetuses. After the completion of the legal procedures, with the approval and decision of the parents, termination was made in the 11th week of pregnancy. External images of the fetuses confirmed the diagnosis. The family denied the autopsy procedure. When we review the literature, twin pregnancies complicated by Cantrell\'s pentalogy are divided into 3 groups: Group1- cases where one of the twins has completely normal phenotype and the other twin has Cantrell\'s pentalogy; Group2- cases where one of the twins has Cantrell\'s pentalogy and the other twin does not have the Cantrell\'s pentalogy but has several anomalies; Group3- cases where both fetuses have Cantrell\'s pentalogy. In conclusion, early diagnosis of twin pregnancy cases complicated by Cantrell\'s pentalogy and determination of all anomalies in both fetuses are very important in terms of obstetric management.

OBJECTIVE: Maxillomandibular fusion (syngnathia) is a rare craniofacial anomaly. It occurs as either fibrous (synechiae) or bony (synostosis) fusion of jaw or fusion of mandible to zygoma, tuberosity, hard palate and temporal bone. There are several documentations delineating this condition but owing to its varying presentation, association with both intraoral and extraoral anomalies and syndromes, reviewing it has been as uphill task. The non-unanimous description of cases in the literature and the use of different nomenclatures make it difficult to classify this condition.
METHODS: Extensive search of the literature was done from the year 1936 to 2018 which included a total of 118 cases with 62 cases of bony fusion, 48 cases of fibrous fusion and 8 cases of combined fusion, i.e., fibrous on one site and bony on another side.
RESULTS: This paper proposes a novel, simple and explicit classification system on the basis of nature of fusing tissues, location and extent, association with syndrome after reviewing the existing literature. Furthermore, this review reports 4 new cases of syngnathia along with their clinical, radiographic features and their management protocol.
CONCLUSIONS: This classification can be generalized to all the cases of syngnathia and can further facilitate in its appropriate diagnosis and treatment plan.