Defects in diffusion barrier function of ciliary transition zone caused by ciliopathy variations of TMEM218.

Primary cilia are antenna-like structures protruding from the surface of various eukaryotic cells, and have distinct protein compositions in their membranes. This distinct protein composition is maintained by the presence of the transition zone (TZ) at the ciliary base, which acts as a diffusion barrier between the ciliary and plasma membranes. Defects in cilia and the TZ are known to cause a group of disorders collectively called the ciliopathies, which demonstrate a broad spectrum of clinical features, such as perinatally lethal Meckel syndrome (MKS), relatively mild Joubert syndrome (JBTS), and nonsyndromic nephronophthisis (NPHP). Proteins constituting the TZ can be grouped into the MKS and NPHP modules. The MKS module is composed of several transmembrane proteins and three soluble proteins. TMEM218 was recently reported to be mutated in individuals diagnosed as MKS and JBTS. However, little is known about how TMEM218 mutations found in MKS and JBTS affect the functions of cilia. In this study, we found that ciliary membrane proteins were not localized to cilia in TMEM218-knockout cells, indicating impaired barrier function of the TZ. Furthermore, the exogenous expression of JBTS-associated TMEM218 variants but not MKS-associated variants in TMEM218-knockout cells restored the localization of ciliary membrane proteins. In particular, when expressed in TMEM218-knockout cells, the TMEM218(R115H) variant found in JBTS was able to restore the barrier function of cells, whereas the MKS variant TMEM218(R115C) could not. Thus, the severity of symptoms of MKS and JBTS individuals appears to correlate with the degree of their ciliary defects at the cellular level.

The Joubert-Meckel-Nephronophthisis Spectrum of Ciliopathies.

The Joubert syndrome (JS), Meckel syndrome (MKS), and nephronophthisis (NPH) ciliopathy spectrum could be the poster child for advances and challenges in Mendelian human genetics over the past half century. Progress in understanding these conditions illustrates many core concepts of human genetics. The JS phenotype alone is caused by pathogenic variants in more than 40 genes; remarkably, all of the associated proteins function in and around the primary cilium. Primary cilia are near-ubiquitous, microtubule-based organelles that play crucial roles in development and homeostasis. Protruding from the cell, these cellular antennae sense diverse signals and mediate Hedgehog and other critical signaling pathways. Ciliary dysfunction causes many human conditions termed ciliopathies, which range from multiple congenital malformations to adult-onset single-organ failure. Research on the genetics of the JS-MKS-NPH spectrum has spurred extensive functional work exploring the broadly important role of primary cilia in health and disease. This functional work promises to illuminate the mechanisms underlying JS-MKS-NPH in humans, identify therapeutic targets across genetic causes, and generate future precision treatments.

Light and scanning electron microscope characterization of mandibular symphysis tissue as a functional adaptation in the mandible development of human fetuses.

When developing, the mandible presents great plasticity and contains condensed mesenchymal cells that develops into Meckel's cartilage, of which the anterior part forms the mandibular symphysis. Mandible human development studies focus on investigating whether the beginning of mandibular fusion in fetal period is related to symphysis ossification and the tensions imposed on it, considering that tongue movements, mouth opening, and closing can be seen in fetuses. This research analyses tissue modifications during human mandibular symphysis growth using light and scanning electron microscopy to relate them to its functional structure. The study sample consisted of 12 human fetuses distributed into two groups: Group I (GI) of 10-14 weeks old and Group II (GII) of 20-24 weeks old. Fragments of mandibular symphysis were removed en bloc together with the surrounding tissues to preserve the relation with adjacent structures. Decalcified specimens were prepared in semi-serial coronal sections 5-µm-thick and stained with hematoxylin and eosin, Masson՚s trichrome, Verhoeff, and Sirius red for histological analysis with light microscopy. Collagen fibers Type I or III and elastic fibers were quantified by volume fraction (Vv). Coronal sections of the GI and GII symphyseal region were submitted to scanning electron microscopy. Comparison between groups used independent t-test. Our study presents the different endochondral ossification stages in the anterior part of Meckel's cartilage in GI. Both groups showed abundantly vascularized mesenchymal tissue with intense cellular activity forming the mandibular symphysis, such as a source of new osteoblasts adjacent to the newly deposited bone matrix. Scanning electron microscopy analysis revealed an invasion of the bony trabecula in the transverse direction from the hemimandible, rectilinear in GI and sinuous in GII due to interdigitating bone process, promoting its ossification. In collagen Vv analysis was verified a prevalence of type I in GII and type III in GI, indicating a proportional relation between maturation and tissue arrangement. Functionally, the collagen and elastic fibers in the mandibular symphysis were arranged in a pantographic network, and the fibrillar interconnectivity clearly contributes to resilience capacity and efficiency of the force transfer. This study inferred the functional significance of the knowledge about the tissue composition of mandibular symphysis, and the importance of this tissue for surrounding structures. The mesenchymal tissue of mandibular symphysis participates in bone growth process, revealing an adaptation mechanism of mandibular symphysis in the fetal period investigated.

Sphenomandibular ligament and degenerating Meckel's cartilage revisited: Sequential variations with temporal bone deformity for ligament attachment in near-term human fetuses.

BACKGROUND: The sphenomandibular ligament (SML) is considered to originate from Meckel's cartilage (MC). However, no study has examined how the os goniale contributes to SML development. METHODS: Semiserial histological sections of heads from 18 near-term fetuses at 27-40 weeks of gestation were examined. OBSERVATIONS: The os goniale and the anterior process of the malleus (AP) provided a long, bar-like membranous bone complex that passed through the petrotympanic and tympanosquamosal fissures. Notably, the AP-goniale complex is sometimes elongated inferiorly to join the SML (n = 4 specimens). Along the complex in the bone fissures, a degenerating MC was often present (n = 12). With (n = 6) or without (n = 3) the MC remnant, the tympanic bone (TYB) protruded inferomedially near the tympanosquamosal fissure, and it sometimes continued to a cartilaginous SML (n = 3). The temporal bone squamosa or petrosa provided a similar bony process approaching the SML. The middle meningeal artery often ran between the sphenoid and petrosa. CONCLUSIONS: Most of the specimens (n = 15) exhibited a sequential change from a cartilaginous SML as a continuation of the MC remnant to the ligament after the disappearance of the cartilage. The degenerating MC appeared to cause transformation from the AP-goniale complex and/or TYB to "another ligament" that replaced the usual SML at the upper part. Near the MC remnant, a similar transformation was also suggested on the squamosa or petrosa. The sphenoid spine appeared to originate often from the sphenoid ala major but sometimes from the TYB.

Meckel's Diverticulum in Adult Geese (Alopochen egyptiacus): A Comprehensive Study of Structure Using Histological, Electron Microscopy, and Immunohistochemical Methods.

INTRODUCTION: The intestine plays an important role in mediating between the bird and its nutritional environment. The yolk stalk, also known as Meckel's diverticulum, is a landmark between the jejunum and ileum. This work aimed to investigate the anatomical, histological, and electron microscopical features of cellular components of the Meckel's diverticulum (MD) in adult geese. METHODS: The intestine was dissected from the bird's body cavity, and Meckel's diverticulum was exposed and prepared for light and electron microscopical examinations. RESULTS: Our results revealed that the MD mucosa is thrown up into villi and crypts, and the mucosal epithelium is a columnar epithelium with goblet cells as well as intraepithelial lymphocytes. Lymphoid follicles and numerous immune cells were demonstrated within the lamina propria. The mucous glands were also observed within the lamina propria and among the lymphoid follicles. The lining epithelium of MD appeared with different staining affinities: dark cells (electron-dense) and light cells (electron-lucent) contained few mitochondria and more secretory vesicles, while dark cells contained more mitochondria and fewer secretory vesicles. Immunohistochemical analysis of MD revealed positive immunoreactivity for several markers, such as CD117, chromogranin, PLCß, cytokeratin, MHC II, and S100. CONCLUSION: Taken together, our findings suggest that MD is considered an immune organ in adult geese.

Detection and molecular characterization of chicken parvovirus and chicken megrivirus in layer breeders affected by intestinal dilatation syndrome.

RESEARCH HIGHLIGHTS: IDS presented pathognomonic dilatation of the jejunum up to Meckel's diverticulum.IDS caused weight loss, decreased egg production, and increased culling and mortality.Chicken parvovirus (ChPV) was consistently detected through PCR assays.Chicken megrivirus (ChMV) was consistently detected through viral metagenomics.

Meckel-Gruber syndrome together with Dandy-Walker malformation: an atypical case report of a 2nd recurrence in a consanguine marriage.

Meckel-Gruber syndrome is a lethal disorder characterized by occipital encephalocele, polycystic kidneys, and polydactyly. In most cases, it is identified and terminated antenatally. In this report, the authors present a case of Meckel-Gruber syndrome together with Dandy-Walker malformation. A pregnant woman referred at the 28th week of gestation with an abnormal ultrasound scan showing posterior encephalocele and bilaterally enlarged kidneys. Further imaging also indicated communication between the 4th ventricle and posterior cerebellar cerebrospinal fluid space, after which the fetus was diagnosed with Meckel-Gruber syndrome and Dandy-Walker malformation. Pregnancy termination was refused by the parents and the offspring was prematurely born to be the 2nd recurrence of Meckel-Gruber syndrome in this consanguine family. Remarkably, at the 3 different pregnancies, ultrasound was inconclusive before the 7th month of gestation. Though up to date Meckel-Gruber syndrome is ultimately lethal, the lifespan of affected newborns varied greatly. We suggest developing a severity classification to estimate life expectancy in unterminated cases.

Predicting balloon shape in percutaneous microcompression : an observational comparative analysis of Meckel's cave imaging and balloon morphology.

Achieving a pear-shaped balloon holds pivotal significance in the context of successful percutaneous microcompression procedures for trigeminal neuralgia. However, inflated balloons may assume various configurations, whether it is inserted into Meckel's cave or not. The absence of an objective evaluation metric has become apparent. To investigate the relationship between the morphology of Meckel's Cave and the balloon used in percutaneous microcompression for trigeminal neuralgia and establish objective criteria for assessing balloon shape in percutaneous microcompression procedures. This retrospective study included 58 consecutive patients with primary trigeminal neuralgia. Data included demographic, clinical outcomes, and morphological features of Meckel's cave and the balloon obtained from MRI and Dyna-CT imaging. MRI of Meckel's cave and Dyna-CT of intraoperative balloon were modeled, and the morphological characteristics and correlation were analyzed. The reconstructed balloon presented a fuller morphology expanding outward and upward on the basis of Meckel's cave. The projected area of balloon was strongly positively correlated with the projected area of Meckel's cave. The Pearson correlation coefficients were 0.812 (P<0.001) for axial view, 0.898 (P<0.001) for sagittal view and 0.813 (P<0.001) for coronal view. Similarity analysis showed that the sagittal projection image of Meckel's cave and that of the balloon had good similarity. This study reveals that the balloon in percutaneous microcompression essentially represents an expanded morphology of Meckel's cave, extending outward and upward. There is a strong positive correlation between the volume and projected area of the balloon and that of Meckel's cave. Notably, the sagittal projection image of Meckel's cave serves as a reliable predictor of the intraoperative balloon shape. This method has a certain generalizability and can help providing objective criteria for judging balloon shape during percutaneous microcompression procedures.

Open-door extended endoscopic transorbital technique to the paramedian anterior and middle cranial fossae: technical notes, anatomomorphometric quantitative analysis, and illustrative case.

OBJECTIVE: The superior eyelid endoscopic transorbital approach (SETOA) provides a direct and short minimally invasive route to the anterior and middle skull base. Nevertheless, it uses a narrow corridor that limits its angles of attack. The aim of this study was to evaluate the feasibility and potential benefits of an "extended" conservative variant of the "standard" endoscopic transorbital approach-termed "open-door"-to enhance the exposure of lesions affecting the paramedian aspect of the anterior and middle cranial fossae. METHODS: First, the authors described the technical nuances of the open-door extended transorbital approach (ODETA). Next, they documented its morphometric advantages over standard SETOA. Finally, they provided a clinical-anatomical application to demonstrate enhanced exposure and better angles of attack to treat lesions occupying the paramedian anterior and middle cranial fossae. Five adult cadaveric specimens (10 sides) initially underwent standard SETOA and then extended open-door SETOA (ODETA to the paramedian anterior and middle fossae). The adjunct of hinge-orbitotomy, through three surgical steps and straddling the frontozygomatic suture, converted conventional SETOA to its extended open-door variant. CT scans were performed before dissection and uploaded to the neuronavigation system for quantitative analysis. The angles of attack on the axial plane that addressed four key landmarks, namely the tip of the anterior clinoid process (ACP), foramen rotundum (FR), foramen ovale (FO), and trigeminal impression (TI), were calculated for both operative techniques and compared. RESULTS: Hinge-orbitotomy of the extended open-door SETOA resulted in several surgical, functional, and esthetic advantages: it provided wider axial angles of attack for each of the target points, with a gain angle of 26.68° ± 1.31° for addressing the ACP (p < 0.001), 29.50° ± 2.46° for addressing the FR (p < 0.001), 19.86° ± 1.98° for addressing the FO (p < 0.001), and 17.44° ± 2.21° for addressing the lateral aspect of the TI (p < 0.001), while hiding the skin scar, avoiding temporalis muscle dissection, preserving flap vascularization, and decreasing the rate of bone infection and degree of orbital content retraction. CONCLUSIONS: The extended open-door technique may be specifically suited for selected patients affected by paramedian anterior and middle fossae lesions, with prevalent anteromedial extension toward the anterior clinoid, the foremost compartment of the cavernous sinus and FR and not completely controlled with the pure endoscopic transorbital approach.

Meckel's diverticulum mimicking acute appendicitis in children: a retrospective cohort study.

BACKGROUND: The aims of this study were to summarize the clinical presentation and histological results of 20 cases of complicated Meckel diverticulum (MD) who were presumed to have acute appendicitis before surgery, as well as to improve the diagnosis and treatment of complicated MD in children. MATERIALS AND METHODS: We retrospectively reviewed the records of 20 complicated MD admitted to our institution who were preoperatively diagnosed with acute appendicitis from January 2012 to January 2019. Patients were divided into the perforated MD group and the Meckel's diverticulitis group. Patient demographics, clinical manifestations, laboratory data, auxiliary examinations, surgical methods, and the result of heterotopic tissue were recorded. RESULTS: A total of 20 cases of complicated MD (perforated or diverticulitis) were identified. Children were aged from 3 to 13 years, with a mean age of 7.75 years (median 7.75; range, 1-13 years). Perforated Meckel's diverticulum occurred in 5 of 20 (25%) cases. For perforated MD versus diverticulitis, no significant differences were found between age, time to intervention, length of hospital stay, and distance from the ileo-cecal valve. Heterotopic tissue was confirmed on histopathology in 75% of all patients, including 10 cases of gastric mucosa, 3 cases of coexistent gastric mucosa and pancreatic tissue, and 2 cases of pancreatic tissue. All patients underwent diverticulectomy or partial ileal resection under laparoscopy or laparotomy; two cases combined with appendectomy owing to slight inflammation of the appendix. CONCLUSIONS: The most common presentation of symptomatic MD is painless rectal bleeding; however, it can present symptoms of acute abdomen mimicking acute appendicitis. The key point of diverticulectomy is to remove the ectopic mucosa completely.

Interdural extra-arachnoidal resection of fifth nerve schwannomas with tailored petrous drilling: Eliminating the blind spot.

BACKGROUND AND OBJECTIVES: Around 20-40% of trigeminal schwannomas (TS) are dumbbell shaped, spanning the middle and posterior cranial fossa The petrous apex is often truncated in these patients, aiding surgical resection of both compartments through the middle fossa approach. However, a less eroded petrous creates a blind spot, making total resection difficult. This study describes the feasibility of an approach combining expanded Meckel cave access with tailored petrous bone drilling to optimize tumor visualization and resection. METHODS: Eleven patients with dumbbell TS and minimal petrous apex erosion underwent the described surgery. Surgical steps included temporo-orbito-zygomatic craniotomy, middle fossa floor drilling, navigation aided (tailored) extradural petrous bone drilling and extra-arachnoidal tumor excision. Extent of resection and postoperative outcomes were recorded. RESULTS: Patients presented with trigeminal nerve dysfunction (n = 9; sensory -9 and motor - 5), headache(8/11), ataxia (7/11) and pseudobulbar palsy (3/11). Complete tumor resection was achieved in all patients. Postoperatively, 8 out of 9 patients had transient increase in facial hypoesthesia with conjunctival injection in 3. It improved within 3-6 months, except in four cases wherein mild hypoesthesia persisted. Motor symptoms improved in 2 of 5 patients. Two developed transient 6th nerve paresis, that resolved in 2 months. Cerebellar and brainstem pressure symptoms resolved in all. No patients developed new onset permanent neurological deficit. Two patients reported mild post-craniotomy masticatory difficulty. CONCLUSIONS: The additional tailored petrous bony drilling enhances the surgeon's view, allowing a higher chance of total resection with no major operative morbidity.

Clinical features and treatment of heterotopic pancreas in children: a multi-center retrospective study.

OBJECTIVE: Heterotopic pancreas, an uncommon condition in children, can present with diagnostic and treatment challenges. This study aimed to evaluate the clinical features and treatment options for this disorder in pediatric patients. METHODS: We conducted a retrospective analysis, including patients diagnosed with heterotopic pancreas at four tertiary hospitals between January 2000 and June 2022. Patients were categorized into symptomatic and asymptomatic groups based on clinical presentation. Clinical parameters, including age at surgery, lesion size and site, surgical or endoscopic approach, pathological findings, and outcome, were statistically analyzed. RESULTS: The study included 88 patients with heterotopic pancreas. Among them, 22 were symptomatic, and 41 were aged one year or younger. The heterotopic pancreas was commonly located in Meckel's diverticulum (46.59%), jejunum (20.45%), umbilicus (10.23%),ileum (7.95%), and stomach (6.82%). Sixty-six patients had concomitant diseases. Thirty-three patients had heterotopic pancreas located in the Meckel's diverticulum, with 80.49% of cases accompanied by gastric mucosa heterotopia (GMH). Patients without accompanying GMH had a higher prevalence of heterotopic pancreas-related symptoms (75%). Treatment modalities included removal of the lesions by open surgery, laparoscopic or laparoscopic assisted surgery, or endoscopic surgery based on patient's age, the lesion site and size, and coexisting diseases. CONCLUSIONS: Only one-fourth of the patients with heterotopic pancreas presented with symptoms. Those located in the Meckel's diverticulum have commonly accompanying GMH. Open surgical, laparoscopic surgical or endoscopic resection of the heterotopic pancreas is recommended due to potential complications. Future prospective multicenter studies are warranted to establish rational treatment options.

A Rare Case of Meckel-Gruber Syndrome with Congenital Intestinal Atresia and Abdominal Pseudocyst Clinic.

Background: Meckel-Gruber syndrome (MGS) is a rare disease with a fatal, autosomal recessive inheritance pattern. This article mentions the neonatal MGS case followed by intestinal atresia and meconium pseudocyst clinic. Case presentation: Bile-containing-fluid was aspirated from the fetus, which was found to have polyhydramnios, gastric dilatation, lung hypoplasia, and cystic formation with a diameter of 68*62mm in the abdomen at 32 weeks of gestation in the intrauterine period. The cyst recurred after 2 weeks. We operated the patient with the preliminary diagnosis of meconium pseudocyst due to intrauterine perforation. The general condition was moderate in the postoperative period, and intermittent bilious vomiting continued. We performed an ileostomy on the patient due to his inability to tolerate oral intake, lack of passage, and abdominal distension. In addition, as a result of liver biopsy, cholestasis, cholestatic changes, bile-duct loss, and ductular reaction were detected. According to the current clinical findings and genetic analysis results, the patient was diagnosed with MGS. Conclusion: Autosomal recessive, fatal diseases such as MGS are pathologies with a high probability of recurrence with each pregnancy. Therefore, awareness needs to be increased to prevent these diseases.

Mcrs1 is required for branchial arch and cranial cartilage development.

Mcrs1 is a multifunctional protein that is critical for many cellular processes in a wide range of cell types. Previously, we showed that Mcrs1 binds to the Six1 transcription factor and reduces the ability of the Six1-Eya1 complex to upregulate transcription, and that Mcrs1 loss-of-function leads to the expansion of several neural plate genes, reduction of neural border and pre-placodal ectoderm (PPR) genes, and pleiotropic effects on various neural crest (NC) genes. Because the affected embryonic structures give rise to several of the cranial tissues affected in Branchio-otic/Branchio-oto-renal (BOR) syndrome, herein we tested whether these gene expression changes subsequently alter the development of the proximate precursors of BOR affected structures - the otic vesicles (OV) and branchial arches (BA). We found that Mcrs1 is required for the expression of several OV genes involved in inner ear formation, patterning and otic capsule cartilage formation. Mcrs1 knockdown also reduced the expression domains of many genes expressed in the larval BA, derived from either NC or PPR, except for emx2, which was expanded. Reduced Mcrs1 also diminished the length of the expression domain of tbx1 in BA1 and BA2 and interfered with cranial NC migration from the dorsal neural tube; this subsequently resulted in defects in the morphology of lower jaw cartilages derived from BA1 and BA2, including the infrarostral, Meckel's, and ceratohyal as well as the otic capsule. These results demonstrate that Mcrs1 plays an important role in processes that lead to the formation of craniofacial cartilages and its loss results in phenotypes consistent with reduced Six1 activity associated with BOR.

Msx1 is essential for proper rostral tip formation of the mouse mandible.

The right and left mandibular processes derived from the first branchial arch grow toward the midline and fuse to create the rostral tip region of the mandible during mandibular development. Severe and mild cases of failure in this process results in rare median cleft of the lower lip and cleft chin, respectively. The detailed molecular mechanisms of mandibular tip formation are unknown. We hypothesize that the Msx1 gene is involved in mandibular tip development, because Msx1 has a central role in other craniofacial morphogenesis processes, such as teeth and the secondary palate development. Normal Msx1 expression was observed in the rostral end of the developing mandible; however, a reduced expression of Msx1 was observed in the soft tissue of the mandibular tip than in the lower incisor bud region. The rostral tip of the right and left mandibular processes was unfused in both control and Msx1-null (Msx1-/-) mice at embryonic day (E) 12.5; however, a complete fusion of these processes was observed at E13.5 in the control. The fused processes exhibited a conical shape in the control, whereas the same region remained bifurcated in Msx1-/-. This phenotype occurred with 100% penetrance and was not restored at subsequent stages of development. Furthermore, Meckel's cartilage in addition to the outline surface soft tissues was also unfused and bifurcated in Msx1-/- from E14.5 onward. The expression of phosho-Smad1/5, which is a mediator of bone morphogenetic protein (Bmp) signaling, was downregulated in the mandibular tip of Msx1-/- at E12.5 and E13.5, probably due to the downregulated Bmp4 expression in the neighboring lower incisor bud. Cell proliferation was significantly reduced in the midline region of the mandibular tip in Msx1-/- at the same developmental stages in which downregulation of pSmad was observed. Our results indicate that Msx1 is indispensable for proper mandibular tip development.

The relationship between Meckel's cartilage resorption and incisor tooth germ in mice.

Our understanding of the initiation and cellular mechanisms underlying endochondral resorption of Meckel's cartilage (MC) remains limited. Several studies have shown that the resorption site of MC and the mandibular incisor tooth germ are located close to each other. However, whether incisor tooth germ development is involved in MC resorption remains unclear. In this study, we aimed to elucidate the spatio-temporal interaction between the initiation site of MC resorption and the development of incisor tooth germs in an embryonic mouse model. To this effect, we developed a histology-based three-dimensional (3D) reconstruction technique using paraffin-embedded serial sections of various tissues in the jaw. The serial sections were cut in the frontal section and the tissue constituents (e.g., MC, incisor, and mineralized mandible) were studied using conventional and enzyme-based histochemistry. The outline of each component was marked on the frontal sectional images and 3D structures were constructed. To assess the vascular architecture at the site of MC resorption, immunohistochemical staining using anti-laminin, anti-factor VIII, and anti-VEGF antibodies was performed. MC resorption was first observed on the lateral incisor-facing side of the cartilage rods at sites anterior to the mental foramen on E16.0. The 3D analysis suggested that: (a) the posterior region of the clastic cartilage resorption corresponds to the cervical loop of the incisor; (b) the cervical portion of the tooth germ inflates probably due to temporal cellular congestion prior to differentiation into matrix-producing cells; (c) the incisor tooth germ tissue is present in close proximity to MC even in mouse with continuously growing tooth and determines the disappearance of MC as the tooth development.

Endoscopic characteristics of Meckel's diverticulum in adults: a retrospective case-series from two tertiary general hospitals in China.

BACKGROUND: To analyze the diversity in endoscopic manifestations of Meckel's diverticulum (MD) in adults by using balloon-assisted enteroscopy (BAE) and supply more information on the application of BAE. METHODS: A retrospective study was carried out on adult patients diagnosed with MD by BAE in two tertiary general hospitals in China, from May 2007 to September 2021. The patients were divided into a small bowel bleeding (SBB) group and a control group according to their main symptoms. Clinical charts and endoscopic images were reviewed, analyzed, and summarized. RESULTS: Single diverticulum in the ileum and double-lumen sign were observed in all patients. The SBB group consisted of 51 patients, among which 35 cases of ulcerative lesions, 9 cases of erosive lesions, 9 cases of active bleeding/blood clots, and 4 cases of lumps inside the diverticulum were observed respectively. Majority of ulcerative lesions were inside the diverticulum (23/35). A circumferential stricture inside the diverticulum was discovered in 11 cases, and ulcerative lesions tended to occur at this structure (10/11). In the control group consisting of 15 patients, 1 case of erosive lesions at the orifice edge was observed. The percentage of patients with MD-associated ulcerative lesions was significantly higher in the SBB group than that in the control group (p < 0.001). CONCLUSIONS: The endoscopic manifestations of MD in adults are extraordinarily complex and connected with the patients' primary symptoms. The internal features of MD should be regarded as crucial observational objectives in adult patients.

Regular use of low-dose of opioids after gastrointestinal surgery may lead to postoperative gastrointestinal tract dysfunction in children: a Chinese national regional health center experience sharing.

BACKGROUND: The need for pain management is increasing in pediatrics, but the side effects of overuse or abuse of analgesics can be harmful to children's health and even life-threatening in severe cases. METHODS: Patients who underwent resection of Meckel's diverticulum at the Children's Hospital of Chongqing Medical University from July 1, 2019, to July 1, 2022, were included in this study. Opioids were administered through patient-controlled analgesia (PCA). Based on the preoperative choices made by the legal guardians, patients were stratified into two groups: PCA Group (PCAG) and Non-PCA Group (NPCAG). Data pertaining to the clinical characteristics and prognoses of these patients were subsequently collected and analyzed to assess the impact of opioid administration. RESULTS: In the study, a total of 126 patients were enrolled, with 72 allocated to the Patient-Controlled Analgesia Group (PCAG) and 54 to the Non-Patient-Controlled Analgesia Group (NPCAG). When compared to the NPCAG, the PCAG exhibited a longer duration of postoperative fasting (median 72 vs. 62 h, p = 0.044) and increased utilization of laxatives (12[16.7%] vs. 2[3.7%], p = 0.022). However, the PCAG also experienced higher incidences of intestinal stasis and abnormal intestinal dilation (13[18.1%] vs. 3[5.6%], p = 0.037). No statistically significant differences were observed in pain assessments at the conclusion of the surgical procedure (0 vs. 1[1.9%], p = 0.429) or within the first 24 h postoperatively (16[22.2%] vs. 18[33.3%], p = 0.164). Additionally, NPCAG patients did not necessitate increased administration of rescue analgesics (2[2.8%] vs. 4[7.4%], p = 0.432). CONCLUSIONS: The administration of opioids did not demonstrably ameliorate postoperative pain but was associated with a heightened incidence of postoperative gastrointestinal tract dysfunction. The retrospective nature of the current research should be considered and should be clarified further.

Trigeminal Schwannoma Surgery: Challenges in Preserving Facial Sensation.

Treatments of schwannoma have dramatically improved in the previous few decades, but preservation of the functions of the originating nerve, such as facial sensation in trigeminal schwannomas, still remains challenging. As the preservation of facial sensation in trigeminal schwannomas has not been analyzed in detail, we here review our surgical experience of more than 50 trigeminal schwannoma patients, particularly focusing on their facial sensation. Since the facial sensation in each trigeminal division showed a different perioperative course even in a single patient, we investigated patient-based outcomes (average of the three divisions in each patient) and division-based outcomes separately. In the evaluation of patient-based outcomes, facial sensation remained postoperatively in 96% of all the patients, and improved in 26% and worsened in 42% of patients with preoperative hypesthesia. Posterior fossa tumors tended to most rarely disrupt facial sensation preoperatively, but were the most difficult to preserve facial sensation postoperatively. Facial pain was relieved in all six patients with preoperative neuralgia. In the division-based evaluation, facial sensation remained postoperatively in 83% of all the trigeminal divisions, and improved in 41% and worsened in 24% of the divisions with preoperative hypesthesia. The V3 region was most favorable before and after surgery, with the most frequent improvement and the least frequent functional loss. To clarify current treatment outcomes of the facial sensation and to achieve more effective preservation, standardized assessment methods of perioperative facial sensation may be required. We also introduce detailed MRI investigation methods for schwannoma, including contrast-enhanced heavily T2-weighted (CISS) imaging, arterial spin labeling (ASL), and susceptibility-weighted imaging (SWI), preoperative embolization for rare vascular-rich tumors, and modified techniques of the transpetrosal approach.

Endoscopic transorbital approach for skull base lesions: a report of 16 clinical cases.

Due to the deep location, complex anatomy, and adjacent vital neurovascular structures, skull base surgery is challenging and requires specific approaches. The emerging endoscopic transorbital approach (eTOA) technique provides a new approach to the orbital content, spheno-orbital region, lateral cavernous sinus, and Meckel's cave. In this study, the clinical utility and effectiveness of the eTOA are reported. Sixteen cases who underwent the eTOA were included in the current study. The patients were divided into 3 groups according to tumor location: Group A (intraorbital, 6 cases), group B (spheno-orbital, 7 cases), and group C (cavernous sinus, and Meckel's cave, 3 cases). The clinical data and surgical results were analyzed. Eight meningiomas, 2 hemangiomas, 1 low-grade glioma, 1 instance of inflammatory hyperplasia tissue, 1 Langerhans cell histiocytosis, 1 epidermoid cyst, 1 trigeminal schwannoma, and 1 bone fibrosis hyperplasia were observed. The mean tumor diameter was 2.4 cm. A single case in Group A and Group C underwent biopsy (12.5%), and 1 case of fibrous dysplasia in Group B underwent sufficient orbit decompression (6.25%). The remaining 13 cases underwent gross total tumor resection (81.25%). No cerebral-spinal fluid leak or infection occurred. And no cosmetic problems or significant complications were observed during the follow-up. As a minimally invasive technique, the eTOA has unique advantages for carefully selected skull base lesions because of its direct route, short working distance, and distinct attack angle.