Comparison of baseline characteristics and postoperative complications in neuromuscular, syndromic and congenital scoliosis.

Nonidiopathic scoliosis encompasses a group of diagnoses, including neuromuscular scoliosis, syndromic scoliosis and congenital scoliosis. The objective of this study was to compare the preoperative and postoperative clinical differences in pediatric nonidiopathic scoliosis patients with neuromuscular scoliosis vs. syndromic scoliosis/congenital scoliosis. This is a single-center retrospective review of all pediatric patients undergoing spinal instrumentation for nonidiopathic scoliosis during a 5-year period. Neuromuscular scoliosis patients ( n = 144), syndromic scoliosis patients ( n = 44) and congenital scoliosis patients ( n = 52) were compared. Demographics, patient characteristics and outcomes were compared. Neuromuscular scoliosis patients had lower BMI z-scores and were more likely to have pulmonary disease, technology dependence and seizure disorder. Additionally, neuromuscular scoliosis patients underwent bigger procedures with more levels fused and a higher rate of pelvis fixation. By direct comparison, neuromuscular scoliosis patients tended to have more complications including deep surgical site infections, readmission in 30 days, return to operating room in 90 days and emergency care visits in 90 days. When controlling for the differences in their preexisting conditions and surgical procedure, we found that pelvic fixation was a major confounding factor, whereas the others had no effect. We further subanalyzed cerebral palsy patients and found this group to exhibit no difference in complications compared to other neuromuscular scoliosis subtypes. Neuromuscular scoliosis patients have different characteristics and subsequent postoperative complications than those with syndromic scoliosis and congenital scoliosis. The difference in complication profile is mainly due to differences in surgical procedure and a higher rate of pelvic fixation. This should be considered when planning nonidiopathic scoliosis surgery among multidisciplinary teams.

Hamate Body Fractures: a Comprehensive Review of the Literature.

PURPOSE OF REVIEW: Due to the rarity and often discrete nature of hamate body fractures, timely diagnosis requires a high level of suspicion on the part of the clinician. Here, the authors have compiled the findings from 6 cohort studies and 33 case reports describing hamate body fractures in order to summarize the natural history, management, and outcomes of these infrequent injuries. RECENT FINDINGS: Fractures of the hamate body typically occur in the coronal plane through axial loading of the metacarpals or loading in the transverse plane by a compressive force. Standard radiographs of the wrist frequently miss hamate fractures. Oblique and carpal tunnel views can be obtained when a fracture of the hamate is suspected. Advanced imaging with high-resolution computed tomography should also be considered if radiographs are negative and high suspicion for fracture remains or for the purpose of pre-operative planning. Co-existing injuries often include subluxation or dislocation of the 4th and 5th metacarpals with or without fracture. Non-displaced injuries that are stable may be treated non-operatively with immobilization. Displaced or unstable fracture patterns typically require closed reduction and percutaneous pinning versus open reduction internal fixation in order to restore anatomical alignment and maximize outcomes. Hamate body fractures are uncommon fractures of the carpus. When appropriately treated, patients with hamate body fractures usually recover full pain-free range of motion and preserved grip strength. Complications are usually secondary to late presentation or noncompliance.

The role of Notch signaling in the mammalian ovary.

The Notch pathway is a contact-dependent, or juxtacrine, signaling system that is conserved in metazoan organisms and is important in many developmental processes. Recent investigations have demonstrated that the Notch pathway is active in both the embryonic and postnatal ovary and plays important roles in events including follicle assembly and growth, meiotic maturation, ovarian vasculogenesis and steroid hormone production. In mice, disruption of the Notch pathway results in ovarian pathologies affecting meiotic spindle assembly, follicle histogenesis, granulosa cell proliferation and survival, corpora luteal function and ovarian neovascularization. These aberrations result in abnormal folliculogenesis and reduced fertility. The knowledge of the cellular interactions facilitated by the Notch pathway is an important area for continuing research, and future studies are expected to enhance our understanding of ovarian function and provide critical insights for improving reproductive health. This review focuses on the expression of Notch pathway components in the ovary, and on the multiple functions of Notch signaling in follicle assembly, maturation and development. We focus on the mouse, where genetic investigations are possible, and relate this information to the human ovary.

Notch signaling regulates ovarian follicle formation and coordinates follicular growth.

Ovarian follicles form through a process in which somatic pregranulosa cells encapsulate individual germ cells from germ cell syncytia. Complementary expression of the Notch ligand, Jagged1, in germ cells and the Notch receptor, Notch2, in pregranulosa cells suggests a role for Notch signaling in mediating cellular interactions during follicle assembly. Using a Notch reporter mouse, we demonstrate that Notch signaling is active within somatic cells of the embryonic ovary, and these cells undergo dramatic reorganization during follicle histogenesis. This coincides with a significant increase in the expression of the ligands, Jagged1 and Jagged2; the receptor, Notch2; and the target genes, Hes1 and Hey2. Histological examination of ovaries from mice with conditional deletion of Jagged1 within germ cells (J1 knockout [J1KO]) or Notch2 within granulosa cells (N2 knockout [N2KO]) reveals changes in follicle dynamics, including perturbations in the primordial follicle pool and antral follicle development. J1KO and N2KO ovaries also contain multi-oocytic follicles, which represent a failure to resolve germ cell syncytia, and follicles with enlarged oocytes but lacking somatic cell growth, signifying a potential role of Notch signaling in follicle activation and the coordination of follicle development. We also observed decreased cell proliferation and increased apoptosis in the somatic cells of both conditional knockout lines. As a consequence of these defects, J1KO female mice are subfertile; however, N2KO female mice remain fertile. This study demonstrates important functions for Jagged1 and Notch2 in the resolution of germ cell syncytia and the coordination of somatic and germ cell growth within follicles of the mouse ovary.