OBJECTIVE: Extended total colonic aganglionosis (ETCA) represents uncommon forms of Hirschsprung disease (HD), with aganglionosis extending into the proximal small bowel. ETCA management is challenging and associated with poor outcomes and high mortality. This study compares management and outcomes of ETCA to more common HD forms.
METHODS: A retrospective cohort of HD patients (2012-2023) from two institutions. Three HD forms were compared: short-segment HD (SSHD, n = 19), long-segment HD or total colonic aganglionosis (LS/TCA, n = 9) and ETCA (n = 7).
RESULTS: Normally innervated segments in ETCA patients ranged 0-70 cm. Median times to first surgery were; ETCA = 3 days versus TCA = 21 days (p = 0.017) and SSHD = 95 days (p < 0.001), respectively. Median number of surgeries were; ETCA = 4, versus TCA = 2 (p = 0.17) and SSHD = 1 (p = 0.002), respectively. All the patients underwent a definitive pull-through procedure, except four ETCA patients with a permanent jejunostomy and residual aganglionic segment of 57-130 cm. ETCA patients had 92% lower odds of enterocolitis (14%) compared to TCA patients (67%, p = 0.054), and comparable odds to SSHD patients (16%, p = 0.92). ETCA mortality was 14%.
CONCLUSIONS: Extended total colonic aganglionosis patients require earlier and multiple interventions. Leaving an aganglionic segment may be advantageous, without increasing risk for enterocolitis. Tailored surgical treatment and rehabilitation programmes may prevent mortality and need for transplantation.

UNASSIGNED: Spinal tuberculosis (STB) accounts for 1% of all tuberculosis (TB) cases and 50% of skeletal TB. The classic presentation is a paradiscal involvement leading to the destruction of bodies, progressive kyphosis that can end with neurological weakness. The advent of magnetic resonance imaging (MRI) has made detection early but at the same time, we find multilevel involvement which can be continuous/noncontinuous.
UNASSIGNED: A 26-year-old male, non-alcoholic, non-smoker presented with complete paraplegia involving the bladder and bowel. His clinical examination did not show any gibbus. He was started on empirical antitubercular therapy and then referred to us as he failed to show improvement. An MRI of the spine showed extensive long-segment continuous spinal involvement with epidural abscess. The patient was taken for surgery with posterior decompression and instrumentation which was proven to be TB. He received complete treatment of 1 year and had complete recovery of his sensory and incomplete recovery of motor of lower limbs (became a wheelchair ambulator) with regain of bowel and bladder control at the end of treatment.
UNASSIGNED: This case report revealed that long-segment continuous STB without extensive destruction is atypical presentation. The clinical findings of paraplegia helped us to evaluate and clinch the diagnosis on MRI. However, the prognosis of rapid paraplegia remains guarded and patient had partial recovery of motor so he could become wheel chair ambulator only.

BACKGROUND: Posterior internal fixation (PIF) is commonly used in the treatment of thoracolumbar fracture (TLF), but there is still no standard for the number of fixed segments. The objective of this meta-analysis was to evaluate the efficacy and safety of short segment (SS), intermediate segment (IS) and long segment (LS) in the fixation of TLF.
METHODS: Two authors independently searched through PubMed, Embase, Cochrane Library and Web of Science for studies of thoracolumbar fracture treated by posterior internal fixation, which were published until the end of April 2021. The Aggregate Data Drug Information System (ADDIS) software was used for data evaluation according to the Markov chain Monte Carlo (MCMC) method based on the Bayesian theorem.
RESULTS: Nineteen trials evaluating a total of 970 patients were enrolled in these studies, of which 340 in the SS group, 429 in the IS group and 201 in the LS group. For anterior vertebral height ratio (AVHR), IS had the highest AVHR, LS had the second highest AVHR. IS also ranked first in reducing visual analogue scale (VAS), SS ranked second. For sagittal Cobb\'s angle (SCA), LS had the lowest SCA and IS had the second lowest SCA. In terms of adverse events, IS had the lowest implant failure rate and LS had the second lowest implant failure rate.
CONCLUSIONS: IS may be the most desirable treatment option for TLF in reducing SCA, implant failure rate, VAS, and improving AVHR. However, more randomized controlled trials are needed to verify these results.

UNASSIGNED: Rosai-Dorfman disease is a rare, histiocytic lymphoproliferative disease of unknown etiology. It manifests mainly as painless cervical lymphadenopathy, with very few cases reported extranodal involvement in the central nervous system. Isolated spinal Rosai-Dorfman disease is sporadic.
METHODS: This case report documents a rare instance of an isolated long-segment spinal Rosai-Dorfman disease (C4-D6) along with the review of relevant literature. A 33-year male presented with progressive quadriparesis and urinary retention. A magnetic resonance scan (MRI) revealed a long segment epidural lesion from C4-D6 levels that led to the displacement of the cord. A core biopsy of the spinal tumor revealed characteristic histiocytic emperipolesis and confirmational immunocytohistochemistry markers, confirming the diagnosis. Surgical resection and laminoplasty were performed.
UNASSIGNED: The following histopathology and immunocytohistochemistry findings showed the presence of histiocytes positive for S100 and CD68 positive. Therefore, it was diagnosed to be a case of Rosai-Dorfman disease. The patient had a smooth postoperative recovery and displayed marked motor improvement in the ensuing days. This is a rare case that posed an intriguing challenge to approach.
CONCLUSIONS: To our knowledge, we have encountered one of the most prolonged segmental lesions in isolated spinal Rosai-Dorfman diseases, where surgical management (surgical resection and laminoplasty) has proven to bring about remarkable improvement.

OBJECTIVE: Buccal mucosa graft (BMG) is long used as favoured substitute by most reconstructive surgeons for substitution urethroplasty (SU). Though inner preputial skin graft (IPG) was described even earlier than BMG, its usage lately has fallen out of favour. The aim of the study was to evaluate the outcome of a SU with IPG from a tertiary care centre.
METHODS: A retrospective analysis of prospectively maintained clinical data was conducted at our tertiary care centre enrolling 80 patients with anterior urethral stricture from January 2015 to January 2018. Patients were evaluated for the aetiology, length and site of the urethral stricture. All patients underwent dorsolateral SU with IPG. Post-operative assessment including uroflowmetry and sexual outcomes using IIEF and MSHQ-EJD questionnaires was done at 3 weeks, 3 months, 12 months and half-yearly thereafter. Success was defined by the stable maximum urinary flow value > 14 ml/s or urethral calibration with 16 French Foley catheter.
RESULTS: Mean age of patients was 40 years (18-69). The most common aetiology was post-instrumentation (65%) and 60% had stricture at penobulbar site. Mean stricture length was 65 mm. At a mean follow-up of 48 months (range 30-66 months), successful outcomes were seen in 69/80 (87%). Patients with failure were managed with optical internal urethrotomy (OIU). Uroflowmetry and obstructive symptoms significantly improved and sexual function remained unaffected using IPG for SU.
CONCLUSIONS: Preputial graft is a tissue familiar to the urologist, located very close to the surgical field, easily harvested and operated under regional anaesthesia. Overall success outcomes are acceptable to BMG urethroplasty.

OBJECTIVE: Urethral stricture is a highly prevalent disease and has a continued rising incidence. The global burden of disease keeps rising as there are significant rates of recurrence with the existing management options with the need for additional repeat procedures. Moreover, the existing treatment options are associated with significant morbidity in the patient. Long segment urethral strictures are most commonly managed by augmentation urethroplasty. We explored the potential for the application of an acellular tissue engineered bovine pericardial patch in augmentation urethroplasty in a series of our patients suffering from urethral stricture disease. The decreased morbidity due to the avoidance of harvest of buccal mucosa, decreased operative time and satisfactory postoperative results make it a promising option for augmentation urethroplasty.
METHODS: Nine patients with long segment anterior urethral strictures (involving penile and/or bulbar urethra and stricture length >4 cm) were included in the study after proper informed consent was obtained. Acellular tissue engineered indigenous bovine pericardial patch was used for urethroplasty using dorsal onlay technique.
RESULTS: A total of nine patients underwent tissue engineered indigenous pericardial patch urethroplasty for long segment urethral strictures, mostly catheter injury induced or associated with balanitis xerotica obliterans. Median follow-up was 8 months (range: 2-12 months). Out of nine patients, eight (88.9%) were classified as success and one (11.1%) was classified as failure.
CONCLUSIONS: Our study brings a product of tissue engineering, already being used in the cardiovascular surgery domain, into the urological surgery operating room with satisfactory results achieved using standard operating techniques of one stage urethroplasty.

The objective of this mini-review is to provide insights on the advances in the understanding of the genetic variants associated with different manifestations of Hirschsprung disease, which may present with a range of denervation from a short segment of colon to total colonic and small bowel or extensive aganglionosis. A recent article in this journal documented potential gene variants involved in long-segment Hirschsprung disease in 23 patients. Gene variants were identified using a 31-gene panel of genes related to Hirschsprung disease or enteric neural crest cell development, as previously reported in the literature. The study identified potentially harmful variants in eight genes across 13 patients, with a detection rate of 56.5% (13/23 patients). Five patients had pathologic variants in RET, NRG1, and L1CAM, and the remainder were considered variants of unknown significance. The authors attempted prenatal diagnosis of Hirschsprung disease utilizing an amniocentesis sample obtained for advanced maternal age in a family with a known deleterious RET mutation, manifested in the father (long-segment Hirschsprung disease) and older daughter (total colonic aganglionosis). The fetus had the same RET variant but, after several years of follow-up, has not developed any symptoms of Hirschsprung disease, supporting the conclusion that this RET mutation is an autosomal dominant gene with incomplete penetrance. This experience suggests that genetic counseling is appropriate to carefully assess the justification of prenatal testing, especially, when the phenotype of long-segment Hirschsprung disease is so variable and the disease is potentially curable with surgery.

Objective: To observe the biomechanical characteristics of bridge-link type combined internal fixation system (BCFS) with mixed-rod in the treatment of long segmental comminuted fracture of femoral shaft. Methods: A total of 16 models of long comminuted segment fracture of femoral shaft with mixed-rod and double-rod were made (8 each structure), and divided into the mixed-rod group and the double-rod group. The axial compression experiment and the radial torsion experiment were carried out on each of the mixed rod group and the double rod group. Four models were randomly selected from the two groups for axial compression experiment and radial torsion experiment, respectively. The changes of axial compression yield load and displacement, and also radial torsional yield load and angle were observed in two groups. At the same time, the maximum load was recorded when the curve had a break point or in a horizontal state, and then calculated the yield load. The data of normal distribution were compared with one-sample t test. Results: When the two groups were compressed axially at the same speed, as the axial compression yield load increased gradually, the longitudinal compression displacement of the mixed-rod group was obviously smaller than that in the double-rod group; and the maximum axial compression yield load of the mixed-rod group was greater than that in the double-rod group ((7 517±4)N vs (2 290±4) N, t=1 848.071, P=0.000). When the two groups were twisted radially at the same speed,as radial torsional yield load increased gradually, the radial torsion angle of the mixed-rod group was smaller than that in the double-rod group; and the maximum radial torsional yield load of the mixed-rod group was greater than that in the double-rod group ((61.3±2.0) Nm vs (24.4±2.2)Nm, t=25.201, P=0.000). Conclusions: For the long segment comminuted fracture of femoral shaft, the mixed-rod of BCFS can fix fracture more strongly than double-rod in longitudinal direction and maintain the stability of fracture end. The transverse torsion resistance is better than that of the double-rod, and the fatigue resistance is strong.
目的： 观察桥接组合式内固定系统（BCFS）混棒结构治疗股骨干长节段粉粹性骨折的生物力学特点。 方法： 制作相同人体股骨干长节段粉碎性骨折混棒结构及双棒结构模型各8根，共16根，并随机分为混棒组和双棒组。混棒组与双棒组各随机选取4根分别行轴向压缩实验及径向扭转实验。观察两组的轴向压缩屈服载荷与位移情况，以及径向扭转屈服载荷与角度变化；同时记录各组曲线开始出现折点，或趋于水平状态时的屈服载荷即最大屈服载荷。正态分布数据比较采用两独立样本设计t检验。 结果： 当两组以相同速度轴向压缩时，随着轴向压缩屈服载荷逐渐增大，混棒组纵向压缩位移明显小于双棒组，并且混棒组最大轴向压缩屈服载荷大于双棒组，差异有统计学意义[（7 517±4）N比（2 290±4）N，t=1 848.071，P=0.000]。当两组以相同速度径向扭转时，随着径向扭转屈服载荷逐渐增大，混棒组径向扭转角度小于双棒组，并且混棒组最大径向扭转屈服载荷大于双棒组，差异有统计学意义[（61.3±2.0）N·m比（24.4±2.2）N·m，t=25.201，P=0.000]。 结论： 对于股骨干长节段粉粹性骨折，桥接系统混棒结构主要在纵向上比双棒结构更能坚强固定骨折，维持骨折端的稳定性；而横向上抗扭转力也优于双棒结构，并且抗疲劳性能强。.

UNASSIGNED: Systematic literature review.
UNASSIGNED: Many studies have provided evidence that short-segment posterior fixation (SSPF-1 level above and 1 below) with screws at the fracture level (SFL) are enough to achieve stability in some injury patterns, such as burst fractures, avoiding the need for circumferential reconstruction and long-segment instrumented fusion (LSIF-at least 2 levels above and 2 below). Given the potential benefits of avoiding unnecessary fusion in mobile healthy spinal segments, we performed a systematic review of biomechanical studies comparing different spinal reconstruction techniques for fractures of the thoracolumbar spine.
UNASSIGNED: A systematic literature review was performed in the PubMed and OVID databases of biomechanical studies comparing biomechanical differences between techniques of spine reconstructions.
UNASSIGNED: Eight studies were included and evaluated. Five of 6 studies reported stiffness improvement with SSPF and SFL, even comparable to circumferential fusion for a burst fracture. Two studies reported that LSPF has higher stiffness and restricts range of motion better than SSPF, but inclusion of screws in the fracture level is similar to LSPF (1 study). Finally, although SSPF is less stiff than anterior reconstruction, adding a SFL in SSPF results in similar stiffness than circumferential fusion for unstable burst fractures.
UNASSIGNED: Biomechanical studies analyzed generally suggested that SFL in SSPF may improve construction stiffness, and can even be compared with long-segment fixation or circumferential reconstruction in some scenarios. This construct option may be used to enhance stiffness in selected injury patterns, avoiding the needs of an additional anterior approach.

OBJECTIVE: The thoracolumbar (TL) area marks the transition of the rigid thoracic spine into the mobile lumbar spine, and it is considered to be the weakest part of the spine. This study was designed to develop a finite element (FE) model of the TL junction (T9-L3) to provide data that could help the clinician and researcher to answer the question of whether short-segment posterior fixation is sufficient for biomechanical performance. In addition, the aim was to examine whether long-segment posterior fixation carries a greater risk of the development of adjacent segment disease.
METHODS: This was a biomechanical finite element model analysis. FE analysis of the spine was conducted with posterior instrumentation under multidirectional loading conditions in order to evaluate the kinematics of the instrumented lumbar spine, as well as stresses in the posterior spinal instrumentation. We analyzed the following: 1) the range of motion of the T9-L3 region; and 2) the von Mises stress nephograms of the pedicle screws, rods, vertebrae, endplates, and intervertebral discs of 2 fixation FE models.
RESULTS: Long-segment stabilization was found to be beneficial in terms of reducing total stress on the spine. However, it is possible to reduce the stress on the system by incorporating the spinal fracture into the stabilization system. Therefore, short-segment stabilization is sufficient to create a safe and robust stabilization system and to maintain neighboring intact vertebrae.
CONCLUSIONS: Short-segment posterior fixation is sufficient to stabilize fractures at the TL junction, where the spinal fracture is included in the stabilization system.