Pathoanatomic Findings Research Articles

Meniere's Disease Patients with Distinct Sac Pathoanatomic Findings React Differently to Endolymphatic Duct Blockage.

To explore the possible difference in response to endolymphatic duct blockage (EDB) treatment in patients with Meniere's disease (MD) with distinct pathoanatomic characteristics of the sac. In a total of 24 patients with MD receiving EDB treatment, the dynamics of the vertigo attack, hearing, vestibular function, and endolymph hydrops (EH) before surgery and 40 months following surgery in patients with normoplastic extraosseous portion of endolymphatic sac (eES) were compared with that in patients with atrophic eES. A higher prevalence of complete vertigo control, better cochlear and vestibular function, and lower endolymph to vestibule-volume ratio were found in patients with normoplastic eES than in those with atrophic eES. Moreover, the reversal of EH was found in a total of six patients in normoplastic eES group, but no reversal of EH was detected in the atrophic eES group after surgery. A difference in response to EDB treatment was shown in the MD patients with normoplastic eES and those with atrophic eES; the reversal of EH was found in the normoplastic eES group, but not in the atrophic eES group after surgery, suggesting two distinct pathologies in the eESs may underlie the pathogenesis of EH in two subgroups of MD patients. 4 Laryngoscope, 133:2761-2769, 2023.

Neoadjuvant chemotherapy (NACT) and HER2 double inhibition including biosimilar trastuzumab (ONTRUZANT) for HER2-positive early breast cancer (EBC): Population-based real world data from the Danish Breast Cancer Group (DBCG).

577 Background: Increasingly, HER2-positive early breast cancer (EBC) is treated by NACT combined with trastuzumab and pertuzumab followed by surgery. Ontruzant is registered as a biosimilar trastuzumab based on the totality of evidence including a randomized phase III study of NACT+Herceptin versus NACT+Ontruzant demonstrating similar pCR-rates (Pivot et al. J Clin Oncol 2018;36:968). However, no data exist for the efficacy of the combination of NACT with pertuzumab+Ontruzant (p+O). This investigator-initiated study was conducted to assess real world efficacy in HER2-positive EBC patients treated with NACT+p+O based on data from DBCG. DBCG has since 1977 provided guidelines for treatment of breast cancer and collected data from Danish hospital departments of surgery, pathology, and oncology prospectively on NACT, date and type of surgery and patho-anatomic findings. Methods: From the DBCG database, information was extracted for consecutive patients with unilateral early HER2-positive breast cancer registered to have received NACT+p+O from September 1, 2018 to August 31, 2019. pCR was defined as absence of residual invasive tumor in the breast and axillary lymph nodes (ypT0/Tis ypN0(i-)). Results: 215 patients received NACT+p+O. Median age was 54.8 years (range 24-81). NACT used, in combination with concurrent p+O, was cyclophosphamide+epirubicin followed by paclitaxel (62% on 6 cycles and 35% on 8 cycles) or other chemotherapy followed by paclitaxel (3%). Overall, 56% of patients achieved a pCR (Table). 68% of node-positive patients before receiving NACT+p+O had tumor-free axillary nodes after completing NACT+p+O. Conclusions: Real-world data from a nationwide population based study demonstrated a pCR-rate with NACT+p+O comparable to that seen in clinical studies with NACT+p+Herceptin (Chen et al. BMC Cancer 2019;19:973). pCR-rate was highly dependent on estrogen receptor (ER)-status and malignancy grade but not on clinical nodal status and tumor size. 68% of patients with cN+ converted to ypN0(i-). [Table: see text]

The Health and Welfare of Pigs from the Perspective of Post Mortem Findings in Slaughterhouses.

Simple SummaryThe health and welfare of pigs was evaluated on the basis of the data on patho-anatomic findings obtained during the veterinary examination of pigs slaughtered in slaughterhouses in the Czech Republic in the period from 2010 to 2017. The highest numbers of lesions in organs were found in lungs (finisher pigs 41%, sows 24% and piglets 52%), kidneys (finisher pigs 14%, sows 32% and piglets 15%) and liver (finisher pigs 12%, sows 18% and piglets 19%). The character of most findings was chronic, however, acute findings were also detected. Parasitic lesions were found mainly in finisher pigs (finisher pigs 4%, sows 1% and piglets 1%). Incidence of traumatic lesions (finisher pigs 0.08%, sows 0.14% and piglets 0.15%) was far below the frequency of other findings. Overall, post mortem findings in the slaughterhouses varied among pig categories (p < 0.001). In order to decrease the number of lesions detected post mortem, it is essential to improve health and welfare of pigs on farms and in transit.The health and welfare of pigs was evaluated on the basis of the data on patho-anatomic findings obtained during the veterinary examination of pigs slaughtered in slaughterhouses in the Czech Republic in the period from 2010 to 2017. High numbers of lesions in organs found especially in lungs (finisher pigs 41%, sows 24% and piglets 52%), kidneys (finisher pigs 14%, sows 32% and piglets 15%) and liver (finisher pigs 12%, sows 18% and piglets 19 %) indicate impaired health and welfare of pigs transported for slaughter. The differences in the number of findings between finisher pigs, sows and piglets were statistically significant (p < 0.001). The character of most findings was chronic, which document health and welfare problems occurring on farms as a result of the current pig husbandry. However, acute findings were also detected and indicated processes occurring shortly before and during transport to the slaughterhouse. An important finding is the incidence of parasitic lesions in the liver in finisher pigs (finisher pigs 4%, sows 1% and piglets 1%) that documents persistent occurrence of parasitic invasions on pig farms. Findings of traumatic lesions on limbs in sows and piglets (finisher pigs 0.08%, sows 0.14% and piglets 0.15%) are far below the frequency of the findings in organs; however, their incidence should be further reduced by adjusting the technology of housing, transport and handling. In conclusion, the level of health and related welfare of pigs based on the assessment of post mortem findings in the slaughterhouses vary. Overall, the worst situation is in piglets, followed by sows and the best evaluated are finisher pigs. Post mortem inspection revealed significant numbers of patho-anatomic changes even in pigs considered fit to be transported to the slaughterhouse and slaughtered for human consumption. It is clear that there is still a considerable space for improving the level of health and welfare of the individual categories of pigs.

Type E2 glenoid bone loss orientation and management with augmented implants

The purpose of this study was 2-fold: (1) to quantify type E2 bone loss orientation and its association with rotator cuff fatty infiltration and (2) to examine reverse baseplate designs used to manage type E2 glenoids. Computed tomography scans of 40 patients with type E2 glenoids were examined for pathoanatomic features and erosion orientation. The rotator cuff fatty infiltration grade was compared with the erosion orientation angle. To compare reconstructive options in light of the pathoanatomic findings, virtual implantation of 4 glenoid baseplate designs (standard, half wedge, full wedge, and patient-matched) was conducted to determine the volume of bone removal for seating and impingement-free range of motion. The mean type E2 erosion orientation angle was 47° ± 17° from the 0° superoinferior glenoid axis, resulting in the average erosion being located in the posterosuperior quadrant directed towardthe 10:30 clock-face position. The type E2 neoglenoid, on average, involved 67% of the total glenoid surface (total surface area, 946 ± 209 mm2; neoglenoid surface area, 636 ± 247 mm2). The patient-matched baseplate design resulted in significantly (P ≤ .01) less bone removal (200 ± 297 mm3) for implantation, followed by the full-wedge design (1228 ± 753 mm3), half-wedge design (1763 ± 969 mm3), and standard (non-augmented) design (4009 ± 1210 mm3). We noted a marked difference in erosion orientation towarda more superior direction as the subscapularis fatty infiltration grade increased from grade 3 to grade 4 (P < .001). The average type E2 erosion orientation was directed towardthe 10:30 clock-face position in the posterosuperior glenoid quadrant. This orientation resulted in the patient-matched glenoid augmentation requiring the least amount of bone removal for seating, followed by the full-wedge, half-wedge, and standard designs. Implant selection also substantially affected computationally derived range of motion in external rotation, flexion, extension, and adduction.

Patho-anatomic findings in finisher pigs, sows, and piglets detected during veterinary slaughterhouse inspection

The number of patho-anatomic findings detected during the veterinary examination of slaughtered pigs was analyzed. From 2010 to 2017, a total of 20 550 072 finisher pigs, 484 710 sows, and 94 279 piglets were slaughtered in the Czech Republic. By comparing the overall amount of patho-anatomic findings expressed as an index of the ratio of the number of findings to the number of slaughtered animals, the highest incidence of patho-anatomic findings was determined in piglets (index 1.52), followed by sows (index 1.23), and the lowest in finisher pigs (index 0.81). The most numerous was the occurrence of chronic findings (finisher pigs: index 0.724%, sows: 0.926, piglets: 0.877). Furthermore, a high frequency of acute findings was found (finisher pigs: index 0.037, sows: 0.207, piglets: 0.373). The findings of traumatic and parasitic nature were significantly (P &lt; 0.001) lower than those of chronic and acute character. The differences in the total number of findings and in the number of findings by the type of damage were significant (P &lt; 0.001) between the individual pig categories. The results show that the worst health condition was observed in piglets, followed by sows, and the best in finisher pigs. Furthermore, the number of findings was significantly decreasing in finisher pigs (r = -0.7143, P = 0.047); no significant change was found in sows (r = -0.643, P = 0.086); however, it was significantly increasing in piglets (r = +0.857, P = 0.007). Therefore, there is a particular need for health improvement in piglets and also in sows.

Comparison of the frequency of patho-anatomic findings in laying hens with findings in broiler chickens and turkeys detected during post-mortem veterinary inspection

We have investigated the health condition of laying hens on the basis of patho-anatomic findings obtained during the veterinary inspection after slaughter. To assess the severity of the health condition of laying hens, we compared the incidence of findings with the findings in broiler chickens and turkeys. In the period from 2010 to 2017, 17,346,183 laying hens, 887,994,167 broiler chickens, and 919,843 turkeys were transported from Czech farms to slaughterhouses in the Czech Republic. The competent veterinary authority in the Czech Republic (the State Veterinary Administration) carried out a veterinary inspection of these birds slaughtered in the slaughterhouse and recorded the patho-anatomic findings. From the recorded data, the number of patho-anatomic findings (intravital origin) of the organs and parts of the bodies (liver, intestines, lungs, heart, spleen, genital tract, urinary system, CNS, skin, head, body, limbs, and whole animal) were analysed. We found increased patho-anatomic findings in the liver (26.76%) and limbs (6.03%) in hens, and also high numbers of findings in the category of total findings (1.57%) were detected. In liver findings, chronic findings (22.09%) were prevailing over the acute findings (4.67%), compared to broiler chickens (chronic 0.04%, acute 0.03%) and turkeys (chronic 1.90%, acute 0.00%). The differences between hens, broilers, and turkeys were significant (P < 0.001). The incidence of traumatic, chronic, and acute findings in hens (3.88%, 1.71%, and 0.44%, respectively) was higher (P < 0.001) in the case of the legs, compared with broiler chickens (0.02%, 0.08%, and 0.02%, respectively) and turkeys (0.27%, 0.23%, and 0.05%, respectively). The results show that the health condition of laying hens is significantly worse than in case of broiler chickens and turkeys, especially in the impact on the liver due to the likely effect of nutrition not corresponding to the intensity of the production of laying hens, and the impact on the limbs due in particular to traumatic changes caused by inappropriate transport handling and inadequate conditions (especially cage housing) of laying hens.

An Autopsy Study of Patho-Anatomic Findings and Inter-Related Conditions in Cases of Deaths Due to Compression of Neck

An Autopsy Study of Patho-Anatomic Findings and Inter-Related Conditions in Cases of Deaths Due to Compression of Neck

Distal Extent of Surgery for Acute Type A Aortic Dissection

Acute type A aortic dissection (TAAD) is a complex disease associated with extremely high morbidity and mortality for which we advocate a coordinated, protocol-driven system of care delivery that begins at patient diagnosis and continues throughout and beyond aortic reconstruction. Essential components of TAAD repair include prompt restoration of true lumen blood flow with obliteration of the false lumen flow, resection of the primary tear sites, restoration of valvular competency, and elimination of any organ malperfusion. This article focuses specifically on extent of repair of the aortic arch and explains our protocols regarding cannulation location and technique, cerebral and distal organ protection strategy, management of the brachiocephalic vessels, and extent of distal aortic reconstruction. We describe an operative strategy for TAAD repair that includes (1) continuous neurocerebral monitoring in all cases, (2) uninterrupted antegrade and/or retrograde cerebral perfusion (depending upon extent of arch repair) during open arch reconstruction, (3) aortic arch replacement technique with or without brachiocephalic vessel replacement using a custom trifurcate graft, and (4) descending aortic stabilization with or without the use of an elephant or frozen elephant trunk (distal stent graft). Our protocol for extent of aortic arch and brachiocephalic reconstruction has been standardized and is predicated on distinct pathoanatomic findings and/or cerebral malperfusion that are outlined.

Pathoanatomic Findings Associated With Duty-Related Cardiac Death in US Firefighters: A Case-Control Study.

BackgroundSudden cardiac death accounts for the greatest proportion of duty‐related deaths among US firefighters. Increased understanding of the pathoanatomic causes of sudden cardiac death and the risk associated with underlying cardiac pathologies is needed to develop evidence‐based screening recommendations.Methods and ResultsUsing autopsy data for duty‐related firefighter fatalities occurring between 1999 and 2014, this retrospective case–control study compared cardiac findings of male firefighters aged 18 to 65 years who died on duty of cardiac‐related causes with those who died of noncardiac trauma‐related causes. Data from 276 cardiac cases and 351 noncardiac trauma controls were analyzed. Among cardiac cases, the most prevalent (82%) underlying pathoanatomic substrate was comorbid coronary heart disease and cardiomegaly/left ventricular hypertrophy. Cardiac cases had a higher prevalence of cardiomegaly (heart weight >450 g), left ventricular hypertrophy (left ventricular wall thickness ≥1.2 cm), and severe coronary artery stenosis (≥75%) than trauma controls (all P<0.001). In multivariate analyses, heart weight >450 g, coronary artery stenosis ≥75%, and evidence of a prior myocardial infarction were strong independent predictors of cardiac death, with odds ratios of 6.1 (95% confidence interval, 3.6–10.4), 9.3 (95% confidence interval, 5.3–16.1), and 6.2 (95% confidence interval, 3.4–11.3), respectively.ConclusionsThe majority of cardiac fatalities had evidence of both coronary heart disease and increased heart mass, and each condition was independently associated with a markedly elevated risk of cardiac death. Targeted screening for coronary heart disease, increased heart mass, and evidence of prior myocardial infarction should be considered to reduce duty‐related cardiac deaths among firefighters.

Retrieving mode of action information by automated effect pattern analysis using the zebrafish embryo toxicity test

Acute type A aortic dissection (TAAD) is a complex disease associated with extremely high morbidity and mortality for which we advocate a coordinated, protocol-driven system of care delivery that begins at patient diagnosis and continues throughout and beyond aortic reconstruction. Essential components of TAAD repair include prompt restoration of true lumen blood flow with obliteration of the false lumen flow, resection of the primary tear sites, restoration of valvular competency, and elimination of any organ malperfusion. This article focuses specifically on extent of repair of the aortic arch and explains our protocols regarding cannulation location and technique, cerebral and distal organ protection strategy, management of the brachiocephalic vessels, and extent of distal aortic reconstruction. We describe an operative strategy for TAAD repair that includes (1) continuous neurocerebral monitoring in all cases, (2) uninterrupted antegrade and/or retrograde cerebral perfusion (depending upon extent of arch repair) during open arch reconstruction, (3) aortic arch replacement technique with or without brachiocephalic vessel replacement using a custom trifurcate graft, and (4) descending aortic stabilization with or without the use of an elephant or frozen elephant trunk (distal stent graft). Our protocol for extent of aortic arch and brachiocephalic reconstruction has been standardized and is predicated on distinct pathoanatomic findings and/or cerebral malperfusion that are outlined.

Traumatic Anterior Instability: Treatment Options for Initial Instability

Because of the lack of bony restraints and minimal articular contact, the glenohumeral joint can attain significant range of motion; however, this results in the propensity for instability. The most generic form of instability, traumatic anterior instability, reliably produces a series of pathoanatomic findings. While reliable, these findings contribute to the complexities of caring for patients after an initial instability event. Numerous studies have examined this issue and determined that careful consideration of patient factors can guide successful treatment, whether it be surgical or nonsurgical, after initial instability. Such forms of treatment have shown to provide a good functional outcome and decreases morbidity. To be able to provide successful treatment requires a thorough understanding of the pathoanatomic of an instability event and the intricacies of the evaluation of a patient after an initial instability event.

Pathoanatomic Findings and Treatment During Hypertrophic Obstructive Cardiomyopathy Surgery: The Role of Mitral Valve

To assess the role of the mitral valve apparatus (leaflets, chordae and papillary muscles, (PM)) in left ventricle outflow tract (LVOT) obstruction, and results of the surgical treatment for hypertrophic obstructive cardiomyopathy (HOCM). Twenty-eight consecutive patients (58±11years, 53% female) undergoing HOCM surgery from 2007 to 2016 at our institute were retrospectively reviewed. Endpoints included the involvement of the mitral valve in LVOT obstruction, mortality, and changes in clinical and echocardiographic characteristics after HOCM surgery. Secondary chordae tendineae tractioning the anterior mitral leaflet to the interventricular septum, and systolic anterior motion were detected in 78% of the patients. Anomalous, hypertrophied, and fused PM with muscularis trabeculae hypertrophy were found in 50%, 25%, and 35% of the patients, respectively. Four patients had posterior leaflet redundancy. Secondary chordae (92%), PM, and muscularis trabeculae resection (71%), and PM splitting and elongation (28%) were added variably to septal myectomy (100%). Nine procedures (32%) on mitral valve leaflets were performed, involving six posterior and three anterior mitral leaflets. Long-term follow-up was 4±2.8years. There was no hospital mortality, and NYHA was reduced from 3±0.5 to 1±0.7 (p<0.0001), the LVOT gradient from 88±35 to 20±18mmHg (p<0.0001), mitral valve regurgitation from grade 3±1 to 1±0.7 (p<0.0001), and septum thickness from 18±3 to 14±2mm (p<0.0001). The mitral valve apparatus contributes with all its components variably to LVOT dynamic obstruction thus surgical correction in addition to extended myectomy is recommended to achieve the best outcome.

A COMBINED TREATMENT APPROACH EMPHASIZING IMPAIRMENT-BASED MANUAL THERAPY AND EXERCISE FOR HIP-RELATED COMPENSATORY INJURY IN ELITE ATHLETES: A CASE SERIES.

Athletes experiencing hip, groin, and low back pain often exhibit similar clinical characteristics. Individuals with hip, groin and low back pain may have the presence of multiple concurrent pathoanatomical diagnoses. Regardless, similar regional characteristics and dysfunction may contribute to the patient's chief complaint, potentially creating a sub-group of individuals that may be defined by lumbopelvic and hip mobility limitations, motor control impairments, and other shared clinical findings. The purpose of this case series is to describe the conservative management of elite athletes, within the identified aforementioned sub-group, that emphasized regional manual therapy interventions, and therapeutic exercise designed to improve lumbopelvic and hip mobility, stability and motor control. Five elite athletes were clinically diagnosed by a physical therapist with primary pathologies including adductor-related groin pain (ARGP), femoral acetabular impingement (FAI) with acetabular labral lesion and acute, mechanical low back pain (LBP). Similar subjective, objective findings and overall clinical profiles were identified among all subjects. Common findings aside from the chief complaint included, but were not limited to, decreased hip range of motion (ROM), impaired lumbopelvic motor control and strength, lumbar hypomobility in at least one segment, and a positive hip flexion-adduction-internal rotation (FADIR) special test. A three-phase impairment-based physical therapy program was implemented to resolve the primary complaints and return the subjects to their desired level of function. Acute phase rehabilitation consisted of manual therapy and fundamental motor control exercises. Progression to the sub-acute and terminal phases was based on improved subjective pain reports and progress with functional impairments. As the subjects progress through the rehabilitation phases, the delivery of physical therapy interventions were defined by decreased manual therapies and an increased emphasis and priority on graded exercise. Significant reductions in reported pain (>2 points Numeric Pain Rating Scale), improved reported function via functional outcome measures (Hip and Groin Outcome Score), and continued participation in sport occurred in all five cases without the need for surgical intervention. The athletes described in this case series make up a common clinical sub-group defined by hip and lumbopelvic mobility restrictions, lumbopelvic and lower extremity motor control impairments and potentially other shared clinical findings. Despite differences in pathoanatomic findings, similar objective findings were identified and similar treatment plans were applied, potentially affecting the movement system as a whole. Subjects were conservatively managed allowing continued participation in sport within their competitive seasons. Comprehensive conservative treatment of the athletes with shared impairments, as described in this case series, may be of clinical importance when managing athletes with hip, groin, and low back pain. Therapy, Level 4, Case Series.

Outcomes After Arthroscopic Pancapsular Capsulorrhaphy With Suture Anchors for the Treatment of Multidirectional Glenohumeral Instability in Athletes

Background: Outcomes after arthroscopic pancapsular capsulorrhaphy (APC) with suture anchors for multidirectional instability (MDI) of the shoulder are not widely reported. Purpose: To compare intraoperative findings and midterm outcomes of APC with suture anchors for MDI between female and male athletes and between a classic, atraumatic onset versus clinical onset of MDI after a traumatic event. Study Design: Cohort study; Level of evidence, 3. Methods: Patients who underwent APC with suture anchors for MDI and were at least 2 years out from surgery were included. Data were prospectively collected and retrospectively reviewed and included the onset of MDI, intraoperative pathoanatomic findings, level of sports participation, and patient satisfaction as well as the American Shoulder and Elbow Surgeons (ASES), Single Assessment Numeric Evaluation (SANE), Quick Disabilities of the Arm, Shoulder and Hand (QuickDASH), and Short Form–12 Physical Component Summary (SF-12 PCS) scores. Information regarding shoulder instability and return to sport was collected, and Kaplan-Meier survivorship analysis was performed. Results: Forty-one patients (45 shoulders; 25 male, 20 female), consecutively treated between October 2006 and January 2013, were included. The onset of MDI was atraumatic in 22 shoulders and traumatic in 23 shoulders. At surgery, 29 of 45 (64.4%) had labral detachment. Seven shoulders (16.7%) experienced instability episodes postoperatively, and 3 of these underwent revision surgery. The mean follow-up was 3.3 years (range, 2.0-6.6 years). All subjective outcome scores improved significantly from preoperative levels (P < .005). At final follow-up, the mean ASES score was 92.0, and 76.7% (23/30) indicated that they had returned to sports participation equal to or slightly below their preinjury level. Kaplan-Meier analysis showed a survivorship rate of 87% at 3 years. Male patients were 2.3 times more likely to have a traumatic onset of instability (68% vs 30%, respectively; P = .017) and were 2.1 times more likely to have concomitant lesions (84% vs 40%, respectively; P = .004) than female patients. Furthermore, male patients demonstrated a higher mean postoperative ASES score than female patients (97.0 ± 4.7 vs 85.5 ± 16.4, respectively; P = .023). Female patients were 6.9 times more likely to undergo an additional rotator interval closure (RIC) procedure (58% vs 4.7%, respectively; P < .001) and to experience postoperative subluxations (40% vs 22%, respectively; P = .035) than male patients. A traumatic onset of MDI was associated with a higher mean postoperative ASES score (96.4 ± 6.9 vs 87.0 ± 15.7, respectively; P = .048), higher median satisfaction score (10 vs 9, respectively; P = .029), and higher return-to-sport rate (83% vs 44%, respectively; P = .030) than an atraumatic onset. Conclusion: APC with suture anchors can be an effective and safe treatment for patients with MDI. Labral tears were commonly found, even in patients with a classic, atraumatic onset. Male patients and patients with a traumatic onset of MDI had more favorable outcomes. Female patients may be more challenging to treat as they were more likely to undergo an additional RIC procedure and experience postoperative subluxations.

Aetiology of the Lumbar Spinal Stenosis in Black Africans: Intraoperative Observations

Background & Aim: Lumbar spinal stenosis (LSS) is a common condition. This congenital or acquired stenosis has multiple etiologies. The goal of this study was to describe intraoperative pathoanatomic findings of lumbar spinal stenosis observed within a black African population. Methods & Materials/Patients: It was a retrospective and descriptive study performed at Departmental Teaching Hospital of Borgou in Republic of Benin (West Africa) from January 2008 to June 2014. All black African patients who underwent surgery for LSS were included. Intra operative aetiologies were recorded. Patients with extruded lumbar discopathy were excluded. Results: During study period, 199 patients were selected. It was 109 men (54.8%) and 90 women (42.2%). Patients mean age was 53.2 years ± 10.96, mean weight 77.11kg ± 14.37 and mean height 171 cm ± 8.64cm. The LSS was acquired in 157 patients (78.9%), congenital in 29 (14.6%) and in 13 cases (6.5%) it was mixed origin. A hypertrophyof ligamentum flavum was observed in 101 patients (50.8%), zygapophyseal joint hypertrophy in 15 cases (7.2%) and in 74 cases (37.2%) the two factors were associated. A disc bulge were observed in 9 cases (4.5%) Conclusion: The ligamentum flavum hypertrophy is the main cause of LSS in black Africans. Studies to determine the mechanism of hypertrophy will be useful in order to initiate preventive treatment.

Fetal Ventricular Hypertrabeculation/Noncompaction: Clinical Presentation, Genetics, Associated Cardiac and Extracardiac Abnormalities and Outcome.

Left ventricular hypertrabeculation/noncompaction (LVHT) is a cardiac abnormality of unknown etiology. Aim of the review was to summarize the current knowledge about fetal LVHT, including clinical presentation, associated cardiac and extracardiac abnormalities and outcome. In 88 cases, LVHT was diagnosed by fetal echocardiography. In 36 %, no additional cardiac abnormalities were reported; in the remaining 64 %, one or more cardiac abnormalities were reported. Eight cases died prenatally, 17 were electively terminated, and 24 patients died after birth. Six patients were lost to follow-up, and 33 patients are alive at a mean age of 26 months. Surviving cases presented less frequently with fetal hydrops (13 vs. 62 %, p = 0.0004), complete heart block (27 vs. 78 %, p = 0.0076), more than three associated cardiac abnormalities (9 vs. 47 %, p = 0.0008) and more frequently with isolated LVHT (52 vs. 19 %, p = 0.009) than cases who died. Of the surviving patients, 16 received pharmacotherapy, three received pacemakers, eight underwent surgical procedures and four underwent heart transplantation. Postnatal regression of left ventricular hypertrophy and development of LVHT was found in four cases, improvement in cardiac function in two, and regression of right VHT in two. At autopsy, endocardial fibrosis was the most frequent abnormality in 92 %. Thirty-eight percentage of cases with fetal LVHT survived. Fetal and postnatal echocardiographic findings challenge the "embryonic pathogenetic" hypothesis of LVHT. Furthermore, fetal pathoanatomic findings like endocardial fibrosis might play a role in clarifying the still unsolved pathogenesis of LVHT.

Therapeutic step-up strategy for management of hereditary pancreatitis in children

Background/PurposeVarious different regimes exist for the treatment of hereditary pancreatitis in childhood. Here, we propose a therapeutic pathway with emphasis on endoscopic and surgical procedures. MethodsFrom 2006 to 2013, 12 patients with a diagnosis of hereditary pancreatitis were prospectively included in a therapeutic step-up schema. The treatment outcome was evaluated and correlated to aetiological factors and pathoanatomic findings. ResultsAfter diagnostic work-up (laboratory data, ultrasound examination, magnetic resonance cholangiopancreatography and genetic testing), all 12 patients underwent early endoscopic retrograde cholangiopancreatography (ERCP), which was successfully performed in ten children. Obstructive pancreatitis was found in eight children, and required sphincterotomy, dilation and stenting for 12months. In two children with unsuccessful ERCP, open surgical drainage procedures were performed. After a mean follow-up of 32months all children are free of recurrence of pancreatitis without any impairment of everyday activities. ConclusionsFor children with hereditary pancreatitis, a therapeutic step plan with early ERCP and open surgical drainage procedures in case of impossible or insufficient endoscopic treatment prevents recurring pancreatitis and offers a normal quality of life without any major complications.

Pontocerebellar hypoplasia.

Pontocerebellar hypoplasia (PCH) is a clinically and genetically heterogeneous group of autosomal recessively inherited neurodevelopmental disorders. Following the rapidly increasing number of genes identified in different subtypes, the clinical spectrum has been broadened to completely different neurological phenotypes. In this review we will address the clinical picture, neuroradiological, pathoanatomic, and genetic findings in the currently known PCH subtypes.

An Epidemiological and Patho- Anatomic Profile of 246 Cases of Hanging -A Cross Sectional Study

This cross sectional study was done over a period of 10 years to study the epidemiological and Patho-anatomic findings in cases of death due to hanging. A total of 246 cases were studied and findings were as follows, victims aged 21–30 years constituted largest group 44.3% (Cases),56.09% (cases) were males, 54.47% (Cases) were married, place preferred for suicidal hanging were indoor in 99.59%(cases). soft ligature material used in 63.41%(cases). The Patho anatomic findings were, ligature material was present in 98.78%, it was above the level of thyroid cartilage in 75.72% cases, protursion of tongue was seen in 25.64% cases, salivary discharge in 30% of cases. No soft tissue hemorrhage, intimal tears of carotid artery, neither fracture of hyoid bone and thyroid cartilage were seen in these 246 cases.

Combined Posterior Osseous Bankart Lesion and Posterior Humeral Avulsion of the Glenohumeral Ligaments

The pathoanatomy of anterior shoulder dislocations has been well documented. Perthes1 described an anterior labral lesion as early as 1906, and Bankart2 wrote his classic article on recurrent anterior dislocations more than seventy years ago. Until recently, much less had been written about posterior shoulder instability; to our knowledge, the first case series was reported by Reeves3 in 1963. Recurrent posterior glenohumeral joint instability is far less common than anterior instability, accounting for approximately 2% to 10% of all cases of shoulder instability4. Pathoanatomic findings in patients with unidirectional posterior instability include a posterior Bankart lesion, posterior humeral avulsion of the glenohumeral ligament (PHAGL), and various combinations of the two5,6. In their article on humeral avulsion of the glenohumeral ligament (HAGL), Bui-Mansfield et al.7 defined the floating posterior inferior glenohumeral ligament (floating PIGHL) as a combined posterior Bankart lesion and PHAGL. Multiple cases of this lesion have been reported in the literature7-11. To our knowledge, there has been no reported case of the specific combination of a posterior osseous Bankart lesion and a PHAGL injury. We present a case of recurrent posterior instability associated with a combined PHAGL injury and posterior osseous Bankart lesion. We also describe four pathoanatomic subtypes of floating PIGHL lesions. The patient was informed that data concerning his case would be submitted for publication, and he provided consent. A twenty-three-year-old left-hand-dominant man fell while snowboarding three years prior to presentation and sustained a left shoulder dislocation. He was not certain about the direction of the dislocation. He was able to relocate the shoulder on the mountain with the help of friends and the ski patrol. Initially, he was managed nonoperatively with a sling and physical therapy as well as a progressive return …