Congenital dislocation of the patella

Purpose Congenital dislocation of the patella is permanent and manually irreducible, and it manifests immediately after birth with flexion contracture of the knee, genu valgus, external tibial torsion and foot deformity. We retrospectively reviewed the results of operative treatment of seven knees in six patients with congenital dislocation of the patella. Methods The age of the six patients at diagnosis ranged from 8 days to 3.6 years, with an average of 1.3 years, and their age at the time of operation ranged from 0.6 to 3.9 years, with an average of 2.1 years. Serial casting and/or a brace was attempted before surgery in five of seven knees, leading to improvement in the flexion contracture of the knee. All knees were treated operatively in combination with lateral release, medial plication, V-Y lengthening of the quadriceps, medial transfer of the lateral patellar tendon and posterior release of the knee. Results Although these deformities were noticed at birth in all seven knees, diagnosis was delayed in three knees due to the low suspicion of the disease and invisible patellae on radiographs. Ultrasonography confirmed the diagnosis of dislocation. The patella was centered in the groove of the femoral condyle after surgery in all knees, but subluxation of the knee with flexion was observed in one knee in which the operation was performed at 3.9 years. Genu valgus and external tibial torsion improved after surgery in all knees. The operated knee was mobile in all cases, with less than 10° flexion contracture of the knee. Flexion contracture did not increase in any of the knees. Conclusion Congenital dislocation of the patella should be suspected in every patient with knee flexion contracture, genu valgus, external tibial torsion, foot deformity and delayed walking. Successful results were obtained when the operation was performed in younger children. Other procedures, such as the semitendinosus tenodesis or tendon transfer, might have to be combined to achieve better stability with flexion in older children. None of the authors received financial support for this study.     

Operative reconstruction of the severe sequelae of infantile septic arthritis of the hip

We retrospectively reviewed the results of operative reconstruction of 21 hips in 21 patients with severe sequelae due to infantile septic arthritis of the hip. Eleven hips were classified as Choi type IIIA, 4 as type IIIB, 1 as type IVA, and 5 as type IVB sequelae. The average age at the time of the first surgery was 4.2 years, and the average follow-up period was 8.9 years. Successful results were obtained in 8 of the 11 type IIIA hips by means of a combination of open reduction, femoral varus osteotomy, and pelvic osteotomy. However, only 2 of the 4 type IIIB hips treated by femoral valgus osteotomy and/or bone grafting and 2 of the 6 types IVA and IVB hips treated by greater trochanter arthroplasty had successful results.     

Surgical treatment of hip dislocation in Kabuki syndrome: use of incomplete periacetabular osteotomy for posterior acetabular wall deficiency

Purpose

Kabuki syndrome is characterized by distinctive facial features, skeletal anomalies, persisting fingertip pads with dermatoglyphic abnormalities, postnatal growth deficiency and mental retardation. This report reviews our results in the operative treatment of hip dislocations in patients with Kabuki syndrome.

Methods

Between 2001 and 2009, seven dislocated hips (three unilateral and two bilateral hips) in five patients (all girls) were operatively treated at our institution. The operative treatment consists of open reduction, femoral derotation varus osteotomy, pelvic osteotomy (Salter in one and incomplete periacetabular osteotomy in six hips) and capsular plication. The age of the patients at the time of surgery ranged from 2.4 to 5.7 years, with an average of 3.6 years.

Results

The follow-up postoperative period ranged from 3.2 to 6.3 years, with an average of 5.0 years. At the final follow-up, all patients reported no click and no pain, and showed well-contained hips by radiographs. All seven hips were graded as Severin class I-II. One patient presented as having habitual dislocation of the hip 4.4 years after surgery. Computed tomographic (CT) scans revealed posterior acetabular wall deficiency, which was not corrected by the anterolaterally directed Salter osteotomy. The incomplete periacetabular osteotomy provided sufficient posterolateral coverage of the acetabulum.

Conclusion

Operative treatment combining open reduction, femoral derotation varus and incomplete periacetabular osteotomies, and capsular plication provided successful results in patients with Kabuki syndrome who had the characteristics of hip instability such as ligamentous laxity, muscular hypotonia and posterior acetabular wall deficiency.     

Urachal anomalies and tumor: clinical investigation of 14 cases

Diseases associated with persistent urachus are relatively rare. During the past 9-year period, there have been 14 patients with urachal disease consisting of 10 with urachal abscess and 4 with urachal cancer. The 10 patients with urachal abscess consisted of 7 males and 3 females aged 19-77 years (mean, 46 years). The 4 patients with urachal cancer consisted of 2 males and 2 females aged 48-81 years (mean, 57 years). As symptoms, lower abdominal pain was frequently observed in the patients with persistent urachus with abscess and gross hematuria in those with urachal cancer. Echo and magnetic resonance imaging (MRI) were useful for visualizing the lesion. Computed tomogtaphic (CT) scanning could not visualize the lesion in 2 patients. Nine patients underwent MRI, which visualized the lesion in all of them. As urachal abscess, an umbilical fistula was observed in 3 patients, urachal cyst in 4, and urachal diverticulum in 1. The preoperative diagnosis was urachal cancer in 6 patients, and pathological examination showed 4 patients with adenocarcinoma, 1 with inflammatory granuloma, and 1 with pseudosarcoma. Urachal abscess was treated by resection of the abscess in 6 patients, transurethral resection in 1, and resection of the umbilicus and urachus and total cystectomy in the other. Of the patients with urachal cancer, 1 underwent total cystectomy and the other 3 underwent total urachal resection and partial cystectomy. In 2 patients with persistent urachus with abscess, the differentiation between abscess and malignant tumor was difficult.     

Pulmonary metastasectomy for pulmonary metastasis of breast cancer has a limited prognostic impact: a multi-institutional retrospective analysis

BackgroundPulmonary metastasectomy (PM) for breast cancer-derived pulmonary metastasis is controversial. This study aimed to assess the prognostic factors and implication of PM for metastatic breast cancer using a multi-institutional database.MethodsClinical data of 253 females with pulmonary metastasis of breast cancer who underwent PM between 1982 and 2017 were analyzed retrospectively.ResultsThe median patient age was 56 years. The median follow-up period was 5.4 years, and the median disease-free interval (DFI) was 4.8 years. The 5- and 10-year survival rates after PM were 64.9% and 50.4%, respectively, and the median overall survival was 10.1 years. Univariate analysis revealed that the period of PM before 2000, a DFI <36 months, lobectomy/pneumonectomy, large tumor size, and lymph node metastasis were predictive of a worse overall survival. In the multivariate analysis, a DFI <36 months, large tumor size, and lymph node metastasis remained significantly related to overall survival. The 5- and 10-year cancer-specific survival rates after PM were 66.9% and 54.7%, respectively, and the median cancer-specific survival was 13.1 years. Univariate analyses revealed that the period of PM before 2000, DFI <36 months, lobectomy/pneumonectomy, large tumor size, lymph node metastasis, and incomplete resection were predictive of a worse cancer-specific survival. Multivariate analysis confirmed that a DFI <36 months, large tumor size and incomplete resection were significantly related to cancer-specific survival.ConclusionsAs PM has limited efficacy in breast cancer, it should be considered an optional treatment for pulmonary metastasis of breast cancer.     

Modafinil augments brain activation associated with reward anticipation in the nucleus accumbens

Rationale

The nucleus accumbens (NAc) works as a key brain structure of the reward system, in which reward-related neural activity is well correlated with dopamine release from mesolimbic dopaminergic neurons.

Objectives

Since modafinil can modulate dopaminergic transmission through re-uptake inhibition of dopamine, we investigated whether modafinil affects the reward-related brain activity in the NAc in healthy subjects.

Methods

Twenty healthy participants underwent two series of functional magnetic resonance imaging while performing monetary incentive delay task in which they were cued to anticipate and respond to a rapidly presented target to gain or avoid losing varying amounts of money, under modafinil or placebo condition. Blood oxygenation-level dependent (BOLD) activation signals during gain and loss anticipations were analyzed in the NAc as an a priori region of interest as well as the whole brain.

Results

Modafinil significantly changed subjective feelings toward positive ones. The activation of BOLD signals was observed during gain anticipation under the placebo and modafinil conditions in the left and bilateral NAc, respectively. The modafinil condition showed significantly higher BOLD signal change at the highest gain (+¥500) cue compared to the placebo condition.

Conclusions

The present study showed that modafinil affects reward processing in the NAc in healthy subjects through enhancing more positive anticipation, and it may provide a basis for the use of this drug for treating anhedonia observed in psychiatric disorders.     

New protocol to detect coronary spastic angina without fixed stenosis

A new combined test, accelerated exercise following mild hyperventilation (HV), was examined to determine whether it is effective at detecting a positive response in patients with pharmacologically-induced coronary vasospasm and near normal coronary arteries. Fifty-eight consecutive patients who underwent both triple non-invasive spasm provocation tests and diagnostic coronary angiography were enrolled. They all had pharmacologically-induced coronary vasospasms and no significant organic stenosis. In these patients, an HV test was performed first, followed by a treadmill exercise test (TET), and finally the new combined test under no medication within 3 days. Of the 58 patients, positive responses were observed in 9 patients to the HV, in 15 to the TET, and in 35 to the newly combined test. The remaining 21 patients had negative responses although the triple sequential tests were perfomed. Thus, the sensitivities of the HV test, TET, and newly combined test were 16% (9/58), 26% (15/58), and 63% (35/56), respectively. Forty-six subjects with near normal coronary arteries and no ACh-provoked spasm served as controls. None of these subjects had positive responses to any of these three tests, and thus their specificity was all 100%. No serious or irreversible complications were seen in this study. We recommend this newly-combined protocol for the induction of coronary artery spasm in patients with vasospastic angina pectoris and without significant stenosis as a diagnostic tool.     

Evidence for the involvement of ecto-5′-nucleotidase (CD73) in drug resistance

Increased ecto-5′-nucleotidase (ecto-5′NT) protein expression in several multidrug-resistant (MDR) cell lines, documented previously by our group, suggests that this enzyme is involved in drug resistance. Here, Northern blot analysis of selected cell lines and their MDR variants positively correlated ecto-5′NT protein with its mRNA expression. An inhibitor of ecto-5′NT enzymatic activity, α,β-methyleneadenosine 5′-diphosphate (AMP-CP), was used to determine if functionally active enzyme had a role in drug resistance. AMP-CP (0.3 mM) reversed the resistance of ecto-5′NT-positive MDR cells (MCF7/A6, L1210/A) to doxorubicin, whereas it did not affect the doxorubicin sensitivity of the ecto-5′NT-negative parental cell lines or that of 2 ecto-5′NT-negative MDR cell lines (HL60/VCR and A2780/DX5). Furthermore, AMP-CP increased rhodamine uptake and inhibited rhodamine efflux from ecto-5′NT-positive MDR cells without affecting ecto-5′NT-negative MDR cells. The presence of exogenous adenosine (0.5 μM) circumvented AMP-CP-induced inhibition of rhodamine efflux from EL4/ADM cells. AMP-CP inhibited the growth of the ecto-5′NT-positive L1210/A MDR cells but had no effect on the growth of the parental cell line. Determination of intracellular ATP levels indicated that MDR cells which had increased ecto-5′NT expression also had a lower intracellular ATP level than their parental cells. Our results suggest that, in certain MDR cell lines, ecto-5′NT serves as a required accessory molecule in resistance mediated by ATP-dependent mechanisms and that growth-sustaining nucleosides are provided by this salvage pathway. © 1996 Wiley-Liss, Inc.