Interest of awake surgery for ruptured cerebral arteriovenous malformations close to speech areas - Surgical note.

BACKGROUND: Cerebral arteriovenous malformations (AVMs) are rare, with incidence of 1.12-1.42 cases per 100,000 person-years (Ozpinar et al., 2017). Few studies report applications of awake surgery. The goal of this report was to assess the interest of awake surgery in complete resection of cortical AVMs located close to eloquent speech areas, enabling detection of real functional cortical reorganization due to the AVM and parenchymal hematoma. CASE REPORT: A 38-year-old right-handed patient was admitted to the University Hospital of Besançon for dysphasia due to a ruptured left temporal arteriovenous malformation. The patient underwent asleep-awake-asleep surgery. Intraoperative cortical mapping revealed the presence of functional language areas in uncommon locations compared to known neuro-functional anatomy. DISCUSSION: In this patient, speech areas were redistributed, probably due to neuroplasticity after cerebral hemorrhage, leading to a new cortical architecture, which would have been unrecognized based on preoperative radiological imaging alone. CONCLUSION: We report the interest of awake surgery to achieve complete safe resection of ruptured AVMs located close to eloquent speech areas, using intraoperative cortical mapping.

Delayed care for patients with newly diagnosed cancer due to COVID-19 and estimated impact on cancer mortality in France.

BACKGROUND: The impact of the first coronavirus disease 2019 (COVID-19) wave on cancer patient management was measured within the nationwide network of the Unicancer comprehensive cancer centers in France. PATIENTS AND METHODS: The number of patients diagnosed and treated within 17 of the 18 Unicancer centers was collected in 2020 and compared with that during the same periods between 2016 and 2019. Unicancer centers treat close to 20% of cancer patients in France yearly. The reduction in the number of patients attending the Unicancer centers was analyzed per regions and cancer types. The impact of delayed care on cancer-related deaths was calculated based on different hypotheses. RESULTS: A 6.8% decrease in patients managed within Unicancer in the first 7 months of 2020 versus 2019 was observed. This reduction reached 21% during April and May, and was not compensated in June and July, nor later until November 2020. This reduction was observed only for newly diagnosed patients, while the clinical activity for previously diagnosed patients increased by 4% similar to previous years. The reduction was more pronounced in women, in breast and prostate cancers, and for patients without metastasis. Using an estimated hazard ratio of 1.06 per month of delay in diagnosis and treatment of new patients, we calculated that the delays observed in the 5-month period from March to July 2020 may result in an excess mortality due to cancer of 1000-6000 patients in coming years. CONCLUSIONS: In this study, the delays in cancer patient management were observed only for newly diagnosed patients, more frequently in women, for breast cancer, prostate cancer, and nonmetastatic cancers. These delays may result is an excess risk of cancer-related deaths in the coming years.

Bone reconstruction after malignant tumour resection using a motorized lengthening intramedullary nail in adolescents: preliminary results.

PURPOSE: Bone reconstruction after malignant tumour of the lower limbs is a challenge. Our aim was to investigate the results of bone lengthening and transport using the Fitbone motorized lengthening nail. METHODS: Eight patients were included. Two patients had had a tumour involving soft tissue only without bone resection. In six patients, the initial bone defect was 15.5 cm (8 to 24). The first step of reconstruction had consisted of temporary fixation and a cement spacer. The second step had consisted of bone grafting in five patients. One patient was managed with bone transport. Eventually, five patients had a limb-length discrepancy (LLD) managed with bone lengthening only. Two patients had a LLD and a nonunion managed with bone transport and lengthening. Mean age at bone lengthening was 15.2 years (11 to 19). Mean follow-up was 30.5 months (10 to 48). RESULTS: In all, 11 nails were implanted in eight patients (eight femurs, three tibias). Mean length gain per procedure was 54.5 mm (30 to 80). Mean healing index was 48.4 days per cm (22.6 to 85.7). The complication rate was 18%. In two cases we observed a loose locking screw, which was revised. In all cases the lengthening involved the short bone (femur or tibia). Mean Musculoskeletal Tumor Society (MSTS) score improved from 52.7 (16.6 to 73.3) to 79.9 (63.3 to 96.6). CONCLUSIONS: Bone reconstruction using a motorized intramedullary nail stands as a safe and reliable alternative after malignant tumour. It allows biological reconstruction with satisfactory clinical and radiological outcome and low complication rate. LEVEL OF EVIDENCE: III.

Day surgery for anterior cruciate ligament reconstruction in children: a prospective study on feasibility and satisfaction.

PURPOSE: To evaluate the feasibility of day surgery anterior cruciate ligament reconstruction (ACLR) in children. Our hypothesis was that proper patient selection, adequate information and a streamlined clinical pathway would allow for a safe and satisfactory procedure. METHODS: A total of 20 patients, seven girls and 13 boys, who underwent outpatient ACLR at mean age of 14 years (8 to 16) were included in this single institution prospective case series. Surgery took place under simple general anesthesia while analgesia protocol combined local anesthesia, oral analgesics for the postoperative period and compressive cryotherapy. The main failure criterion was the inability to return home the day of surgery or the need for rehospitalization during the first week after discharge. Secondary evaluation parameters were the rate of postoperative complications, postoperative pain, quality of life (QOL) and patients' satisfaction outcome questionnaire (PSOQ) at postoperative day 1, 7 and 31. RESULTS: Of the 20 patients included, only one failed the ambulatory mode. The mean PSOQ score was, respectively for children and parents, 91.4% and 90.7% at day 7 and 94.6% and 95.7% at one month. Postoperative QOL at day 7 was very satisfying for all patients. Two minor early complications were reported. Both of the late complications, which included one partial wound dehiscence and one persistent knee swelling, resolved spontaneously. CONCLUSION: This study indicates that, in a targeted and well-informed population, day surgery for children ACLR yields high level of patient and family satisfaction in a safely manner. LEVEL OF EVIDENCE: IV, prospective case series.

Stereophotogrammetric three-dimensional photography is an accurate and precise planimetric method for the clinical visualization and quantification of human papilloma virus-induced skin lesions.

BACKGROUND: The quantification of human papilloma virus (HPV)-induced skin lesions is essential for the clinical assessment of the course of disease and the response to treatment. However, clinical assessments that measure dimensions of lesions using a caliper do not provide complete insight into three-dimensional (3D) lesions, and its inter-rater variability is often poor. OBJECTIVE: The aim of this study was to validate a stereophotogrammetric 3D camera system for the quantification of HPV-induced lesions. METHODS: The camera system was validated for accuracy, precision and interoperator and inter-rater variability. Subsequently, 3D photographs were quantified and compared to caliper measurements for clinical validation by Bland-Altman modelling, based on data from 80 patients with cutaneous warts (CW), 24 with anogenital warts (AGW) patients and 12 with high-grade squamous intraepithelial lesions of the vulva (vulvar HSIL) with a total lesion count of 220 CW, 74 AGW and 31 vulvar HSIL. RESULTS: Technical validation showed excellent accuracy [coefficients of variation (CV) ≤ 0.68%] and reproducibility (CVs ≤ 2%), a good to excellent agreement between operators (CVs ≤ 8.7%) and a good to excellent agreement between different raters for all three lesion types (ICCs ≥ 0.86). When comparing 3D with caliper measurements, excellent biases were found for diameter of AGW (long diameter 5%), good biases were found for diameter of AGW (short diameter 10%) and height of CW (8%), and acceptable biases were found for the diameter of CW (11%) and vulvar HSIL (short diameter 14%, long diameter 16%). An unfavourable difference between these methods (bias 25%) was found for the assessment of height of AGWs. CONCLUSION: Stereophotogrammetric 3D imaging is an accurate and reliable method for the clinical visualization and quantification of HPV-induced skin lesions.

A randomized controlled proof-of-concept trial of digoxin and furosemide in adults with cutaneous warts.

BACKGROUND: Topical ionic contraviral therapy (ICVT) with digoxin and furosemide inhibits the potassium influx on which DNA viruses rely for replication. Therefore, ICVT was hypothesized to be a potential novel treatment for cutaneous warts. OBJECTIVES: To assess the clinical efficacy, safety and tolerability of ICVT in adults with cutaneous warts. The secondary objective was to gain insight into the underlying working mechanism of ICVT. METHODS: Treatment with ICVT was assessed for efficacy, safety and tolerability in a single- centre, randomized, double-blind, placebo-controlled phase IIA trial. Eighty adult patients with at least two cutaneous warts (plantar or common) were randomized to one of four treatments: digoxin + furosemide (0·125%), digoxin (0·125%), furosemide (0·125%) or placebo. The gel was administered once daily for 42 consecutive days. Predefined statistical analysis was performed with a mixed-model ancova. The trial was registered at ClinicalTrials.gov with number NCT02333643. RESULTS: Wart size and human papillomavirus (HPV) load reduction was achieved in all active treatment groups. A statistically significant reduction in wart diameter of all treated warts was shown in the digoxin + furosemide treatment group vs. placebo (-3·0 mm, 95% confidence interval -4·9 to -1·1, P = 0·002). There was a statistically significant reduction in the HPV load of all treated warts in the digoxin + furosemide group vs. placebo (-94%, 95% confidence interval -100 to -19, P = 0·03). With wart size reduction, histologically and immunohistochemically defined viral characteristics disappeared from partial and total responding warts. CONCLUSIONS: This study demonstrates the proof of concept for the efficacy of topical ICVT in adults with cutaneous warts.

Salvage of congenital pseudarthrosis of the tibia by the induced membrane technique followed by a motorised lengthening nail.

An 18-year-old male with neurofibromatosis type I was treated for congenital pseudarthrosis of the tibia using the Masquelet induced-membrane technique with internal fixation by retrograde implantation of a transplantar intramedullary nail. Bone healing was obtained at the expense of malunion with external rotation and 5.5cm of lower limb shortening. A motorised intramedullary-lengthening nail (Fitbone®, Wittenstein, Igersheim, Germany) was implanted. This treatment was successful in correcting the rotational malalignment and limb length discrepancy. The motorised nail Fitbone® may be a valid option for treating complex cases of limb length discrepancy, including those combined with limb deformities.

Prediction of local and metastatic recurrence in solitary fibrous tumor: construction of a risk calculator in a multicenter cohort from the French Sarcoma Group (FSG) database.

BACKGROUND: Solitary fibrous tumors (SFT) are rare unusual ubiquitous soft tissue tumors that are presumed to be of fibroblastic differentiation. At present, the challenge is to establish accurate prognostic factors. PATIENTS AND METHODS: A total of 214 consecutive patients with SFT diagnosed in 24 participating cancer centers were entered into the European database (www.conticabase.org) to perform univariate and multivariate analysis for overall survival (OS), local recurrence incidence (LRI) and metastatic recurrence incidence (MRI) by taking competing risks into account. A prognostic model was constructed for LRI and MRI. Internal and external validations of the prognostic models were carried out. An individual risk calculator was carried out to quantify the risk of both local and metastatic recurrence. RESULTS: We restricted our analysis to 162 patients with local disease. Twenty patients (12.3%) were deceased at the time of analysis and the median OS was not reached. The LRI rates at 10 and 20 years were 19.2% and 38.6%, respectively. The MRI rates at 10 and 20 years were 31.4% and 49.8%, respectively. Multivariate analysis retained age and mitotic count tended to significance for predicting OS. The factors influencing LRI were viscera localization, radiotherapy and age. Mitotic count, tumor localization other than limb and age had independent values for MRI. Three prognostic groups for OS were defined based on the number of unfavorable prognostic factors and calculations were carried out to predict the risk of local and metastatic recurrence for individual patients. CONCLUSION: LRI and MRI rates increased between 10 and 20 years so relapses were delayed, suggesting that long-term monitoring is useful. This study also shows that different prognostic SFT sub-groups could benefit from different therapeutic strategies and that use of a survival calculator could become standard practice in SFTs to individualize treatment based on the clinical situation.

Relevance of MRI for management of non-displaced lateral humeral condyle fractures in children.

INTRODUCTION: The treatment for non-displaced (<2 mm displacement) fractures of the lateral humeral condyle in children is controversial. Most studies recommend non-surgical treatment. However, plain radiographs are not sufficient to evaluate extension of the fracture line through the articular cartilage. This explains the high frequency of secondary displacements and non-unions, despite well-conducted conservative treatment. We hypothesized that MRI could be used to analyse whether the fracture is complete or incomplete. This could help to determine whether surgical or conservative treatment is indicated. MATERIAL AND METHODS: This prospective study enrolled children being treated for a non-displaced (< 2 mm gap) fracture of the lateral humeral condyle. All patients were treated with a long-arm cast in the emergency room. An MRI was done later on without sedation. A specific protocol was used to reduce the duration of the examination. T2-weighted and proton density fat-saturated sequences were used. RESULTS: Twenty-seven patients were enrolled: 16 boys and 11 girls with a mean age of 5 years (2-10). The MRI was performed an average of 7 days (1-23) after the fracture. The MRI could not be interpreted in two cases because the child had moved during the examination. In the other 25 patients, the fracture was incomplete in 17 patients and complete in 8 patients. Two children had secondary displacement diagnosed 7 and 11 days after the fracture event. These two patients underwent open reduction and internal fixation. There was no correlation between patient age and the fracture being complete or incomplete. There were no cases of non-union. CONCLUSION: MRI appears to be a reliable method for determining whether the fracture line is complete or incomplete. It can be performed without sedation, even in children as young as 2 years of age. Use of an injury-specific MRI protocol reduces the length of the examination, thereby improving its performance. We recommend that it be used to analyse non-displaced fractures of the lateral humeral condyle in children. LEVEL OF EVIDENCE: 3 Prospective study.

Does patella lowering improve crouch gait in cerebral palsy? Comparative retrospective study.

BACKGROUND: Patella lowering aims to improve quadriceps function as a means of correcting crouch gait in patients with cerebral palsy. Few studies have assessed the effects of patella lowering as a component of multilevel surgery. HYPOTHESIS: Including patella lowering into the components of multilevel surgery is beneficial in patients with crouch gait and patella alta. MATERIAL AND METHODS: In 12 lower limbs with patella alta (Caton-Deschamps index>1.4) in 41 children with cerebral palsy, patella lowering was performed, without distal femoral extension osteotomy or hamstring release. Among limbs with similar surgical procedures (e.g., hamstring lengthening, rectus femoris transfer) except for patella lowering, controls were selected retrospectively by matching on a propensity score for patella lowering. The propensity score was computed based on preoperative knee flexion contracture, knee extension lag, and minimum knee flexion at mid-stance. Clinical and 3D kinematic data were compared between the two groups. RESULTS: The improvement in minimum knee flexion at mid-stance was significantly greater in the group with patellar lowering (-24°±12°vs. -12°±7°). The Gait Deviation Index improved similarly in the two groups. Knee flexion contracture improved only in the group with patellar lowering. Extension lag did not improve in either group. Peak knee flexion during the swing phase remained unchanged in both groups. DISCUSSION: Patellar lowering is effective in diminishing minimum knee flexion at mid-stance in patients with patella alta and crouch gait due to cerebral palsy. Patellar lowering has not adverse effects on gait. These findings cannot be assumed to apply to patients with normal patellar height. LEVEL OF EVIDENCE: IV (retrospective study).

MRI of acute osteomyelitis in long bones of children: Pathophysiology study.

INTRODUCTION: The classic pathophysiology of acute osteomyelitis in children described by Trueta has a metaphyseal infection as the starting point. This hypothesis was recently brought into question by Labbé's study, which suggested a periosteal origin. Thus, we wanted to study this disease's pathophysiology through early MRI examinations and to look for prognostic factors based on abnormal findings. MATERIAL AND METHODS: This was a prospective, multicentre study that included cases of long bone osteomyelitis in children who underwent an MRI examination within 7days of the start of symptoms and within 24hours of the initiation of antibiotic therapy. We also collected clinical, laboratory and treatment-related data. RESULTS: Twenty patients were included, including one with a bifocal condition. The lower limb was involved in most cases (19/21). Staphylococcus aureus was found most frequently. Metaphyseal involvement was present in all cases. No isolated periosteal involvement was found in any of the cases. No prognostic factors were identified based on the various abnormal findings on MRI. CONCLUSION: Our study supports the metaphyseal origin of acute osteomyelitis in children. LEVEL OF EVIDENCE: II.

Arthroscopic treatment of intercondylar eminence fractures with intraepiphyseal screws in children and adolescents.

INTRODUCTION: Tibial intercondylar eminence fracture rarely occurs in childhood. Its treatment requires anatomic reduction to provide knee stability and a rigid fixation to minimize postoperative immobilization time. HYPOTHESIS: Arthroscopy combined with fluoroscopy with intra-epiphyseal ASNIS screw fixation can meet the requirements of this treatment. MATERIAL AND METHODS: The series comprised 24 patients (mean age: 11 years) with Meyers and McKeever type II tibial intercondylar eminence fractures (n=15) or type III (n=9), operated on between 2011 and 2013. Fixation with 4-mm ASNIS screws was placed arthroscopically. The demographic data, associated lesions, radiological union, stability, functional result, and the Lysholm score were evaluated. RESULTS: With a mean follow-up of 2 years, the mean Lysholm score was 99.3 for type II and 98.6 for type III fractures. At the 6th postoperative week, range of motion in the operated knees was identical to the healthy knees. At the 12th postoperative week, there was no sign of anterior laxity. Twelve cases included meniscal entrapment, but no significant difference was observed in the functional results. DISCUSSION, CONCLUSION: ASNIS screw fixation under arthroscopy can be successfully applied in the treatment of types II and III tibial intercondylar eminence fractures in children. This technique provides excellent stability, allows early weigh-tbearing, and preserves function of the knee and its growth. LEVEL OF EVIDENCE: IV, retrospective study.

[Pseudotumoral soft tissue masses in children and adolescents]. / Masses des tissus mous d'allure tumorale de l'enfant et de l'adolescent.

INTRODUCTION: Pseudotumoral soft tissue masses in children and adolescents are a frequent reason for consultation and a diagnostic dilemma. Soft tissue malignancies are relatively uncommon, unlike the large number of benign lesions that may be seen in the superficial tissue and that can be diagnosed with clinical characteristics. MATERIALS AND METHODS: This retrospective study concerns 161 children and adolescents less than 20 years old, referred for a soft tissue mass between 2007 and 2011. It describes their epidemiology, clinical characteristics, and course of care to validate a diagnostic strategy for such masses. RESULTS: Final diagnoses were malignant tumors (44%), benign tumors (32%), and pseudotumoral lesions (24%). Clinical features were similar between these three groups except for age and tumor location, with more benign thoracic masses in younger children. Clinical and radiological association led to an accurate diagnosis for 50% of benign masses and with cytological analysis contribution in 79% of benign tumors and 86% of pseudotumoral lesions. Malignant tumors were suspected in only 39% of cases with radiological exams and in 89% after fine-needle aspiration, an essential additional diagnostic tool. Final diagnoses were formally established through simple standard clinical and radiological evaluation in 19 patients (11.8%; benign tumors, seven patients; malformations, eight patients; post-traumatic lesions, two patients; infection and inflammation, one patient each); ultrasound exam in five patients (3.1%; hemangioendotheliomas, two patients, fascial dehiscence, hemangioma, and vascular malformation, one patient each); MRI in four patients (2.5%; three vascular malformations and one lipoma); CT in two cases (1.2%; vascular malformation and myositis ossificans), and radiological examinations associated with cell aspiration in 15 cases (9.3%; ten benign tumors and five malignant tumors). CONCLUSIONS: A multidisciplinary approach should be requested from oncological, radiological, and pathologic experts to optimize soft tissue mass management as soon as initial investigations start. The authors advise a diagnostic strategy for children with pseudotumoral soft tissue masses.

Neuromuscular scoliosis.

Scoliosis is a common deformity in many types of neuromuscular disease. Severe spinal curvature can cause difficulty in sitting. Conservative and surgical treatment of neuromuscular scoliosis differs from idiopathic scoliosis, being more complex and with a higher complications rate. Non-surgical measures rarely fully control progressive scoliosis, but aim to prevent spinal deformities secondary to muscular hypotonia or contracture. Twenty-four hour bracing should be adjusted throughout growth, and may induce functional impairment and loss of independence. Corrective surgery requires multidisciplinary management and perioperative screening. Pelvic obliquity is commonly associated with neuromuscular scoliosis, making sitting difficult: correction needs to be considered during surgical planning. The goal of surgical correction is to obtain and maintain a well-balanced spine above a well-positioned pelvis. Preoperative multidisciplinary assessment enables potential problems of terrain to be anticipated. Respiratory function investigation will guide possible non-invasive perioperative ventilation. Nutritional and psychosocial assessment should also be incorporated in this preparation, as should overall postoperative care. Implementing this overall strategic planning can achieve a good surgical and functional result in the vast majority of cases.

Congenital tibial deficiencies: treatment using the Ilizarov's external fixator.

INTRODUCTION: Congenital longitudinal deficiency of the tibia is a rare and often syndromic anomaly. Amputation is usually the preferred treatment option in complete absence of the tibia; however, a conservative management might be implemented in partial forms or in case of amputation refusal. Our experience with the Ilizarov fixator, convinced us this device was the best suited for progressive correction of lower limbs length discrepancies and articular or bone angular limb deformities (ALD). The aim of this study is to highlight the interest of the Ilizarov fixator in the multistage conservative treatment of congenital tibial deficiencies. MATERIAL AND METHODS: A retrospective study was conducted in nine patients suffering from Type I or II congenital tibial deficiencies (Jones) and sequentially managed using the Ilizarov technique. The functional outcome after treatment completion was then clinically assessed. RESULTS: The different stages of correction were recorded for each individual patient. Patients were assessed at a mean follow-up of 18,3 years (4-32 years). The mean maximum knee flexion was 35 degrees (0 degrees -90 degrees ) in type I deficiencies and 118 degrees (90 degrees -140 degrees ) in type II deficiencies. One patient underwent amputation and a bilateral knee arthrodesis was performed in another case. DISCUSSION: Few series in the literature report a comparable length of follow-up period in the conservative management of severe congenital tibial deficiencies. In our study, the Ilizarov fixator provided satisfactory progressive corrections of severe congenital tibial deficiencies. LEVEL OF EVIDENCE: Level IV therapeutic retrospective study.

Popliteal pterygium knee contracture: treatment with the Ilizarov technique.

INTRODUCTION: Various treatment options are in use to address severe knee flexion contractures in children. Their success depends on an adequate selection of the proper one applying to each individual anatomical situation. HYPOTHESIS: Applied to limb deformity, the Ilizarov technique combines progressive correction, to joint structures flexibility restitution in case of severe knee flexion contracture. We review a continuous series of popliteal pterygium syndrome patients managed with this technique. PATIENTS AND METHODS: Medical records of eight children (11 knees), consecutively treated between 1986 and 2007, were reviewed. Knee flexion ranged from 40 to 120 degrees. Contracture (> 90 degrees) was extremely severe in 10 cases. Progressive correction was gained by Ilizarov external fixation. Complications during and following articular chain distraction-lengthening were noted. Follow-up ranged from 1 to 21 years. RESULTS: Surgical realignment was rendered particularly complex by the popliteal cutaneous band itself, partly responsible of the joint stiffness and sciatic nerve shortening. Deformities were corrected by the Ilizarov technique. Complete extension was obtained in all cases. In six cases, flexion contracture reccurrence required to repeat the correction, using the same technique, at a mean interval of 3-4 years. During follow-up, four evolutive partial posterior tibial dislocations and one complete dislocation were diagnosed, all associated with recurrence of the flexion contracture. LEVEL OF EVIDENCE: Level IV. Therapeutic Study.

[Mini-invasive double-incision for patellar tendon harvesting in anterior cruciate ligament reconstruction]. / Prélèvement du transplant patellaire par double voie mini invasive dans la reconstruction du ligament croisé antérieur.

PURPOSE OF THE STUDY: Injury to infrapatellar branches of the medial saphenous nerve are incriminated in disorders of the anterior aspect of the knee, particularly following bone-tendon-bone reconstruction. We demonstrated in prior anatomic work the usefulness of using a double-incision minimal approach for harvesting the patellar transplant in order to spare the nerve branches. MATERIAL AND METHODS: The patellar transplant is harvested via two vertical incisions, one on the apex of the patella and the other along the protrusion of the anterior tibial tubercle. After harvesting the bony transplant from the patella, discision of the patellar tendon fibers is advanced subcutaneously towards the anterior tibial tubercle, allowing extraction of the patellar graft via the tibial incision using a small forceps and respecting the peritendon. The tibial bone is then harvested. The standard anterolateral and anteromedial approaches are used for the ligament reconstruction. RESULTS: We have conducted a case control study between this harvesting technique using the double-incision technique (42 knees) versus the conventional single incision harvesting technique. We studied the influence of the harvesting technique on anterior knee pain, the surface area of the sensorial disorders involving the anterior aspect of the knee, and kneeling problems. DISCUSSION: Our technique has enabled a significant decrease in the surface area of sensorial disorders (7.4 cm2 versus 17.4 cm2) and problems kneeling compared with the conventional method.

The human ATP binding cassette gene ABCA13, located on chromosome 7p12.3, encodes a 5058 amino acid protein with an extracellular domain encoded in part by a 4.8-kb conserved exon.

The ABCA subfamily of ATP-binding cassette (ABC) transporters includes eleven members to date. In this study, we describe a new, unusually large gene on chromosome 7p12.3, ABCA13. This gene spans over 450 kb and is split into 62 exons. The predicted ABCA13 protein consists of 5,058 ami- no acid residues making it the largest ABC protein described to date. Like the other ABCA subfamily members, ABCA13 contains a hydrophobic, predicted transmembrane segment at the N-terminus, followed by a large hydrophilic region. In the case of ABCA13, the hydrophilic region is unexpectedly large, more than 3,500 amino acids, encoded by 30 exons, two of which are 4.8 and 1.7 kb in length. These two large exons are adjacent to each other and are conserved in the mouse Abca13 gene. Tissue profiling of the major transcript reveals the highest expression in human trachea, testis, and bone marrow. The expression of the gene was also determined in 60 tumor cell lines and the highest expression was detected in the SR leukemia, SNB-19 CNS tumor and DU-145 prostate tumor cell lines. ABCA13 has high similarity with other ABCA subfamily genes which are associated with human inherited diseases: ABCA1 with the cholesterol transport disorders Tangier disease and familial hypoalphalipoproteinemia, and ABCA4 with several retinal degeneration disorders. The ABCA13 gene maps to chromosome 7p12.3, a region that contains an inherited disorder affecting the pancreas (Shwachman-Diamond syndrome) as well as a locus involved in T-cell tumor invasion and metastasis (INM7), and therefore is a positional candidate for these pathologies.

[Disseminated histoplasmosis detected by lingual and tonsillar erosions in an immunocompetent patient]. / Histoplasmose disséminée révélée par des érosions linguales et amygdaliennes chez un malade immunocompétent.

BACKGROUND: Histioplasmosis is a usually asymptomatic fungal infection. In the immunocompetent patient, it leads to chronic disseminated infection. Mucosal involvement is common and can provide the diagnosis. CASE REPORT: A metropolitan Frenchman with a history of alcoholism and smoking and living in Guyana consulted for lingual and tonsil erosion. Squamous cell carcinoma was suspected but not confirmed at pathology. The patient had a bi-apical infiltration on the chest x-ray and was treated empirically for tuberculosis. The diagnosis of histoplasmosis was reached when rare Histoplasma capsulatum were evidenced from a buccal swab. Itraconazole led to cure in 6 months. DISCUSSION: This case illustrates the importance of mucosal signs in the diagnosis of disseminated histoplasmosis in immunocompetent subjects. Histoplasmosis is rarely the cause of active infection in immunocompetent subjects. In these patients, the fungal infection generally progresses to chronic dissemination. Mucosal signs are frequent in this form but are rare in case of cutaneous histoplasmosis. Itraconazole (200 mg/d) is indicated for 6 months.

Delayed gastric emptying after pancreaticoduodenectomy and pancreaticogastrostomy.

OBJECTIVE: To find out which factors influence the development of delayed gastric emptying (DGE) after pancreaticoduodenectomy with pancreaticogastrostomy. DESIGN: Prospective clinical study. SETTING: University hospital, France. SUBJECTS: 88 patients of 103 consecutive patients who had had pancreaticoduodenectomies, November 1991-November 1997. INTERVENTIONS: Whipple resection, and parenteral and enteral nutrition. MAIN OUTCOME MEASURES: Mortality, morbidity, and development of DGE (defined as the need for a postoperative nasogastric tube for 10 days or longer). RESULTS: One patient died, and 44 developed postoperative complications. 36 patients (41%) developed DGE in 21 of whom (58%, 24% of the total) it was in isolation, with no other complication; and 52 (59%) did not. There were significant differences between those who developed DGE and those who did not: 30 men (83%) compared with 6 women (17%) developed DGE compared with 32 (62%) and 20 (38%) (p = 0.03;); 15 (42%) developed a complication as well as DGE compared with 8 (15%) (p = 0.005); 10(28%) who developed DGE required reoperation compared with 4(8%) (p = 0.011); mean (SD) hospital stay was 30(12) days among those with DGE compared with 17 (5) days (p= 0.0001); and their mean (SD) serum protein concentration on day 1 was 46 (1) compared with 51 (7) g/L (p=0.01). Multivariate analysis showed that three factors independently influenced the development of DGE: sex (p = 0.01), the need for reoperation (p = 0.03) and the mean serum protein concentration on day 1 (p = 0.04). CONCLUSION: Postoperative complication and the need for reoperation remain the most common factors linked to the development of DGE. However, in a quarter of patients DGE was not associated with any postoperative complication.