Petit-Morel method for hip development dysplasia in toddlers: A retrospective observational study in 34 patients.

BACKGROUND: The Petit-Morel method allows the treatment of developmental hip dysplasia in toddlers by combining gradual traction to achieve reduction followed by immobilisation during which pelvic osteotomy is performed. The objective of this study was to assess the radiographic and clinical outcomes in a retrospective cohort of patients. HYPOTHESIS: The Petit-Morel method is associated with low rates of avascular necrosis and residual acetabular dysplasia at skeletal maturity, as well as with satisfactory medium-term clinical outcomes. MATERIAL AND METHODS: We conducted a single-centre retrospective study of 34 patients (35 hips) treated between 1997 and 2014. The radiological assessment criteria included an evaluation for avascular necrosis classified according to Kalamchi and MacEwan, the vertical centre edge (VCE) angle, femoral head sphericity according to Mose, and acetabular dysplasia at skeletal maturity according to Severin. Hip function was assessed by determining the Postel-Merle d'Aubigné (PMA) score. RESULTS: Mean age at treatment was 19±4 months (range, 14-29). Mean follow-up was 11 years (range, 5-20). There were two failures including one case of recurrent dislocation requiring surgical reduction. Group II avascular necrosis occurred in 1 (3%) patient. Tönnis Grade IV dysplasia was significantly associated with resolving irregularity of the ossification centre, seen in 19 (54%) cases (p=0.002). In the 18 patients followed-up to skeletal maturity, with a mean follow-up of 15 years (range, 12-20 years), 17 hips were Severin Class I. The mean VCE angle was 29° (range, 15°-38°), and the head was spherical for 34 (98%) hips. The PMA score at last follow-up was excellent (17-18). The mean VCE angle was greater in all 5 patients who experienced pain during long walks (35° [range, 32°-37°]) than in the asymptomatic patients (28° [range, 15°-38°]) (p=0.009). DISCUSSION: The Petit-Morel method is a reliable treatment that provides good clinical and radiological outcomes. Overcorrection of the VCE angle was noted in the patients who experienced walking-related pain in adulthood. LEVEL OF EVIDENCE: IV, retrospective observational cohort study.

Fryns type mesomelic dysplasia of the upper limbs caused by inverted duplications of the HOXD gene cluster.

The HoxD cluster is critical for vertebrate limb development. Enhancers located in both the telomeric and centromeric gene deserts flanking the cluster regulate the transcription of HoxD genes. In rare patients, duplications, balanced translocations or inversions misregulating HOXD genes are responsible for mesomelic dysplasia of the upper and lower limbs. By aCGH, whole-genome mate-pair sequencing, long-range PCR and fiber fluorescent in situ hybridization, we studied patients from two families displaying mesomelic dysplasia limited to the upper limbs. We identified microduplications including the HOXD cluster and showed that microduplications were in an inverted orientation and inserted between the HOXD cluster and the telomeric enhancers. Our results highlight the existence of an autosomal dominant condition consisting of isolated ulnar dysplasia caused by microduplications inserted between the HOXD cluster and the telomeric enhancers. The duplications likely disconnect the HOXD9 to HOXD11 genes from their regulatory sequences. This presumptive loss-of-function may have contributed to the phenotype. In both cases, however, these rearrangements brought HOXD13 closer to telomeric enhancers, suggesting that the alterations derive from the dominant-negative effect of this digit-specific protein when ectopically expressed during the early development of forearms, through the disruption of topologically associating domain structure at the HOXD locus.

Characteristics and clinical outcomes after treatment of a national cohort of PCR-positive Lyme arthritis.

OBJECTIVES: To describe the clinical and microbiological characteristics and outcomes after antibiotic treatment of a national cohort of patients with Lyme arthritis confirmed by PCR testing on synovial fluid and by serology, when available. METHODS: Using the French National Reference Center for Borrelia database, patients with a positive PCR on synovial fluid for Borrelia were identified. Patient clinical and biological characteristics were reviewed from patient records. Long-term outcomes after treatment were studied through a questionnaire and with follow-up data. RESULTS: Among 357 synovial fluid testing by PCR between 2010 and 2016, 37 (10.4%) were positive for Borrelia. Patients' median age was 36 years (range 6-78) with 61% of men and 28% patients under 18. The presentation was monoarticular in 92% and the knee was involved in 97%. Contrary to the Borrelia species repartition in European ticks, B. burgdorferi sensu stricto was the most prevalent species found in synovial fluid (54%) followed by B. azfelii (29%) and B. garinii (17%). Antibiotic treatments were mainly composed of doxycycline (n = 24), ceftriaxone (n = 10) and amoxicillin (n = 6), for a median duration of 4 weeks (range 3-12). Despite a properly conducted treatment, 34% of patients (n = 12) developed persistent synovitis for at least 2 months (median duration 3 months, range 2-16). Among those, 3 developed systemic inflammatory oligo- or polyarthritis in previously unaffected joints with no signs of persistent infection (repeated PCR testing negative), which mandated Disease-Modifying Antirheumatic Drugs (DMARD) introduction, leading to remission. CONCLUSION: In France and contrary to ticks ecology, Lyme arthritis is mainly caused by B. burgdorferi sensu stricto. Despite proper antibiotic therapy, roughly one third of patients may present persistent inflammatory synovitis and a small proportion may develop systemic arthritis. In such cases, complete remission can be reached using DMARD.

Intercalary segmental reconstruction of long bones after malignant bone tumor resection using primary methyl methacrylate cement spacer interposition and secondary bone grafting: the induced membrane technique.

BACKGROUND: Bone reconstruction after surgical resection of malignant bone tumor in children remains a difficult challenge and various techniques exist. Induced membrane reconstruction as described by Masquelet et al has been reported in traumatic large bone defects. We have been using this 2-stage technique after primary malignant bone tumors resection in children since 2000. METHODS: We retrospectively studied 12 cases: 6 Ewing sarcomas and 6 osteosarcomas. Mean age of the patients was 9 years old (range, 3 to 15.5 y). Surgical treatment consisted of wide resection and insertion of a cement spacer then secondary bone grafting. All patients had neoadjuvant and adjuvant chemotherapy and 2 patients had adjuvant radiotherapy. RESULTS: Surgical excision was complete in all cases. There was no local recurrence at 6.2 years (range, 4.6 to 9.1 y) follow-up. Three patients had pulmonary metastasis (of whom 1 deceased) and 1 had a metastasis on the contralateral limb. The 11 patients operated on the lower limb achieved weight bearing 4.1 months (range, 0.2 to 14.2 mo) after the second stage of the procedure. Complications were numerous with 7 nonunions (4 unifocal and 3 bifocal), 5 fractures (in 4 patients), 5 protruding wires (in 4 patients), and 2 femoral varus deformities. There was no infection. CONCLUSIONS: Induced membrane reconstruction seems to be a simple and reliable technique in pediatric bone tumors and these results are promising. Extended use of locking nails could reduce the high rate of nonunion though it is not always possible in skeletally immature patients. LEVEL OF EVIDENCE: IV (case series).

Standard preoxygenation vs two techniques in children.

BACKGROUND: Preoxygenation is recommended in pediatric anesthesia but it has been poorly assessed. Fractional expired oxygen concentration (F(ET)O(2)) is a preoxygenation monitor. The aim of this prospective study in children was to compare three techniques of preoxygenation by the measurement of F(ET)O(2). METHODS: Twenty children (6-15 years) were included. F(ET)O(2) was measured with the child in a supine position, holding the face mask. The F(ET)O(2) value was measured after 3 min of calm breathing of room air (baseline) and during the three preoxygenation techniques performed in random order: 3 min of tidal volume breathing using an O(2) flow of 9 l x min(-1) (TV x 3 min)--four deep breaths within 30 s using an O(2) flow of 15 l x min(-1) (4 DB)--eight deep breaths within 1 min using an O(2) flow of 15 l x min(-1) (8 DB). Between each technique, at least 5 min breathing room air was allowed to return to baseline F(ET)O(2). Fisher's exact test was used and P < 0.05 was considered significant. RESULTS: Twenty children were studied (age: 11.5 +/- 3 years, weight: 42 +/- 21 kg). The F(ET)O(2) > or = 90% was found to be 79% in 74 +/- 40 s with TV x 3 min, 11% with 4 DB, and 68% with 8 DB. CONCLUSIONS: In children, Vt x 3 min is the most efficient preoxygenation technique to reach F(ET)O(2) > or = 90%.

[PEComa of soft tissue: report of an articular case]. / PECome des tissus mous: à propos d'un cas de localisation articulaire.

We report a case of PEComa (or perivascular epithelioid cell tumor) in an unusual articular localization in a 13-year-old boy. The tumor, of 4 cm in diameter, showed an infiltrative pattern and was composed of both epithelioid and spindle cells with clear to granular eosinophilic cytoplasm and some multinucleated giant cells. Focal nuclear pleomorphism was present and we found up to 2 mitotic figures /50 high power field. There was no necrosis. Immunohistochemistry showed HMB-45 and smooth muscle actin positivity. Ultrastructurally, premelanosomes were present. Some rare cases of PEComa were reported in the soft tissues. The immunohistological profile (HMB-45 and smooth muscle actin positivity and PS-100 negativity) is helpful to the diagnosis. The histological prognostic criteria of these tumors are not well established. We discuss here the differential diagnosis, notably clear cell sarcoma of soft tissue.

Roentgenographic measurement of angle between shaft and distal epiphyseal growth plate of radius.

To assess the accuracy of the use of the growth plate to shaft angle (GP-S) in the fractured distal radius, two prospective studies were performed. The first intraobserver study was made by three different observers who measured the GP-S angles of anteroposterior and lateral views of 62 wrist radiographs on two different occasions. The results showed a 95% concordance for a 5 degrees tolerance on anteroposterior views and 7 degrees on lateral views. The second, an interobserver study with six different observers, showed the same results. The results favored the use of the distal radius GP-S angle. It is an easy and accurate measurement that can be useful for the assessment of distal forearm deformities in children.