Background: Idiopathic genu valgum is a frequently diagnosed growth disorder in adolescence. Whenever the possibilities of conservative therapy have been exhausted, leg straightening by means of hemiepiphysiodesis has become the standard form of treatment. Because of their flexible screw-plate connection, eight-Plates have been reported in the literature to lead to lower complications regarding implant loosening and fracture compared to other implants. The aim of this retrospective survey was to analyse our own patient population who were treated for genu valgum by means of temporary hemiepiphysiodesis near the knee using eight-Plates to modulate growth. Methods: Between July 2007 and July 2015, 198 eight-Plates were implanted near the knee in 132 children suffering from genu valgum to modulate growth. Depending on the deformity analysis, an eight-Plate was implanted on the distal medial femur and/or the proximal medial tibia. By December 2015, they had been removed from 105 patients. The etiology of genu valgum was mainly idiopathic or associated withobesity. Evaluation was carried out clinically and radiologically (whole-leg X-ray in standing) including determination of the joint angles. Results: The median follow-up period was about 46 months (12-102 months). The median age at implantation was 12.7 +/−6.76 years. Of the 105 patients, 45.7% (n = 48) were girls. The eight-Plates remained in place for a median period of 13 +/−1.76 months. Irrespective of the location of hemiepiphysiodesis, the intermalleolar distance was corrected to a median of 0 +/−2.1 cm while the anatomical femorotibial angle was corrected by on average 9 +/−2.7°M echanical lateral distal femoral angle changed an average 7 +/− 7.72 degrees. Medial proximal tibial angle changed an average 4 +/− 6.02 degrees. Complications necessitating surgery occurred in 2.8% of cases (1 wound infection, 3 corrective osteotomies following overcorrection). Conclusion: Temporary hemiepiphysiodesis using eight-Plates is a gentle, simple and effective procedure used to treat genu valgum by modulating growth. Slight overcorrection is desirable due to the rebound phenomenon, especially in young patients with high growth potential and risk groups such as obese children. In adolescents with only low growth potential (older than 14 years), owing to the low correction potential, the indication should be strictly reviewed and the possible failure of therapy should be discussed with the patient. No differences were observed regarding the location of the implanted eight-Plates (femoral or tibial).
Surgical treatment with a DET screw seems to be a safe procedure for both the affected hip and the hip to be treated prophylactically. This method is an adequate alternative to the widespread technique of pinning with K‑wires.
In summary, the study shows that even with a late treatment start (U3) good results could be achieved, but with a rising number of repositioning failures and femoral necroses. Ultrasound screening on U3 seems to be sufficient; however, for high-risk groups an additional screening in the first week of life should be performed, which does not replace a second evaluation at U3 if there are normal findings.
Routine well-child visits, implemented as a means of secondary prevention and covered by health insurance, lead to early identification of disorders and abnormalities in child development."Guiding principles for children" (by the G-BA) have determined the content of the eleven examinations, ranging from U1 immediately after birth to J1 in adolescence; eight of them take place within the first four years of age. Since cases of child maltreatment, neglect, or abuse became public in 2007, almost all German federal states have established mandatory examination and notification processes in the new child welfare surveillance programs. First results in the German federal states (six of which are exemplarily illustrated) point out that mandatory requirements have collectively increased the frequency of medical check-ups in children, especially starting from four years of age and most significantly in families with social disadvantages (young/single parents, immigrant background, uneducated or socially disadvantaged families), which have so far been difficult to reach. Subsequently, provision of primary prevention (vaccinations and health promotion advice) by pediatricians has also increased. As a sole instrument for the complete identification of threats for children's welfare, however, systems inviting and reminding parents about check-ups are only of limited benefit.
Despite massive osteolysis with resorption of up to a whole bone segment, radiation with a dose of 22.8 Gy and the use of antiresorptive substances, as reported in our case, have often been shown to limit the destructive progression of the disease. A causal therapy of the disease is not known yet.
The authors give a report on their first results of percutaneous transluminal angioplasty of aortic arch branches in 4 patients. In 3 cases, stenosis of left subclavian arteries were treated by PTA with good result. One patient had a stenosis of both carotid arteries which were dilated with moderate effect. In all patients symptoms were removed. There were no complications.
RIPPLY2 is an essential part of the formation of somite patterning during embryogenesis and in establishment of rostro‐caudal polarity. Here, we describe three individuals from two families with compound‐heterozygous variants in RIPPLY2 (NM_001009994.2): c.238A > T, p.(Arg80*) and c.240‐4 T > G, p.(?), in two 15 and 20‐year‐old sisters, and a homozygous nonsense variant, c.238A > T, p.(Arg80*), in an 8 year old boy. All patients had multiple vertebral body malformations in the cervical and thoracic region, small or absent rib involvement, myelopathies, and common clinical features of SCDO6 including scoliosis, mild facial asymmetry, spinal spasticity and hemivertebrae. The nonsense variant can be classified as likely pathogenic based on the ACMG criteria while the splice variants must be classified as a variant of unknown significance. With this report on two further families, we confirm RIPPLY2 as the gene for SCDO6 and broaden the phenotype by adding myelopathy with or without spinal canal stenosis and spinal spasticity to the symptom spectrum.
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