Normal and abnormal spine and thoracic cage development

Background Large plexiform neurofibromas can cause significant morbidity, functional impairment, and may be even life threatening. They most commonly arise in association with major nerve trunks and follow the distribution of the involved peripheral nerves, and can be subject to transformation into malignant peripheral nerve sheet tumors. They produce large infiltrative masses, which can involve major portions of the trunk or extremities. The main goal of this project is to analyze our surgical experience with plexiform neurofibromas excision and to describe our surgical technique that may help the surgeon to reduce intraoperative bleeding and facilitate tumor excision. Methods A retrospective chart and radiograph review was carried out for all cases of children with neurofibromatosis type 1 followed at our institution. Patients with plexiform neurofibromas of the trunk or extremities and who underwent surgical resection of the tumor were reviewed. Results Eleven female and 3 male patients with neurofibromatosis type 1 with 20 surgically treated lesions were evaluated. The mean age of the patients at time of surgery was 13.4 years (range, 2.9 to 19.1 y). The average follow-up was 65.5 months (range, 24 to 145 mo). Five lesions (25%) were surgically treated before the age of 10 years and 15 lesions (75%) were treated after the age of 10 years. Further debulking was required in 7 lesions and a below knee amputation was performed in 1 case. Conclusions The main goal of surgical resection of large lesions is to partially restore the normal appearance and function of the affected part and to ameliorate pain, when possible. One of the major complications of this particular surgery is the potential for significant blood loss. This complication seems to be particularly frequent in large plexiform neurofibromas located in those parts of the skeleton where the application of a tourniquet is not possible. Applying large retention sutures during dissection helps in minimizing bleeding and reduces the risk of complications. Level of Evidence Level IV, retrospective study. See the guidelines online for a complete description of level of evidence.

Idiopathic scoliosis is a growing spine disorder. Only a perfect knowledge of normal growth parameters such as bone age, stages of puberty, standing and sitting height, arm spam, Tanner stages, and weight allow the surgeon to plan the best treatment at the right moment. Measurements should be repeated and carefully recorded at regular intervals. Puberty is not a sudden event: it has a gradual onset and it is preceded by an incubation phase. It is not a point on a chart: it is a period that lasts two years beginning at a bone age of 11 and 13 years in girls and in boys, respectively. Two years of rapid growth (“acceleration phase”) are followed by three years of steady reduction of growth rates (“deceleration phase”). Skeletal maturation needs to be evaluated carefully to evaluate the progression risk of scoliosis during the phase of accelerating growth velocity. This article explains how to identify pubertal growth spurt, both clinically and radiographically, in order to correctly monitor patients with idiopathic scoliosis.

The purpose of this study was to compare the outcome of percutaneous curettage with intralesional injection of methylprednisolone and bone marrow for unicameral bone cysts (UBCs). This was a retrospective review of 46 children and adolescents with UBC treated with autologous bone marrow injection, methylprednisolone acetate injection or percutaneous curettage alone. Inclusion criteria were a radiological diagnosis of UBC and at least 24 months follow-up from the last procedure. Healing was determined using Neer/Cole 4-grades rating scale. The 3 treatment groups were comparable with regard to age, sex, location of the cyst, and the number of procedures undertaken. At 2 years follow-up, the proportion of patients with satisfactory healing (Neer/Cole grades I and II) was greatest among those who underwent percutaneous curettage (70%) compared with bone marrow injection (21%) and methylprednisolone acetate injection (41%) (P = 0.03). We found no association between healing and age (P = 0.80) nor between healing and sex (P = 0.61). These results suggest that mechanical disruption of the cyst membrane may be helpful in healing of cysts and that this technique may be preferred to simple intralesional injections. Level III.