CCHS should be suspected in individuals presenting with unexplained hypoventilation and/or seizures after anesthetics or sedatives. This is the first report of LO-CCHS in a kindred with the PHOX2B 20/24 genotype. The incomplete penetrance observed in this family suggests a gene-environment interaction.
Developmental dysplasia of the hip (DDH) has been recognized to be a condition leading to osteoarthritis. Periacetabular osteotomy (PAO) has showed good results on hip preservation treatment for these cases. Nevertheless, intra-articular damage may be responsible for persistent post-operative symptoms, so treat the articular damage before or during the PAO has emerged as an alternative to address it. The objective is to identify the prevalence of intra-articular damage, functional outcomes of patients undergoing PAO with untreated intra-articular lesions and the survivorship free total hip arthroplasty (THA) at long-term follow-up. A retrospective review of 103 hips in 92 patients, mean age 26 years old (19–31), 96% females. Mean follow-up 7 years (range: 3–16). Intra-articular damage was evaluated with high-resolution magnetic resonance imaging (MRI) previous to perform the PAO, the chondral damage was evaluated using International Cartilage Repair Society classification. Harris Hip Score (HHS) was obtained in all patients. One hundred per cent of the cases had labral tears on MRI, hypertrophic labrum in 80.8% and paralabral cysts in 20.8%. Acetabular chondral damage was Grade 2 in 88.5% of the hips. HHS was good and excellent in 94%. Survivorship free of THA at 15 years was 87%. Chondrolabral damage is a common finding in patients with DDH. Despite that, excellent results are obtained with PAO without labral repair. We think the focus should be in the biomechanical and anatomical correction of the hip in patients with DDH.
Percutaneous iliosacral screw placement has become the technique of choice for treating injuries to the posterior pelvis. However, the technique requires an understanding of the anatomy surrounding the bone corridors to avoid complications and detect them early if they occur. We present the clinical case of a patient with a U-shaped fracture of the sacrum that evolves with gluteal pain and left foot equine paresis after percutaneous fixation with iliosacral screws. Angio-CT of the pelvis shows active arterial bleeding from the superior gluteal artery associated to extensive hematoma in the thickness of the gluteus medius muscle. Emergency embolization is performed by installing coil and gelatin. Successful control of bleeding is achieved. To avoid this complication, a complete imaging study is recommended in planning the surgery and to avoid multiple repositioning of the guide or screw. Arterial injury should be suspected in case of increasing pain despite analgesia, functional impairment or neurological deficit and the angiographic study and resolution by selective embolization of the bleeding vessels must be performed.
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