We describe an unclassified overgrowth syndrome characterised by unregulated growth of dermal fibroblasts in the lower limbs of a 35-year-old woman. A gene mutation resulted in lower limb gigantism. Below the waist, she weighed 117 kg with each leg measuring over 100 cm in circumference. Her total adiposity was 50% accounted for by her legs mainly. Liposuction and surgical debulking were performed to reduce the size of the limbs but had exacerbated the overgrowth in her lower limbs. Systemic sepsis from an infected foot ulcer necessitated treatment by an above-knee amputation. Postoperatively, the stump increased in size by 19 kg. A trial of rapamycin to reverse the growth of the stump has shown promise. We discuss the clinical and genetic features of this previously unclassified disorder and the orthopaedic considerations involved.
Melorheostosis is a rare, nonhereditary, benign, mesenchymal condition of unknown aetiology affecting the bones and surrounding tissues. A male patient complaining of left shoulder pain, swelling, and mildly limited range of motion has an exclusive combination of the classic dripping wax lesion in the scapula and the myositis ossificans-like lesion in the deltoid muscle; this combination is the first to be reported in the shoulder. Both lesions showed typical findings of melorheostosis in radiographs, CT, MRI, and bone scan. This case has a stationary course over the follow-up period, and no specific treatment is needed in due course.
Introduction Guidelines for nasal injury state that assessment should be at 7–10 days post-injury and manipulation within 14 days. We performed a plan, do, study, act improvement cycle to assess whether a dedicated nasal fracture service led to better outcomes. Materials and methods A retrospective study was carried out of all patients undergoing manipulation under anaesthesia for nasal trauma between February 2013 and December 2016 in a district general hospital. A dedicated nasal fracture clinic providing manipulation under local anaesthesia was implemented followed by a prospective study of all patients presenting to the clinic between February and November 2017. Main outcome measures included time from injury to otolaryngology assessment, time from injury to manipulation and incidence of secondary septorhinoplasty. Results The retrospective series involved 525 patients including 381 males (72.6%) and 144 females (27.4%). Mean time from injury to assessment was 10 days. Mean time from injury to surgery was 14.5 days. Mean time from assessment to surgery was five days. The incidence of septorhinoplasty was 2.3%. The prospective series involved 119 patients including 78 males (65.5%) and 41 females (34.5%). Following implementation of a nasal fracture clinic, mean time from injury to assessment and manipulation was 6.1 days and 5.4% of patients underwent septorhinoplasty for secondary deformity. Discussion Implementation of a nasal fracture clinic providing reduction under local anaesthesia reduced the time to assessment and manipulation. The incidence of septorhinoplasty is low following reduction under general or local anaesthesia. Assessment earlier than seven days is feasible and advice for referral can be changed accordingly.
In contrast to laryngeal SCC treated by RT, TV does not appear to portend oncological outcome in glottic SCC managed specifically by TLM and consequently does not warrant incorporation into current prognostic models for such patients.
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