Introduction: Congenital insensitivity to pain and anhydrosis (CIPA) is a rare genetic disease transmitted through an autosomal recessive mode. It is known also as HSAN (Hereditary Sensory and Autonomic Neuropathy) type 4. Affected patients suffer from: anhydrosis, mental retardation, poikilothermia and musculo-skel-etal anomalies. The actual knowledge on musculo-skel-etal aspects in CIPA is based on case reports.

Aim of the study: To describe systematically the musculo-skel-etal aspects related to CIPA in a large group of patients followed over the years.

Material and Methods: 40 patients with CIPA were followed in our institution. The age range was 3 months to 19 years of age, and the mean follow up was 8 years. There was some degree of relationship between the parents Their charts were reviewed, radiographs, and bacterial cultures were examined.

Results: The main features that we observed: a) Joint instability with a spectrum that varies from positive provocative test to recurrent dislocation. b) Bone and Joint infection-often with high production of purulent discharges and associated with subluxation of affected joint or with pathologic fractures. Infections can be multimicrobial, are difficult to eradicate and lead to bacteremia episodes. c) Wound healing problems. Wounds hardly heal in CIPA patients. The scar is formed slowly if at all. Chronic sinus drainage and frequent wound dehiscence is the rule. d) Radiological abnormalities: osteomyelitis, pathological fractures with giant callus formation, vanishing bones, heterotrophic ossification, and pseudo-arthrosis were observed.

Conclusions: There is a wide spectrum of musculo-skel-etal pathologies in CIPA affected children. Their orthopedic conditions determine ambulation capacity, life quality and life expectancy and influence dramatically on their families. Complications both mechanical and infectious are very often. A multidisciplinary approach to this chronic illness is needed.

Medial transfer of the tibial Tuberosity remains the treatment of choice for skeletally mature patients with patellar malalignment (recurrent dislocation, subluxation with or without patellar tilt). As many patients with patellar malalignment have patellar articular cartilage lesion or patella alta, anteriorisation and distalisation of the tibial tuberosity is advised.

Material and Methods: Tibial tuberosity transfer was performed in our center on 80 knees in 66 patients (40 females, 26 males) during the past 13 years (mean age 23 range 15 – 52). One surgeon supervised all the operations. The average follows-up was 6.2 years (one to thirteen years). All patients were examined clinically for the purpose of this study. The Lisholm and Karlsson scoring system were used to evaluate the results. Radiographs of both knees were also taken. Fifteen knees had no dislocation of the patella prior to the operation, seventeen knees had 1 to 10 eleven knees had 10 to 50, ten knees 50 to 100, and twenty-seven knees had more than hundred dislocations of the patella prior to surgery. Ten of these knees had daily dislocations of the patella.

All operations were done either by selective epidural anesthesia (only sensory and not motor) or general anesthesia without muscle relaxant using quadriceps muscle stimulation. The mean tibial tuberosity medialisation, anteriorisation and distalisation was 1.4 cm (0–2.5 cm) 0.4cm (0–1.1cm) and 0.87cm (0–1.2cm) respectively.

Results: When interviewed by an independent examiner 87% of the patients reported improvement and only 4.3% (3 patients) reported worsening of their condition after the operation.

84% of the patients stated they would have the operation again. All patients had full active range of motion on both knees without extension lag.

At the final evaluation visit the Lisholm and Karlsson scores were good and excellent in 72% and 72.5%, 18.8% and 23.5% had fair results and only 8.7% and 4.4% had poor results respectively. The poor results correlated well with the degree of the patella cartilage damage found during surgery, poor selection of patients and extreme ligamentous laxity. There were two complications: one non-union of the tibial tuberosity treated successfully with bone grafting and one non displaced fracture bellow the osteotomy, treated conservatively. Both had excellent results.

Conclusion: We conclude that distal patella re-alignment done by tibial tuberosity transfer is a reliable technique for the treatment of patello femoral pain secondary to mal-alignment.

Introduction: Meralgia paresthetica (MP) is a mononeuropathy of the lateral femoral cutaneous nerve, mainly associated with either injury or pressure to the nerve. Although this entity is well described, most clinical series have employed numerous treatment methods; therefore, the efficacy of individual modalities is unclear.

We describe our experience of 79 patients with complaints consistent with MP, treated, by the senior author, over a 13-year period.

Materials and Methods: Patients were managed in accordant to a structured protocol:

Initial management consisted of anti inflammatory agents, rest and redaction of aggravating factors.

Diagnostic nerve block test was carried out for those who were refractory to the above treatment. All patients who responded to the local anesthetic test were treated with local infiltration of corticosteroids.

Surgical intervention was reserved for patients who responded to the lidociane test but were refractory to repeated corticosteroids injections. Patients who failed to respond to the test injection were evaluated by CT-scan of the lumbar spine and by abdominal ultrasound (for female patients only). Follow-up ranged 1–13 years.

Results: Twenty-one patients (27%) reported satisfactory results flowing initial management. Local anesthetic yielded rapid relief of symptoms in 50 patients (86%), two additional patients responded to a second nerve block test (where a wider area was infiltrated).

In forty-two out of 52 patients (81%) who responded to the nerve block test and received treatment with corticosteroid injections, Long-lasting relief was obtained.

Three patients refractory to repeated injections of corticosteroid underwent surgery (neurolysis in one patient and nerve resection in two).

CT-scan of the lumbar spine revealed significant spinal stenosis and nerve root compression at the level of L1–3 in 3 out of 6 patients.

Conclusion: Idiopathic MP usually improves with nonoperative modalities. Temporary relief of symptoms following LFCN block is a specific diagnostic test, and is a good predictor of success following local corticosteroid injection. Surgery is seldom necessary and should be considered only if intractable pain persists despite such measures.