Giant lipofibromatous hamartoma of median nerve in a child

Lipofibromatous hamartoma (LFH) is a rare fibrofatty tumor of adipocytes within peripheral nerves, affecting mainly children. It typically presents as a palpable mass surrounding the nerves of the upper limbs, causing pain and neurological deficits in the affected nerve distribution. We report the case of a child with a 2-years presentation of a mass in the right wrist associated with pain and paresthesia, who underwent investigation with magnetic resonance imaging (MRI). It showed thickening of the median nerve with spaghetti-like appearance associated with lipomatous tissue in a coaxial cable-like pattern, both features characteristic of LFH. This case illustrates the importance of MRI in the differential diagnosis of limb masses in the pediatric population.

Pulseless Supracondylar Humerus Fracture With Anterior Interosseous Nerve or Median Nerve Injury-An Absolute Indication for Open Reduction?

BACKGROUND: Optimal management for a pulseless supracondylar humerus fracture associated with anterior interosseous nerve (AIN) or median nerve injury is unclear. The purpose of this study was to determine the incidence of pulseless supracondylar humerus fractures associated with AIN or median nerve injury, to assess open versus closed surgical management, to determine factors associated with the need for neurovascular intervention, and to report the outcome. METHODS: A retrospective review was performed at 4 pediatric trauma hospitals on all patients who sustained a Gartland III or IV supracondylar humerus fracture with the combination of absent distal palpable pulses and AIN or median nerve injury between 2000 and 2014. Choice of treatment, details regarding preoperative and postoperative exam findings, follow-up course, and outcome were recorded. RESULTS: A total of 71 patients met inclusion criteria; 52 patients (73%) underwent closed reduction (CR); 19 patients (27%) underwent open reduction (OR) and early antecubital fossa exploration. The index procedure of CR plus percutaneous pinning was sufficient treatment in 50 (of 52, 96%) patients with only 2 requiring reoperation. One patient developed compartment syndrome approximately 9 hours after CRPP (13.5 h after time of injury) and underwent emergent fasciotomies. Of the 19 patients who underwent OR and early exploration, 6 needed vascular procedures, 5 required detethering of entrapped surrounding fibrous tissues. Forty patients were diagnosed with median nerve palsy versus 31 diagnosed with AIN palsy. There was no significant difference between patients presenting with median nerve versus AIN palsy, with similar rates of need for OR (10/40; 25% vs. 9/31; 29%), rate of compartment syndrome (3/40; 7.5% vs. 3/31; 9.7%), need for reoperation (4/40; 10% vs. 6.5%), and ultimate resolution of nerve palsy (4/36; 20.1% vs. 3/30; 10%). Compartment syndrome developed in 6 (of 71, 8.5%) patients and was associated with poor perfusion status on presentation and delayed time from injury to surgery. In patients with at least 3-month neurological follow-up, 59 (of 61, 97%) patients had complete resolution of nerve palsy. CONCLUSIONS: Although previous authors have suggested a pulseless SCH fx with an associated AIN or median nerve injury should be treated with exploration and OR, 70% (50/71) of the patients in this series were treated with a CR. In this series, both AIN and median nerve palsies among patients presenting with pulseless extremity and Gartland III or IV SCH fracture, offer similar rates of OR, risk of compartment syndrome, and resolution of nerve palsy. LEVEL OF EVIDENCE: Level IV.

Recurrent Median Nerve Injury in a Weight Lifter.

Recurrent median neuropathy is an uncommon injury that can be commonly mistaken for other pathological causes. It is important to be aware of the potential causes and symptoms of injury as delay in diagnosis can lead to permanent decrease in hand function and disability. This case is a description of a 33-year-old male patient who previously presented with radicular symptoms and had complete resolution of his pain after a C7 selective nerve root block. Several months after presentation, he developed significant thenar atrophy, localized pain, and weakness associated with weight lifting activities. He was originally diagnosed with a recurrence of his C7 symptoms and a wrist sprain and was treated with a prednisone taper and wrist splint. After no improvement, he was referred to a physiatrist at an outpatient orthopedic clinic where he was examined and found to have a recurrent median neuropathy on electrodiagnostic studies. A left hand MRI was ordered which demonstrated no anatomical evidence of a compressive nerve lesion, and it was concluded that his injury was secondary to direct compression from high intensity weight lifting.

Proximal median nerve entrapment caused by a distal biceps tendon cyst: an ultrasonographic diagnosis.

A 60-yr-old woman reported severe pain in the proximal part of her left forearm. The pain was also radiating toward the median nerve-innervated areas of the arm, especially during elbow flexion. Ultrasonographic imaging showed a well defined ganglion cyst around the distal biceps tendon close to the median nerve. Under ultrasound guidance, the cyst was aspirated and corticosteroid injection was performed. The patient's complaint improved 1 wk after the injection, and she started to flex her elbow without any pain. This case highlights the role of ultrasonography as a useful adjunctive tool not only to morphologically confirm a peripheral nerve entrapment but also to uncover the possible underlying etiology and to guide precisely during an intervention.

Lipofibromatous hamartoma of the median nerve: a comprehensive review and systematic approach to evaluation, diagnosis, and treatment.

Many modalities exist for diagnosing and treating lipofibromatous hamartoma (LFH), with no clear consensus. This is the first comprehensive study to review the existing literature on LFH of the median nerve and to suggest a systematic approach to its diagnosis and treatment. An electronic and manual search was conducted on Medline, Embase, Google Scholar, Current Contents, and Science Citation Index for original and review articles in English or French, from 1946 to November 2012. After 2 levels of screening, 106 references containing case reports were retained. Data extraction included patient demographics, clinical information, diagnostic modalities, treatment, and follow-up. A total of 180 cases were reported in the literature. One third of patients had associated macrodactyly (32%). Gender distribution is equal in LFH with or without macrodactyly, with most patients (71%) presenting before age 30 years. The main presenting symptom is an enlargement (88%) over the volar forearm, wrist, or hand, with or without digital hypertrophy, followed by paresthesia (39%). A soft, mobile, nontender, nonfluctuant mass with variable degree of compressive median neuropathy is found on physical examination. Biopsy, which reveals abundant mature fat cells and fibrous connective tissue infiltrating between nerve fascicles and the space between the epineurium and the perineurium, is not necessary because the pathognomonic features of the mass on magnetic resonance imaging offer an accurate diagnosis. Treatment of nerve compression symptoms and macrodactyly should be addressed separately. Carpal tunnel release is the mainstay of treatment for neuropathy, and ray or digital amputation, wedge osteotomy, middle phalangectomy with arthroplasty, and epiphysiodesis are suggested options in the management of macrodactyly. Based on our review of the literature, we propose an algorithm for the diagnosis and treatment of LFH of the median nerve with or without macrodactyly.

Pronator syndrome and anterior interosseous nerve syndrome.

Dysfunction of the median nerve at the elbow or proximal forearm can characterize two distinct clinical entities: pronator syndrome (PS) or anterior interosseous nerve (AIN) syndrome. PS is characterized by vague volar forearm pain, with median nerve paresthesias and minimal motor findings. AIN syndrome is a pure motor palsy of any or all of the muscles innervated by that nerve: the flexor pollicis longus, the flexor digitorum profundus of the index and middle fingers, and the pronator quadratus. The sites of anatomic compression are essentially the same for both disorders. Typically, the findings of electrodiagnostic studies are normal in patients with PS and abnormal in those with AIN syndrome. PS is a controversial diagnosis and is typically treated nonsurgically. AIN syndrome is increasingly thought to be neuritis and it often resolves spontaneously following prolonged observation. Surgical indications for nerve decompression include persistent symptoms for >6 months in patients with PS or for a minimum of 12 months with no signs of motor improvement in those with AIN syndrome.

The evaluation and treatment of elbow injuries.

This article reviews elbow injuries that occur at the medial, lateral, anterior, and posterior aspects, including pediatric elbow injuries. A description, imaging, anatomy (where appropriate), mechanism of injury, physical examination, diagnostics, differential diagnosis, management, cause for referral to an orthopedic surgeon, controversies, and advancements in management for each type of injury are included.

[Proximal median nerve compression. A series of 35 consecutive cases]. / Compressions du nerf médian dans la région du coude et de la partie proximale de l'avant-bras. À propos d'une série de 35 cas consécutifs.

Median nerve entrapment at the elbow and the proximal forearm represents 7 to 10 % of median nerve mononeuropathies. Literature distinguishes two distinct syndromes: the pronator syndrome and the anterior interosseous nerve syndrome. We report a retrospective series of 35 cases of proximal compression of the median nerve, including a previous study of 13 cases assessed in 2001. Thirty-four patients were operated on between 1994 and 2011. The series included 15 men and 19 women with a mean age of 57 years. Subjective complaints were the main reason of consulting with or without a deficit. All but one benefited from a preoperative electrical study. Neurography showed abnormalities in 18 cases and myography in 30 cases. At least one site of compression was found at surgery. Thirty-one cases, including nine of the 13 cases previously evaluated in 2001, were assessed with a mean follow-up of 69 months. Twenty-height considered them improved and all but one were objectively improved by surgery. The nine cases evaluated in 2001 had better results in 2011. Through this series and an exhaustive literature review, we concluded that there are no preoperative criteria that can differentiate a pronator teres syndrome from an anterior interosseous nerve syndrome. If no improvement occurs, surgical treatment should be proposed, one must then assess all potential sites of nerve entrapment. Patients must be informed that improvement can take several years.

Nerve injuries about the elbow.

The ulnar, radial, median, medial antebrachial cutaneous, and lateral antebrachial cutaneous nerves are subject to traction and compression in athletes who place forceful, repetitive stresses across their elbow joint. Throwing athletes are at greatest risk, and cubital tunnel syndrome (involving the ulnar nerve) is clearly the most common neuropathy about the elbow. The anatomy and innervation pattern of the nerve involved determines the characteristic of the neuropathy syndrome. The most important parts of the work-up are the history and physical examination as electrodiagnostic testing and imaging are often not reliable. In general, active rest is the first line of treatment. Tailoring the surgery and rehabilitation protocol according to the functional requirements of that athlete's sport(s) can help optimize the operative outcomes for recalcitrant cases.

Long-term observation of auto-cell transplantation in non-human primate reveals safety and efficiency of bone marrow stromal cell-derived Schwann cells in peripheral nerve regeneration.

Based on their differentiation ability, bone marrow stromal cells (MSCs) are a good source for cell therapy. Using a cynomolgus monkey peripheral nervous system injury model, we examined the safety and efficacy of Schwann cells induced from MSCs as a source for auto-cell transplantation therapy in nerve injury. Serial treatment of monkey MSCs with reducing agents and cytokines induced their differentiation into cells with Schwann cell properties at a very high ratio. Expression of Schwann cell markers was confirmed by both immunocytochemistry and reverse transcription-polymerase chain reaction. Induced Schwann cells were used for auto-cell transplantation into the median nerve and followed-up for 1year. No abnormalities were observed in general conditions. Ki67-immunostaining revealed no sign of massive proliferation inside the grafted tube. Furthermore, (18)F-fluorodeoxygluocose-positron emission tomography scanning demonstrated no abnormal accumulation of radioactivity except in regions with expected physiologic accumulation. Restoration of the transplanted nerve was corroborated by behavior analysis, electrophysiology and histological evaluation. Our results suggest that auto-cell transplantation therapy using MSC-derived Schwann cells is safe and effective for accelerating the regeneration of transected axons and for functional recovery of injured nerves. The practical advantages of MSCs are expected to make this system applicable for spinal cord injury and other neurotrauma or myelin disorders where the acceleration of regeneration is expected to enhance functional recovery.

A split and conjoined median nerve in the forearm.

We observed a variation of the median nerve that split into two divisions in the proximal forearm and conjoined into a single cord in the distal forearm before entering the carpal tunnel. The medial division was larger than the lateral division. An aberrant muscular slip from the flexor digitorum superficialis ran over the medial division of the split median nerve. The muscular slip over the medial division might have caused an entrapment syndrome of this nerve due to several muscular and cutaneous branches originating from the medial division. This variation of the median nerve has not been reported to our knowledge and may affect diagnosis and treatment of any neuropathy of the median nerve.

Lipofibromatous hamartoma of the median nerve. Three case reports.

Nervous lipofibromatous hamartoma is a rare tumour-like condition involving the peripheral nerves, whereby the epineurium and perineurium are enlarged and distorted by excess fatty and fibrous tissues that infiltrate between and around nerve boundaries. The median nerve is much more likely than other nerves to develop a hamartoma with a predilection for the carpal tunnel. In this article, we describe three cases of lipofibromatous hamartoma of the median nerve and discuss its management.

[Dupuytren's disease simulated by epithelioid sarcoma with atypical perineural invasion of the median nerve. Case report]. / Malattia di Dupuytren simulata da un sarcoma epitelioide con atipica invasione perineurale del nervo mediano. Caso clinico.

Epithelioid sarcoma is an infrequent tumour, especially in upper limb, which nerve spread is not common. We describe the case of a 27 years old caucasian male with initial diagnosis of Dupuytren's disease. He underwent palmar fascia surgery, but the surgical treatment was followed by recurrence of the deformity in retraction of 4th and 5th finger of the left hand. When he presented himself at our attention, the patient underwent surgical biopsy and new histological examination was made with diagnosis of epithelioid sarcoma. We highlight essentially two aspects of this case: the diagnostic delay of tumour caused by simulation of the Dupuytren's disease and the atypical perineural spread along the median nerve.

Ulnar dominant hand and forearm: an electrophysiologic approach.

An ulnar-to-median anastomosis in the forearm is a rare condition, but may be present in any electromyographic case. A thorough approach to this condition is required to avoid misinterpretation of the electrodiagnostic report and confusion during the test. Prior to concluding that an anomaly is present, technical reason should be taken into consideration. The presence of volume-conducted potentials from various nearby muscles may confuse the electromyographer. Therefore, instead of using surface electrodes with unintended supramaximal intensity of stimulation, the needle electrodes may be used (in some cases) to localize specific muscles and to minimize volume-conducted potentials by not utilizing supramaximal stimulation intensity. The authors will discuss ulnar-to-median anastomosis in the forearm. This is the first attempt to put together all the information available in the literature about such an anastomosis.

[Anterior interosseous nerve lesions revisited in 2004]. / Les atteintes du nerf interosseux antérieur en 2004.

When the anterior interosseous nerve (AIN) is involved, a motor deficit of the distal pinch appears. It usually occurred suddenly, with or without pain and the deficit may be complete or partial. Other nerve lesions and some precipitating conditions can be noticed and are important to assess the exact mechanism of the nerve lesion. Partial AIN lesion is frequently misdiagnosed as tendon rupture. The electrodiagnostic examination should always study the pronator quadratus or another muscle innervated by the AIN, the AIN conduction and the motor and sensory median nerve conduction. Thus electrodiagnosis will always assess the AIN lesion and precise its axonal or compressive mechanism. Mononeuritis as neuralgic amyotrophy is the most frequent cause of AIN palsy and never requires surgery. Traumatic lesions are less frequent and usually recover spontaneously. Thus, surgery is rarely required before 12 to 18 months except when no recovery occurs after trauma.

Concomitant compression of median and ulnar nerves in a hemophiliac patient: a case report.

A 15-year-old boy, with a diagnosis of hemophilia A, suffered bleeding into his left forearm 5 months before being admitted to our medical center. His neurological examination revealed a pronounced median neuropathy and a minor ulnar neuropathy on the left side. There was marked muscle atrophy on the thenar side and, to a lesser degree, on the hypothenar side and in the forearm. Electromyographic findings demonstrated an evident, nearly complete, sensorimotor axonal loss in the median nerve. Magnetic resonance imaging studies showed atrophy in muscles of the left forearm and median nerve. The patient was diagnosed as having median nerve axonotmesis and ulnar nerve neuropraxia due to compartment syndrome. In hemophiliac patients, frequent single nerve compressions (often involving the femoral nerve) can be seen. However, concomitant median and ulnar nerve injuries with differing severity are rare.