The association of three physical findings including capillary malformation, varicosities, and hypertrophy of bony and soft tissues corresponds to Klippel-Trenaunay syndrome. This triad of findings, described by the two French physicians Klippel and Trenaunay in 1900, differs from Parkes-Weber syndrome, in that Klippel-Trenaunay syndrome does not incorporate significant hemodynamic arteriovenous fistulas. Generally, management of this disease process should be individualized. Surgery should be considered in cases where skin ulcerations lead to persisting and recurrent bleeding, or where digital deformities lead to functional disabilities or where significant limb overgrowth leads to both functional and psychological impairment. Persistent hematochezia, hematuria, and vaginal and esophageal bleeding are considered indications for surgical intervention. Recurrent attacks of thrombophlebitis and cellulitis are treated medically with antiinflammatory agents and antibiotics. Otherwise, management of this syndrome is generally conservative, consisting of psychological encouragement, reassurance, and the continued use of graduated compressive stockings for varicosities and intermittent pneumatic compression pumps for lymphatic edema.

Microelectrode recordings in adult mammals have clearly demonstrated that somatosensory cortical maps reorganize following peripheral nerve injuries and functional modifications; however, such reorganization has never been directly demonstrated in humans. Using magnetoencephalography, we have been able to demonstrate the somatotopic organization of the hand area in normal humans with high spatial precision. Somatosensory cortical plasticity was detected in two adults who were studied before and after surgical separation of webbed fingers (syndactyly). The presurgical maps displayed shrunken and nonsomatotopic hand representations. Within weeks following surgery, cortical reorganization occurring over distances of 3-9 mm was evident, correlating with the new functional status of their separated digits. In contrast, no modification of the somatosensory map was observed months following transfer of a neurovascular skin island flap for sensory reconstruction of the thumb in two subjects in whom sensory transfer failed to occur.Understanding the presence, degree, and functional correlations for human cortical plasticity is of major significance for fundamental neuroscience as well as a potential aid in the diagnosis and rehabilitation of patients with peripheral and central nervous system injuries. That cortical maps can undergo reorganization in adult mammals has been demonstrated following a variety of peripheral sensory alterations including nerve transection (1-3), digital amputation (4,5) and syndactyly (6), and behavioral modification (7,8). Direct neurophysiological evidence for human somatosensory cortical plasticity, however, has never been demonstrated (9).Magnetoencephalography (MEG) is a noninvasive functional brain imaging technique that provides information regarding neuronal activity with high temporal and spatial resolution (10-14). Initial studies using MEG (10,12,14) demonstrated the somatotopy of the human somatosensory cortex, and present-day technology allows such functional maps to be defined with millimeter precision (14). We have been investigating the detailed functional organization of the hand area in normal adult humans and in patients with a variety of congenital and traumatic hand abnormalities. We report here evidence of adult human cortical plasticity, occurring over a time period of weeks to months following surgical correction of congenital syndactyly in two adult males. METHODSA 14-and a 37-channel biomagnetic recording system (Biomagnetic Technologies, San Diego) were used. The orientation of the subject's head in relation to the MEG recording probes was calculated using the sensor position indicator (SPI), a system of transmitters located on the dewars and receivers. affixed to a headband on the patient's head. Sensor locations were expressed using a head-based coordinate system whose origin was defined as the point of perpendicular intersection of the line extending from the nasion and the line connecting the penauricular points. The x, y, and z axes were the lines from t...

We retrospectively reviewed 26 patients who underwent reconstruction of the shoulder for a medial rotation contracture after birth injury of the brachial plexus. Of these, 13 patients with a mean age of 5.8 years (2.8 to 12.9) received an injection of botulinum toxin type A into the pectoralis major as a surgical adjunct. They were matched with 13 patients with a mean age of 4.0 years (1.9 to 7.2) who underwent an identical operation before the introduction of botulinum toxin therapy to our unit. Pre-operatively, there was no significant difference (p = 0.093) in the modified Gilbert shoulder scores for the two groups. Post-operatively, the patients who received the botulinum toxin had significantly better Gilbert shoulder scores (p = 0.012) at a mean follow-up of three years (1.5 to 9.8). It appears that botulinum toxin type A produces benefits which are sustained beyond the period for which the toxin is recognised to be active. We suggest that by temporarily weakening some of the power of medial rotation, afferent signals to the brain are reduced and cortical recruitment for the injured nerves is improved.

Eleven children ranging in age from 9 to 21 months underwent late nerve reconstruction for persistent shoulder paralysis following an upper brachial plexus birth injury. Only neurolysis was performed in three patients. Neurolysis and nerve grafting bypassing the neuroma with proximal and distal end-to-side repairs was performed in the other eight. All patients were followed for 2 or more years. Two patients underwent a secondary procedure before their final follow-up evaluation. All infants demonstrated significant improvement when assessed by a modified Gilbert shoulder motion scale.

Of 22 infants aged between 11 and 29 months who underwent a combined reconstruction of the upper brachial plexus and shoulder for the sequelae of a birth injury, 19 were followed up for two or more years. The results were evaluated using a modified Gilbert scale. Three patients required a secondary procedure before follow-up. Three patients had a persistent minor internal rotation contracture. All improved by at least two grades on a modified Gilbert scale.

Ninety-one infants who sustained a brachial plexus birth injury were treated with only physical and occupational therapy. The children were evaluated at 3-month intervals and followed for a minimum of 2 years. Sixty-three children with an upper or upper-middle plexus injury recovered good to excellent shoulder and hand function. In all of these children, critical marker muscles recovered M4 by 6 months of age. Twelve infants sustained a global palsy, with critical marker muscles remaining at M0-M1 at 6 months, resulting in a useless extremity. Sixteen infants with upper and upper-middle plexus injuries failed to recover greater than M1-M2 deltoid and biceps by 6 months, resulting in a very poor final outcome. These data provide useful guidelines for selection of infants for surgical reconstruction to improve ultimate outcome.

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In children younger than 18 months with brachial plexus injury, the MRI finding of pseudomeningocele has a low sensitivity and a high specificity for nerve root avulsion. MRI and MR myelography cannot image well the exiting nerve roots to determine consistently the presence or absence of avulsion of nerve roots. The MRI finding of post-traumatic neuroma has a high sensitivity and specificity in determining the side of the brachial plexus injury but cannot reveal the exact anatomic area (i.e. trunk or division) involved. The information obtained is, however, useful to the surgeon during intraoperative evaluation of spinal nerve integrity for reconstruction.