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Multiple vascular malformations in the blue rubber bleb nevus syndrome: a case with aneurysm of vein of Galen and vascular lesions suggesting a link to the Weber-OslerRendu syndrome. Multiple subcutaneous hemangiomas, together with multiple lipomas, occurring in enormous numbers in an otherwise healthy, muscular subject. A study of 459 cases of lipoma with review of literature on infiltrating angiolipoma. Pharyngeal traumatic neuromas and traumatic neuromas with mature ganglion cells (pseudoganglioneuromas). Oral traumatic neuroma with mature ganglion cells: a case report and review of the literature. Comparative light microscopic and immunohistochemical study of traumatic and palisaded encapsulated neuromas of the skin. Traumatic neuroma of the oral cavity: report of thirty-one new cases and review of the literature. Palisaded encapsulated neuroma (solitary circumscribed neuroma of skin) of the eyelid: report of two cases and review of literature. Multi-focal diffuse glomus tumor: a case report of glomangiomyoma and review of the literature. Cutaneous muscle neoplasms: Clinical features, histologic findings, and treatment options. Clinical features of multiple cutaneous and uterine leiomyomatosis, an underdiagnosed tumor syndrome. Familial cutaneous leiomyomatosis is a two-hit condition associated with renal cell cancer of characteristic histopathology. On evaluation, the patient had multiple papules around her mouth as well as on her groin. Further history and physical found the patient to have features consistent with Goltz syndrome. Pathology confirmed focal dermal hypoplasia, papillomas of the mouth, and condyloma of the genitals. Patents with Goltz syndrome commonly develop papillomas in the oral and genital region. Given the widespread lesions, our patient presents a diagnostic and treatment conundrum. We review the recent literature on Goltz and discuss the therapeutic challenge presented in our patient. Patient History We present a 27-year-old female referred to our dermatology clinic by her primary physician for warts of the mouth and genitals. Our patient reported lesions around her mouth present for approximately one year and lesions in her genital area for over three years. She reports no symptoms of her oral lesions, and describes itching and burning of the lesions in the genital area. The patient did report being rather promiscuous in her late teens and early twenties. On physical exam, the patient was noted to have two 4mm, flesh-colored, verrucous papules on the left commissure, with a few scattered 2mm pink papules periorally. There were many 2-8mm, flesh-colored, pedunculated and verrucous papules scattered bilaterally in the genital area and inner thighs. Further examination noted atrophic, telangiectatic plaques with soft, orange nodules in a Blaschko distribution on both the arms and legs. When asked if she had ever been given a diagnosis of Goltz syndrome, the patient was unable to recall but remembered being told she had "some disorder" when she was young. The two larger papules on the mouth were biopsied, and two representative lesions in the groin, one from the thigh and one from the labia majora, were biopsied with a ddx of condyloma acuminata vs. The histopathology of the oral lesions showed acanthosis with fibrous submucosa, and no viral changes were noted.

For those trained in joint manipulation techniques, they are effective for selective facet joint stretching and have been found to be an effective part of a total treatment approach when there is instability in specific areas and restricted mobility in neighboring facet joints. If there are bony changes and osteophytic spurs, the patient should avoid postures and activities of hyperextension, such as reaching or looking overhead for prolonged periods of time. Adaptations in the environment might include using a stepstool so reaching is at shoulder level. Postures and motions emphasizing flexion of the spine that increase the size of the intervertebral foramina are usually preferred. Because of the potential instabilities from necrotic tissue and bone erosion, subluxations and dislocations may cause damage to the spinal cord or vascular supply and be extremely debilitating or life-threatening. Some of the patients may have a history of trauma, repeated manipulations, or early signs of spondylosis. Mobility testing of the spinal segments reveals increased mobility at one or more segments. There may be decreased activity in the stabilizing musculature, particularly in response to postural perturbations, and there may be faulty respiratory patterns. Correction of Meniscoid Impingements If there is entrapped synovial or meniscoid tissue in a facet joint that blocks motion into extension, release of the trapped meniscoid relieves the pain and the accompanying muscle guarding. Cervical Traction, Mobilization, Manipulation Traction to the spine may be applied manually or mechanically. Techniques of manual traction, self-traction, and positional traction with rotation are described in the stretching section of Chapter 16. Traction applied longitudinally along the axis of the spine has the effect of sliding the facets joint surfaces and thus places tension on the facet capsules. Traction with side bending and rotation of the spine has the effect of distracting the facet joint surfaces as well as placing tension on the capsules. See also the mobilization section later in this chapter Management When Acute Symptoms Have Stabilized General guidelines for subacute and chronic spinal prob- Identification of Clinical Instability Stress radiographs are typically used by the medical profession to identify instability. Those with more than 4 mm of translation or 10 of rotation are considered candidates for surgery. To identify impairments in the musculature and the ability to control movement, techniques have been developed that specifically address core muscle activation and endurance and global muscle stabilization. Patients may demonstrate difficulty moving smoothly in the mid-ranges and demonstrate a shifting or fluctuation in movement. In the lumbar region it is possible to palpate the transversus abdominis and multifidus muscles while the patient attempts to contract them. Devices to measure activation, such as using a biopressure feedback unit or ultrasound imaging, have been developed for both research and clinical usage43 (see next section under Principles of Management as well as Chapter 16). Several protocols have been developed to test the stabilizing function of the global musculature. Progression of Stabilization Exercises Progressing from core muscle activation to general stabilization exercises using the global musculature emphasizes cervical and pelvic control while superimposing extremity motions. Included are weight-bearing activities such as wall slides, partial lunges, and partial squats, with emphasis on the "drawing-in" maneuver and spinal control in the neutral spinal position while doing the activities. The patient is encouraged to activate the core musculature consciously and maintain a neutral spinal position until it becomes habitual. The traction procedures described in the non-weight-bearing section earlier in this chapter may also be beneficial. Principles of Management Passive Support Braces or corsets may be necessary for external support to provide stability and reduce pain. Core Muscle Activation Activation of core musculature may not be automatic in patients with pain or instability. Techniques used to instruct patients in addition to verbal and tactile cues include use of a biofeedback pressure cuff (Chattanooga) and ultrasound imaging. Ultrasound imaging is primarily used in research settings because of the cost of the units. The pressure cuff has been shown to have clinical relevance in providing immediate feedback to patients. Once the patient learns to activate the core muscles, emphasis is placed on sustaining the contraction over a period of time and on increasing the repetitions of the static hold to reinforce the postural function. These contractions are of low intensity to minimize the compressive activity of the global muscles.

When full passive range of extension is available, but the person cannot actively move the joint through the full range of extension, it is called an extensor lag. It can occur as the result of weakness but is frequently caused by adhesions that prevent gliding of the tendons when the muscles contract. One of the purposes of the following exercises is to maintain mobility and thus prevent adhesions. Mobi- C H A P T E R 1 9 the Wrist and Hand 633 lization of adhesions is described immediately following the differential gliding of extensor tendon exercises. Then instruct the patient to attempt to actively keep the fingers against the table while one of the digits is passively flexed. Progress by having the patient actively maintain the fingers in extension with the fingers spread out and then actively flex each finger in turn while the other fingers remain extended. Have the patient flex the middle and ring fingers while maintaining extension of the index and little fingers (long horn sign). This promotes isolated control of the extensor indicis and extensor digiti minimi tendons and promotes their gliding on the extensor digitorum communis tendons. Scar Tissue Mobilization for Tendon Adhesions Ideally, the tendon-gliding exercises described previously in this section maintain or develop mobility between the long tendons and surrounding connective tissues or within their sheaths. However, when there has been inflammation and immobilization during the healing process following trauma or surgery, scar tissue adhesions may form and prevent gliding of the tendons. Contraction of the muscle does not result in movement of the joint or joints distal to the site of the immobile scar. Techniques to mobilize the adhesive scar tissue include the application of friction massage directly to the adhesion. This is superimposed on active and passive stretching techniques (described in the next section), and the tendon-gliding techniques already described. To apply friction massage, hold the tendon in its lengthened position; apply pressure with your thumb, index, or middle finger and massage perpendicular to the tendon and longitudinally in proximal and distal directions. A sustained force against the adhesion allows for creep and eventual movement of the scar. To Mobilize the Long Finger Flexor Tendons Adhesions between the flexor tendons and their sheaths or between tendons and underlying bones restrict tendongliding in both a proximal and distal direction so the joints distal to the scar do not flex when the muscle contracts. Extensor Tendon-Gliding Exercises Differential gliding of the extensor digitorum communis tendons to each of the fingers can be achieved by the following progression. If the patient has difficulty doing this, begin with the involved hand resting on a table with the palm up. Full range of extension of the joints distal to the scar is not possible actively or passively owing to the inability of the tendon to glide distally. The following is a suggested progression in intensity of scar tissue mobilization. Begin the stretching routine by passively moving the tendon in a distal direction by extending the finger joints as far as possible and apply a sustained hold to allow for creep. Follow this with active contraction of the flexor muscle to create a stretch force against the adhesion in a proximal direction109 using the patterns of movement described for tendon-gliding exercises. When applying friction massage in a proximal direction, ask the patient to simultaneously contract the flexor muscle in order to superimpose an active stretch force. After friction massage, have the patient repeat the flexor tendon-gliding exercises to utilize any gained mobility. To Mobilize the Extensor Tendons and the Extensor Mechanism As with the flexor tendons, if the extensor tendons or extensor mechanism has restricted mobility because of adhesions, muscle action is not transmitted through the mechanism to extend the joint or joints distal to the restriction. As defined earlier, an extensor lag is the loss of active extension when there is full passive extension. Stretch the adhesion in a distal direction by passively flexing the joint distal to the site. Follow this by having the patient attempt to actively extend the joint and put tension on the scar in a proximal direction.

The delayed weight-bearing group was advised not to wear a shoe on the operated side and remain non-weight bearing during ambulation with crutches for the first 2 weeks. After that, there were no restrictions placed on the progression of weight bearing. With the exception of weight-bearing status, the rehabilitation program for all patients was the same. However, patients in the immediate weight-bearing group had a lower incidence of anterior knee pain than patients in the delayed weight-bearing group (8% and 35%, respectively). The investigators concluded that immediate weight bearing did not compromise knee joint stability or function and was beneficial in that it resulted in a lower incidence of postoperative anterior knee pain. However, recommendations for a period of protected weight bearing immediately after surgery vary, ranging from some degree of restricted weight bearing the first 2 weeks to weight bearing as tolerated with use of two crutches immediately after surgery. Although a valid mechanism for controlling postoperative pain and initiating early motion,48,134,169,211 it is used less frequently today than in the recent past. If protective bracing has been prescribed, exercises are carried out while wearing the brace. For example, patient-related facts, such as age and preinjury health status, affect the healing process, enabling younger, healthier patients to progress exercises more rapidly. The type of graft and graft fixation also may influence the progression of exercise. Some resources advocate more rapid progression of exercise for bone-to-bone fixation with a patellar tendon graft than for tendon-to-bone fixation with a quadrupled hamstring graft, suggesting that bone-to-bone healing may be faster than soft tissue-tobone healing. Early motion places beneficial stresses that strengthen the graft but must be carefully controlled to avoid stretching the graft while in a weakened state, particularly during the first 6 to 8 weeks after implantation. The following goals and exercise interventions are emphasized during the first 4 weeks after surgery when considerable protection of knee structures is required. Patient education during the first phase of rehabilitation focuses on the home exercise program. When weight-bearing exercises are initiated, they are performed in a protective brace if one has been prescribed. Low-intensity closed-chain exercises and proprioceptive/ neuromuscular control training are initiated as soon as weight bearing is permissible. All patients wore a protective brace and ambulated with crutches, bearing weight as tolerated. When strengthening exercises were initiated, one group followed an open-chain regimen and the other a closed-chain regimen. One year after surgery 66% of patients participated in a follow-up examination that included subjective and objective measurements; it was conducted by someone blind to group assignment. Patients in the closed-chain exercise group compared with the open-chain group had significantly less anterior knee pain, knee stability closer to normal as measured by an arthrometer, earlier return to functional activities, and greater overall satisfaction with the outcome of the surgery. Add external resistance when the patient is able to maintain knee control during hip movements. Initiate low-intensity, multipleangle isometrics of the knee musculature with emphasis on quadriceps control. To activate the hamstrings dynamically include supine heel-slides to a comfortable level of hip and knee flexion, active knee flexion in a standing position (hamstring curls without resistance added), and scooting forward while seated on a rolling stool. To increase passive knee extension, assume a supine or long-sitting position and prop the heel on a rolled towel or bolster with the knee unsupported. Neuromuscular control, proprioception, and dynamic stability of the operated lower extremity. While wearing a protecitve brace, if prescribed, begin trunk and lower extremity stabilization exercises in a standing position with weight distributed equally on both lower extremities and some weight on the hands for support, progressing to bilateral mini-squats in the 0 to 30 range and weight-shifting, stepping, and marching movements. When the knee is pain-free and full weight bearing is possible, begin unilateral activities.

The central and proximal lymphatic vessels, such as the abdominal, inguinal, and cervical nodes. Then, for the most part, exercises proceed distally from shoulders to fingers or from hips to toes. N O T E: Because no single sequence of exercises has been shown to be more effective than another, the upper and lower extremity sequences of exercises outlined in this sec- tion do not reflect the exercises included in any one specific protocol. Rather, the exercise sequences are based on the recommendations of several authors. Therapists are encouraged to modify or add other exercises to the sequences in this chapter as they see fit to meet the individual needs of their patients. Exercises Common to Upper and Lower Extremity Sequences these initial exercises should be included in programs for unilateral or bilateral upper or lower extremity lymphedema. They are designed to help the patient relax and then to clear the central channels and nodes. Then, isometrically contract and relax the muscles of the lower trunk (abdominals and erector spinae) followed by the hips, lower legs, feet, and toes. N O T E: If lymphedema is present in only one lower extremity, initiate the knee-to-chest exercises with the uninvolved lower extremity. With arms at sides and elbows flexed, bilaterally retract the scapulae, pointing elbows posteriorly and medially. N O T E: Be sure to shrug the shoulders as high as possible and then actively pull down the shoulders (depress the scapulae) as far as possible Exercises Specifically for Upper Extremity Lymphedema Clearance the following sequence of exercises is performed after the general, total body exercises just described. The exercises, which are performed in a proximal to distal sequence, are done specifically for upper extremity lymph clearance. N O T E: Periodically during the exercise sequence have the patient perform self-massage to the axillary node area of the uninvolved side proceeding from the axilla to the chest. While lying supine, flex the involved arm to 90 (reach toward the ceiling) and perform active circular movements of the arm about 6 to 12 inches in diameter. P R E C A U T I O N: Avoid pendular motions or circumduction of the edematous upper extremity with the arm in a dependent position. While lying supine on a firm foam roll (approximately 6 inches in diameter), perform horizontal abduction and adduction as well as flexion and extension of the shoulder. For home exercises, if special equipment such as an Ethyfoam roller is not available, have the patient perform these exercises on a foam pool "noodle. With arms elevated to shoulder level or higher and the elbows flexed, place the palms of the hands together in front of the chest or head. Press the palms together (for an isometric contraction of the pectoralis major muscles) while breathing in for a count of 5. Incorporate several exercises to increase shoulder mobility and to decrease congestion and assist lymph flow in the upper extremity. The following exercises are done with the patient seated and the arm supported at shoulder level on a tabletop or countertop or with the patient supine and the arm supported on a wedge or elevated overhead. Turn the palm up, then down, by rotating the shoulder, not simply pronating and supinating the forearm. Horizontally adduct and abduct the shoulders by bringing the elbows together and then pointing them laterally. Face a wall; place one or both palms on the wall with the hands above shoulder level. If swelling is present in the wrist and hand, repetitive active finger movements are indicated with the arm elevated. One finger at a time, press matching fingers together and then pull them away from each other. To complete the exercise sequence, perform additional curl-ups (about five repetitions) with hands sliding on the thighs. Rest in a supine position with the involved arm elevated on pillows for about 30 minutes after completing the exercise sequence. Exercises Specifically for Lower Extremity Lymphedema Clearance N O T E: After completing the general lower body, neck, and shoulder exercises previously described, have the patient perform self-massage first to the axillary lymph nodes on the involved side of the body.