Find information on thousands of medical conditions and prescription drugs.

Proteus syndrome

Proteus Syndrome (PS) is a congenital disorder that causes skin overgrowth, atypical bone development, and tumour appearance over half the body. Proteus Syndrome is extremely rare. Since Dr. Michael Cohen identified it in 1979, only a few more than 100 cases have been confirmed worldwide. more...

Bubonic plague
Pacman syndrome
Paget's disease of bone
Paget's disease of the...
Palmoplantar Keratoderma
Pancreas divisum
Pancreatic cancer
Panic disorder
Paramyotonia congenita
Parkinson's disease
Parkinson's disease
Paroxysmal nocturnal...
Patau syndrome
Patent ductus arteriosus
Pelizaeus-Merzbacher disease
Pelvic inflammatory disease
Pelvic lipomatosis
Pendred syndrome
Periarteritis nodosa
Perinatal infections
Periodontal disease
Peripartum cardiomyopathy
Peripheral neuropathy
Periventricular leukomalacia
Pernicious anemia
Persistent sexual arousal...
Pes planus
Peutz-Jeghers syndrome
Peyronie disease
Pfeiffer syndrome
Photosensitive epilepsy
Pica (disorder)
Pickardt syndrome
Pili multigemini
Pilonidal cyst
Pityriasis lichenoides...
Pityriasis lichenoides et...
Pityriasis rubra pilaris
Placental abruption
Pleural effusion
Plummer-Vinson syndrome
Pneumocystis jiroveci...
Pneumonia, eosinophilic
POEMS syndrome
Poland syndrome
Polyarteritis nodosa
Polycystic kidney disease
Polycystic ovarian syndrome
Polycythemia vera
Polymyalgia rheumatica
Polyostotic fibrous...
Pompe's disease
Popliteal pterygium syndrome
Porphyria cutanea tarda
Portal hypertension
Portal vein thrombosis
Post Polio syndrome
Post-traumatic stress...
Postural hypotension
Poxviridae disease
Prader-Willi syndrome
Precocious puberty
Premature aging
Premenstrual dysphoric...
Primary biliary cirrhosis
Primary ciliary dyskinesia
Primary hyperparathyroidism
Primary lateral sclerosis
Primary progressive aphasia
Primary pulmonary...
Primary sclerosing...
Prinzmetal's variant angina
Proconvertin deficiency,...
Progressive external...
Progressive multifocal...
Progressive supranuclear...
Protein S deficiency
Protein-energy malnutrition
Proteus syndrome
Prune belly syndrome
Pseudomyxoma peritonei
Pseudotumor cerebri
Pseudoxanthoma elasticum
Psychogenic polydipsia
Psychophysiologic Disorders
Pubic lice
Puerperal fever
Pulmonary alveolar...
Pulmonary hypertension
Pulmonary sequestration
Pulmonary valve stenosis
Pulmonic stenosis
Pure red cell aplasia
Purpura, Schoenlein-Henoch
Purpura, thrombotic...
Pyoderma gangrenosum
Pyruvate kinase deficiency

There may be many more than this, but those individuals correctly diagnosed usually have the most obvious manifestations of Proteus syndrome, leaving them severely disfigured.

This extremely rare condition would have remained obscure, were it not for the fact that Joseph Merrick - immortalized as the "Elephant Man" for a look imparted by his large facial tumour and the grayish hue of his overgrown skin - was lately diagnosed as having a particularly severe case of Proteus syndrome rather than, or in addition to, the neurofibromatosis (NF) doctors once thought he had.

Proteus syndrome is a progressive condition, wherein children are usually born without any obvious deformities. As they age, tumours as well as skin and bone growths appear. Some affected individuals may suffer from learning disabilities as a result of these growths, and a significantly shortened lifespan. The disorder affects both genders equally, and can be found in all ethnicities.

Like Merrick, however, many people with Proteus syndrome are of normal or above-average intelligence. Their greatest difficulty lies in how society treats them on account of their serious deformities.

Researchers are still trying to determine the cause(s) of Proteus syndrome. While doctors can treat some of the symptoms (by removing tumours, for example), there is no cure.

Many sources classify Proteus syndrome to be a type of epidermal nevus syndrome (see external links).


[List your site here Free!]

Elbow Arthrodesis after War Injuries
From Military Medicine, 2/1/05 by Bilic, Ranko

Arthrodesis is a surgical procedure that results in fusion of the joint and bony ankylosis. With this operation, we can achieve satisfactory function of the limb in cases in which options for different treatments no longer exist. Severe joint destruction, joint infections, and nonunions, as well as complex war injuries (with large bone and soft tissue defects), are indications for arthrodesis. The elbow is the most susceptible joint of the upper limb to war injury. Between 1992 and 1995, we performed elbow arthrodesis for nine patients in our department after war injuries. As analysis of our results shows, elbow arthrodesis, although a rarely performed surgical procedure, is the best treatment for patients with complex war injuries of the elbow. For arthrodesis, we used external fixation in combination with internal fixation (cancellous bone screw) and additional autologous cancellous bone grafts.


Elbow arthrodesis is a rare surgical procedure that results in bony ankylosis (bony overgrowth of the joint). Satisfactory upper limb function can be achieved for patients with painful and minimally mobile elbows or patients with large defects of the elbow joint.1 Extra-articular and intra-articular techniques, as well as combinations of those techniques, have been described in the literature.1-11 Extra-articular techniques have been used only with large chronic elbow defects.1,9,10 The descriptions of earlier techniques included local and free bone transplants with only 50% bony ankylosis of the elbow.2 Along with improvements in external and internal fixation, new techniques for intra-articular elbow arthrodesis were developed.4,6 Methods that are now used include external fixation alone or in combination with internal fixation (with cancellous bone screws or compression screws) or solely internal fixation with a plate (with or without compression screws).3 The results of these techniques proved to be much better (between 50% and 100% bony ankylosis) and healing times much shorter.4,5,12,13 Mueller et al.6 described an intra-articular technique for elbow arthrodesis with resection of all three elbow joint surfaces (humerus, ulna, and radius) and a combination of external and internal fixation (compressive osteosynthesis with external fixation and an axial cancellous bone screw with washer). The war in the Republic of Croatia caused an increase in the number of elbow injuries. We used the Mueller technique for elbow arthrodesis, combined with autologous cancellous bone grafts (from the iliac bone), when large bone defects were present. In this article, we present this modified procedure and the results after surgery.


Patients and Assessments

The study was a records review study, in which we analyzed the results after elbow arthrodesis for patients treated in the Department of Orthopaedic Surgery, School of Medicine, University of Zagreb. Patients were initially treated at the injury site (mostly first lines of fire), where primary wound treatment and temporary immobilization with a CMC external fixator14 were performed. All patients were treated for war-related elbow injuries. Indications for the operation were complex comminuted fractures and nonunions after primary immobilization. Postoperative X-rays were taken, and bone fusion was observed. After the records review, all patients were invited for a clinical examination. Control X-rays were taken during the examination, and patient satisfaction and functioning in everyday activities were evaluated. For patient satisfaction, the following gradation was used: very satisfied, satisfied, or unsatisfied. For functioning in everyday activities, the following gradation was used: no restriction, mild restriction, severe restriction, or incapable. No restriction or mild restriction was considered a good result, severe restriction was considered a satisfactory result, and incapable of everyday activities was considered a poor result. For pain evaluation, a visual analog scale was used (0 points, no pain; 10 points, worst pain ever).

Operative Technique

A tourniquet is used for the surgical procedure. We use a posterior approach for the elbow exposure. After exposure, resection of the distal humerus and olecranon is performed, followed by resection of the head of the radius just above the insertion of the biceps muscle. After remodeling of the resected surfaces, two Schanz screws are drilled into the humerus and two into the ulna. The proximal humeral Schanz screw is connected to the distal ulnar Schanz screw with one bar, and the distal humeral screw and the proximal ulnar screw are connected with another bar. The bars are then connected together, for better fixation of the whole construction (Fig. 1). After external fixation is completed, internal fixation with a cancellous bone screw and washer is applied (Fig. 2). Before final tightening of the screw and final compression between the Schanz screws, autologous cancellous bone from the iliac bone is inserted between and around the resected surfaces. Postoperative treatment is based on finger exercises, which start on the first day, and shoulder and wrist exercises, which start on the third postoperative day.


A total of nine patients were surgically treated. There were eight men and one woman, with a mean age of 29.7 years (range, 22-40 years). Indications for the operation were complex comminuted fractures for four patients and nonunions for five patients. The operation was performed an average of 8.5 months after the primary war injury (range, 3 weeks to 37 months). Follow-up periods were 8 to 11 years.

The wounds were primarily infected for three patients (two patients with Pseudomonas and Proteus and one patient with Acinetobacter sp.), but infections were treated before the elbow arthrodesis. Temporary fixation with a CMC fixator was performed for six patients. External fixation in combination with cancellous bone screws was used in seven cases, and external fixation alone was used in two cases. The median elbow joint position was 90 degrees of flexion (average, 88 degrees; range, 60-100 degrees). X-ray analyses showed that the shortest period necessary for fusion of the elbow (bony ankylosis) was 5 months and the longest was 7.5 months. The average time necessary for fusion (bony ankylosis) after elbow arthrodesis was 6 months 10 days (Fig. 3). The external fixator was left in place an average of 12.5 months (range, 9-15 months). Revision operations were performed in two cases; we performed rearthrodesis in one case (because of nonunion after arthrodesis) and refixation with new Schanz screws in the other (because of loosening of the originally placed Schanz screws). In the second case, repositioning of the ulnar nerve was also performed because of the cubital tunnel syndrome (ulnar nerve compression was seen). Long-term follow-up monitoring (8-11 years) showed no complications (no loosening and no infections). All patients were very satisfied or satisfied with the results, and eight of nine patients had good results regarding everyday activities. For one patient, satisfactory results regarding everyday activities were achieved.


War-related elbow injuries, because of their complexity (large bone defects, large soft tissue defects, and wound contaminations) and because mainly younger populations are affected, demand fast definitive treatment. For these large, open, intraarticular fractures (after trauma or wounding) associated with excessive bone and soft tissue loss, elbow arthrodesis is a method of choice (Fig. 4). During the decision-making process regarding elbow arthrodesis, one must take into account medical and occupational indications, the age of the patient, and the condition of nearby joints that will partially take over the function of the elbow. Candidates for elbow arthrodesis are younger patients with greater need for strength and stability of the arm.2,15 Arthrodesis is commonly used in cases in which the elbow is painful and minimally mobile or in which complications or failure of other surgical procedures4 is expected.

Elbow arthrodesis is also a method of choice for the treatment of complications of septic or tuberculous arthritis when extensive destruction of joint surfaces occurs.4,16 Post-traumatic elbow arthrosis and failed elbow arthroplasty are also indications for elbow arthrodesis.4,13 Every operative procedure, including elbow arthrodesis, has contraindications. Relative contraindications are reduced mobility of the ipsilateral shoulder, wrist, or cervical spine. For patients for whom both elbow joints are involved, alloarthroplasty or resection arthroplasty of at least one elbow is preferred.2,7,12,16 The optimal elbow joint position for arthrodesis varies; it depends mostly on specific functional demands, the patient's occupation, and compensatory arm and neck movement ability. Most authors suggest 90 degrees of flexion as the best position for elbow arthrodesis on the dominant side, with normal shoulder, wrist, and spine mobility. This is the best position for activities of everyday living (writing, eating, and personal hygiene) and allows maximal arm strength.1,2,6,8,15,17 Arthrodesis with 45 to 60 degrees of flexion is sometimes better (for some professions) and provides a better cosmetic appearance.4,15 The best position of the forearm for the arthrodesis, in cases in which rotation is not possible, is a neutral position, slight pronation (for better writing ability and better ability to use a computer), or slight supination (for better object-holding ability).4,17 For both-side elbow arthrodesis, 90 degrees of flexion on one side and 45 to 60 degrees of flexion on the other side is proposed.2,16 It is advisable to immobilize the patient's elbow in the desired position before the operation, for better evaluation of the patient's preferences.18,19


Elbow arthrodesis is the definitive treatment for complex, open, intra-articular fractures with bone and soft tissue loss after wounding or large traumatic defects. The best position for elbow arthrodesis is 90 degrees of flexion, which ensures easy functioning in activities of everyday living and personal hygiene maintenance. Our results show that external fixation, along with internal fixation with cancellous bone screws and washers combined with autologous cancellous bone grafts, is an effective method for achieving arthrodesis of the war-injured elbow. Patients should be cautioned, however, that prolonged immobilization with an external fixator is required.


1. Rashkoff E, Burkhalter WE: Arthrodesis of the salvage elbow. Orthopedics 1986; 9: 733-8.

2. Beckenbaugh RD: Arthrodesis. In: The Elbow and Its Disorders, pp 696-703. Edited by Morrey BF. Philadelphia, WB Saunders, 1985.

3. Orozco R, Giros J, Sales JM, Videla M: A new technique of elbow arthrodesis: a case report. Int Orthop 1996; 20: 92-9.

4. McAuliffe JA, Burkhalter WE, Ouellete EA, Carneiro RS: Compression plate arthrodesis of the elbow. J Bone Joint Surg Br 1992; 74: 300-4.

5. Irvine GB, Gregg PJ: A method of elbow arthrodesis: brief report. J Bone Joint Surg Br 1989; 71: 145-6.

6. Mueller ME, Allgoewer M, Schneide R, Willenegger H: Manual der Osteosynlhese, Ed 3. Berlin, Springer Verlag, 1991.

7. Hahn MP, Ostermann PA, Richter D, Muhr G: Elbow arthrodesis and its alternative. Orthopaede 1996; 25: 112-20.

8. Justi EI: Arthrodesis of shoulder, elbow, and wrist. In: Cambell's Operative Orthopaedics, pp 357-68. Edited by Crenshaw AH. St Louis, MO, Mosby, 1992.

9. Sakkers R, Lammens J, Reynaert P, Fabry G: Ilizarov reconstruction after a failed upper limb salvage procedure in oncology: a case report. J Shoulder Elbow Surg 2000; 9: 148-51.

10. Gunzburg R, Mokassa L, Wagner J: Arthrodesis of the elbow using Ilizarov's external fixator. Acta Orthop Belg 1989; 55: 593-7.

11. Pippow A, Krahenbuhl L, Michel MC, Witschger P: Combination of plate and external fixator for biological osteosynthesis of comminuted fractures. Swiss Surg 2002; 8: 230-6.

12. Morrey BF: Distraction arthroplasty: clinical applications. Clin Orthop 1993; 293: 46-54.

13. Wolfe SW, Figgie MP, Inglis AE, Bonn WW, Renawat CS: Management of infection about total elbow prostheses. J Bone Joint Surg Am 1990; 72: 198-212.

14. Korzinek K, Delimar D, Kolundzic R: Der CMC-Außenfixator. Med Orth Tech 1997; 117: 213-6.

15. Snider WJ, DeWitt HJ: Functional study for optimal position for elbow arthrodesis or ankylosis. J Bone Joint Surg Am 1973; 55: 1300-8.

16. Arafiles RP: A new technique of fusion for tuberculous arthritis of the elbow. J Bone Joint Surg Am 1981; 63: 1396-1400.

17. Morrey BF, Askew LJ, Chao EY: A biomechanical study of normal functional elbow motion. J Bone Joint Surg Am 1981; 63: 872-7.

18. Nagy SM III, Szabo RM, Sharkey NA: Unilateral elbow arthrodesis: the preferred position. J South Orthop Assoc 1999; 8: 80-5.

19. Tang C, Roidis N, Itamura J, Vaishnau S, Shean C, Stevanovic M: The effect of simulated elbow arthrodesis on the ability to perform activities of daily living. J Hand Surg Am 2001; 26: 1146-50.

Guarantor: Ranko Bilic, MD PhD

Contributors: Ranko Bilic, MD PhD; Robert Kolundzic, MD; Goran Bicanic, MD; Kresimir Korzinek, MD PhD

Department of Orthopaedic Surgery, Clinical Hospital Centre Zagreb, School of Medicine, University of Zagreb, Salata 7, 10000 Zagreb, Croatia.

This manuscript was received for review in June 2003. The revised manuscript was accepted for publication in March 2004.

Reprint & Copyright © by Association of Military Surgeons of U.S., 2005.

Copyright Association of Military Surgeons of the United States Feb 2005
Provided by ProQuest Information and Learning Company. All rights Reserved

Return to Proteus syndrome
Home Contact Resources Exchange Links ebay