Miscellaneous Disorders of Growth and Development

Osteochondroses


Osteochondrosis is disordered behavior of growing cartilage under load. This may include compressive or tensile loads. Most common age to present is 7 to 12 years.


4.1.1 Classification




  1. Spine: Scheuermann kyphosis.



  2. Upper extremity:




    1. Panner disease (capitellum).



    2. Madelung deformity (distal radial physis).



  3. Lower extremity:




    1. Perthes disease.



    2. Osgood–Schlatter disease (tibial tubercle).



    3. Blount disease (medial tibial physis).



    4. Kohler disease (tarsal navicular).



    5. Freiberg disease (second metatarsal head).



    6. Sever disease (calcaneal apophysis).


4.1.2 Treatment




  1. Cartilage heals and remodels with time.



  2. Minimize load.



  3. Bracing may help (Blount, Scheuermann).



  4. Reconstruct if deformity develops (Perthes, Scheuermann, Blount, Madelung).




    Bibliography





    1. Crawford H. Localized disorders of bone. In: Flynn J, Weinstein SL, eds. Lovell and Winter’s Pediatric Orthopaedics. 7th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2014:278–319



    2. Maier GS, Lazovic D, Maus U, Roth KE, Horas K, Seeger JB. Vitamin D deficiency: the missing etiological factor in the development of juvenile osteochondrosis dissecans? J Pediatr Orthop. 2019 Jan;39(1):51-54



4.2 Musculoskeletal Tumors


Most pediatric skeletal tumors are benign. The most common primary malignancies include osteosarcoma, Ewing sarcoma, and rhabdomyosarcoma. Secondary pediatric skeletal involvement may occur with leukemia and neuroblastoma and peripheral neuroectodermal tumor.


4.2.1 Symptoms


Symptoms indicating possible malignancy include night pain that is not activity related, rapid increase in pain, increasing fatigue, or bruising.


4.2.2 Laboratory Studies




  1. Erythrocyte sedimentation rate: mildly elevated for most malignant tumors.



  2. Complete blood cell count: abnormal in leukemia and lymphoma.


4.2.3 Imaging




  1. Plain films: Location, morphology, and host reaction are the most diagnostic features. Locations of most common benign and malignant tumors are shown in ▶ Fig. 4.1.



  2. Radionuclide scans: Very sensitive for malignant bone and soft-tissue tumors; may be negative in eosinophilic granuloma.



  3. CT: Best when bony changes need to be better defined.



  4. MRI: Does not show bony detail well but does show soft tissue and intramedullary detail well.



    Sites of musculoskeletal tumors in skeletally immature and mature children. See tables below for explanation of abbreviations.


    Fig. 4.1 Sites of musculoskeletal tumors in skeletally immature and mature children. See tables below for explanation of abbreviations.



4.2.4 Tumor Types




  1. Benign.




    1. Eosinophilic granuloma: Reticuloendothelial lesion usually centrally located in one or several bones; poorly or well circumscribed. Usually waxes and wanes spontaneously.



    2. Osteoid osteoma: Painful nidus surrounded by sclerosis; usually found in patients aged 6 to 25 years.



    3. Osteochondroma (osteocartilaginous exostosis), metaphyseal, solitary, or multiple; growth ceases at maturity.



    4. Chondromyxoid fibroma: Eccentric local lesion, usually in lower extremity progressively enlarging. Patients aged 10 to 25 years.



    5. Chondroblastoma epiphyseal tumor of adolescence; lucent with foci of internal calcification.



    6. Unicameral bone cyst: Central lucent metaphyseal lesion usually of proximal humerus or femur; expands and thins cortex; resolves at maturity.



    7. Nonossifying fibroma: Eccentric intracortical deficit in metaphysis; resolves by maturity.



    8. Adamantinoma (Adam): Sclerotic anterior cortical deficit, usually of tibia.



    9. Enchondroma (ench) central lucent defect with internal calcification.



    10. Giant cell tumor: Lucent epimetaphyseal tumor just after maturity.



    11. Aneurysmal bone cyst: Expansile metaphyseal lesion of late adolescence that destroys cortex but leaves thin shell.



  2. Malignant:




    1. Fibrosarcoma.



    2. Osteosarcoma: Most common primarily malignant bone tumor, metaphyseal; located in fastest growing regions.



    3. Chondrosarcoma: Central or peripheral, expansile tumor of young adults.



    4. Ewing sarcoma: Small cell tumor of diaphysis; patients aged 5 to 15 years; usually lasts with extensive periosteal reaction.


4.2.5 Tumors Common to Specific Locations in Children




  1. Long bones (▶ Fig. 4.1).



  2. Spine:




    1. Posterior elements:




      1. Aneurysmal bone cyst.



      2. Osteoid osteoma.



      3. Osteoblastoma.



    2. Vertebral body:




      1. Histiocytosis.



      2. Hemangioma.



      3. Osteosarcoma.



      4. Ewing sarcoma.



      5. Chordoma.



  3. Ribs:




    1. Fibrous dysplasia.



    2. Ewing sarcoma.



    3. Chondrosarcoma.



    4. Metastasis.



  4. Pelvis:




    1. Ewing sarcoma.



    2. Fibrous dysplasia.



    3. Aneurysmal bone cyst.



    4. Osteoblastoma.



    5. Eosinophilic granuloma.



    6. Leukemia.



    7. Osteosarcoma.



  5. Scapula:




    1. Ewing sarcoma.



    2. Osteoblastoma.



    3. Aneurysmal bone cyst.


4.2.6 Staging




  1. Malignant tumors (▶ Table 4.1).



  2. Benign tumors:




    1. Latent.



    2. Active.



    3. Aggressive; may expand into soft tissues or metastasize (▶ Table 4.2).










































      Table 4.1 Staging of malignant tumors

      Surgical stage


      Surgical grade (G)


      Site (T)


      Metastases (M)


      IA

      Low (G1)

      Intracompartmental (T1)

      M0B

      Low (G1)

      Extracompartmental (T2)

      M0


      IIA

      High (G2)

      Intracompartmental (T1)

      M0

      High (G2)

      Extracompartmental (T2)

      M0


      III

      Any

      Any T


      Source: Data from Enneking WJ. Musculoskeletal Tumor Surgery. New York, NY: Churchill Livingstone; 1983.





























































































      Table 4.2 Common bone tumors

      Type of tumor


      Abbreviation


      Name


      Benign



      EG

      Eosinophilic granuloma

      OO/OB

      Osteoid osteoma/osteoblastoma

      OC

      Osteochondroma

      CMF

      Chondromyxofibroma

      CB

      Chondroblastoma

      UBC

      Unicameral bone cyst

      NOF/FCD

      Nonossifying fibroma/fibrous cortical defect

      Adam

      Adamantinoma

      Ench

      Enchondroma

      GCT

      Giant cell tumor

      ABC

      Aneurysmal bone cyst


      Malignant



      FS

      Fibrosarcoma

      OS

      Osteosarcoma

      CS

      Chondrosarcoma

      MFH

      Malignant fibrous histiocytoma

      POS

      Parosteal osteosarcoma

      Met

      Metastasis

      SS

      Synovial sarcoma

      RMS

      Rhabdomyosarcoma



4.3 Musculoskeletal Problems in Hemophilia


Hemophilia A (factor VIII) and B (factor IX deficiency) are the two most common bleeding disorders, followed by von Willebrand disease. Factor level below 5% of normal level indicates risk of serious bleeding. These conditions should be jointly managed by hematology and orthopaedic specialists (▶ Table 4.3).





















Table 4.3 Factor doses and kinetics

Factor


Rise after one unit/kg dose (%)


Approximate half-life (h)


VIII

2

12


IX

1

24



4.3.1 Treatment of Acute Hemarthropathy




  1. Factor replacement, 50% every 48 hours × 6 days.



  2. Aspiration.



  3. Immobilization.



  4. Rehabilitation.


4.3.2 Treatment of Subacute Hemarthropathy




  1. Factor replacement, 30% × 2 to 6 weeks.



  2. Strengthen and increase range of motion.



  3. Consider synovectomy if not resolved.


4.3.3 Control of Bleeding after Fracture




  1. Factor replacement, 50% × 1 to 2 days.



  2. Factor replacement, 30% × 1 week.


4.3.4 Imaging (Arnold Classification)




  1. Soft-tissue swelling.



  2. Osteopenia, epiphyseal overgrowth.



  3. Subchondral cysts; wide or square contours.



  4. Irregular joint space.



  5. Absent joint space.




    Bibliography

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Mar 20, 2020 | Posted by in ORTHOPEDIC | Comments Off on Miscellaneous Disorders of Growth and Development

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