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Comorbidity cerebral palsy – Sprengel disease

Autor: Ligia Robănescu Cristina Bojan
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We present the case of a 7-year-old girl from the countryside sent with the diagnosis of left spastic hemiparesis, treated with some non-specific elements of kinesiotherapy, which mainly focused on the dorsal dextroconvex scoliosis curvature.

The patient was born at 31 weeks with a weight at birth of 1,900 grams, SA 6/8, with a short neck with a compensating head inclination to the left, the lower angles of the shoulder blades were skidded, with the left one greatly ascended.

Parents were told that the girl was born with a brachial plexus paresis given that the left upper limb motility was much diminished. Living in the countryside, the child did not receive recovery treatment except sporadically and inadequately.

Approximately around the age of 4 years, a spastic left hemiparesis was diagnosed by the occurrence of tricipital hypertonia (spasticity) in the lower limb, for which orthopaedic boots were prescribed which obviously did not improve the posture.

At the age of 7, when the child was examined in the Swedish DALA Foundation’s surgery, the little girl used to move with difficulty, with her left leg fixed in equinovarus posture. The upper left limb showed an exaggerated internal rotation, the forearm was semi flexed, and the hand virtually unusable. Her head was tilted to the left, the lower angles of the shoulder blades were scuffed, with the left one far ascended and drawn to the chest, tipped close to the line of the spiny apophyses.

A dextroconcave cervical scoliosis was also highlighted. The movements at shoulder level were altered: the active abduction in the left arm reached a maximum of 75 °, while the passive abduction was not possible much farther, either.

When the arm was lowered, there was a minimal movement of the left scapula; the abduction movement of the arm is normal in the scapular-humeral joint; what was absent in this case was the lifting movement above the horizontal line which is normal when the shoulder blade moves relative to the thorax. When the scapula was moved to the chest level, cracks could be felt at palpation.

Also at palpation, there was a tension to be felt of the shortened left sternocleidomastoid muscle; a diminution of the upper end of the trapeze could be noted, too.

At palpation, a rigid, bony bar was found on the spinal edge of the shoulder blade in the upper third part, climbing upward and inward towards the line of spiny apophyses, especially towards C7. The lower toe of the shoulder was taken off the chest and closer to the spiny apophysis line.

The acromial angle was more pronounced compared to the healthy side. If the child was asked to straighten her head, which was tilted to the left, she managed to do so, but her left shoulder rose significantly.

The tilting of the head was therefore compensatory, intended to mask the lifting of the malformed shoulder blade or to avoid embarrassment, the discomfort that occurred in these cases. With an eyeliner, it was possible to draw the contour of the raised shoulder blade, and the unevenness of the lower angles of the scapulae could be observed (fig. 1).

Following the path of spiny apophyses, the coexistence of a dextroconcave cervicodorsal scoliotic curvature is noted.

Radiography confirmed the scapular tilting and its deformation. The superior angle of the scapula was shifted between T2-C5, forming a hook which caught at the upper chest (Cavendish grades II-III). At palpation, this deformation translated into a supraclavicular convex tumour.

Neurological damage was evident, left hemibody pyramidal hypertonia with a non-functional hand, foot fixed in talipes equinovarus with compensatory Genu Recurvatum  during walking.

Brisk deep-tendon reflexes, Babinsky reflex present, parachute reflex absent on the left side. It is a case of cerebral palsy coexisting with Sprengel’s disease, which is the congenital elevation of the scapula, characterized by the medial rotation of the distal pole of the scapula (scapula alata). Normally, the scapula is situated between the 2nd and the 7th intercostal space.

The boys / girls ratio in Sprengel’s disease is 3/1.

The syndrome in question is characterized by varying degrees of scapular dysplasia, smaller dimension of the involved scapula compared to the other, rotation of the medial distal angle and reduced elevation of the upper limb (3, 5, 6) The cranial angle of the scapula is curled up before the thoracic aperture, sometimes over it, affecting the shoulder joint.

In milder forms, scapular belt mobility is not altered, but in severe cases, shoulder abduction across the horizontal line is restricted.

This limitation is due to the presence of the osmovertebral bone or a cartilaginous structure between the scapular cranial angle and a spinal vertebral apophysis in the lower cervical spine.

Scapular modification may be associated with other congenital malformations such as: coastal fusion, cortical agenesis, supernumerary ribs, congenital scoliosis, Klippel-Feil syndrome, spina bifida.

The congenital elevation of the scapula was described in 1868 by Eulenberg. In 1883 Willet and Walsham published a post-mortem anatomical study based on dissection.

In 1891 Sprengel and Kolliker described several cases, and in 1972 Cavendish formulated the classification system (4,5) In 1908 Putti proposed a surgical treatment method, which considers that the scapula should be transplanted to a lower level. Subsequent changes were made by Green, Allan, Woodward between 1957 and 1961 (2.7).

Embryological aetiology: The scapula is a cervical appendage that differentiates from the 5th, 6th cervical vertebrae at about 5 weeks of gestation. This structure normally descends into the thorax until the end of the third month of intrauterine life; any impediment to its descent leads to an elevated hypoplastic scapula.

The high position of the scapula is characterized by an interruption of normal migration and probably occurs between weeks 9-12 of gestation.

There is also a cessation of bone, cartilage and muscle development that may be hypoplasic (2) Genetic aetiology – rarely  has there been noted a dominant autosomal form. Cavendish rating scale (used these days, too) (1):

Grade I: very mild form – Shoulder joints are at the same level.

Grade II: mild – Shoulder joints are at about the same level, but the modification is visible even when the patient is dressed.

Grade III: moderate; the shoulder joint is elevated with 2-5 cm, the deformity is easily visible.

Grade IV: severe – the affected shoulder is much more elevated so that the upper angle of the scapula is situated near the occipital bone.

Investigations: CT or MRI, radiography (2,7) Classification after radiological examination:

Grade I: The scapular superior angle is between T4-T2. Grade II: The scapular superior angle is between T2-T5. Grade III: The scapular superior angle is above C5.

Differential diagnosis:

– False increased elevations by hypoplasia of the shoulder blade.

– Secondary increased elevations of an untreated muscular torticollis.

– Fractures of the clavicle healed with shortening, which produces secondary over elevation of the shoulder blade.

– Cervical and thoracic scoliosis.

Treatment:

– For Cavendish I, rehabilitation concerns the extension of scapular region muscles, rhomboid muscles, deep abdominal muscles and back muscles.

– Surgical treatment is only indicated for Cavendish grades II and III:

  1. Schrock Method: involves extensive resection of the shoulder blade that includes the suprasinous fossa, the inferior angle and the inner border.
  2. Ombredanne – Huc Method: the acromioclavicular joint is opened, the acromioclavicular ligaments are sectioned and the clavicle is fastened to the coracoid.
  3. The Woodward Method – is a gentler procedure: moving the scapula to a lower level by moving the spinal origin of the rhomboid and caudal trapezoid muscles (2, 7).

If the omo-vertebral bone is present, it must be removed.

The optimal age for intervention is between 5 years and adolescence. In our patient, the surgery involved moving the spinal origins of the rhomboid and trapezoid muscles (Woodward method).

The evolution was very good, and, after about one month, the child could start rehabilitation treatment including treatment of the neurological impairment following the condition of a better mobility of the scapular-humeral joint.

BIBLIOGRAFIE:

  1. Cavendish M E, Congenital elevation of the scapula. J Bone Joint Surg Br. Aug 1972; 54 (3): 395-408 (2).
  2. Crha B, Gal P. Surgical treatment of Sprengelʼs deformity. Scripta medical (Brno) 74(4): 245-254 Oct.2001.
  3. Eulenberg M. Casuistische mittelheilungen aus dem gembeite der othopedie. Arch Klin Chir. 1863; 4: 301-311.
  4. Jeannopoulos CL. Congenital elevation of the scapula. J Bone Joint Surg Am. Oct 1952; 34 A(4): 883-892.
  5. Ombredanne L. Précis clinique operatoire de chirurgie infantile. Ed. Masson et Cie editeurs Paris. 1932 p.999-1011.
  6. Sprengel O K. Die angeborene verschiebung des schulterblattes nach oben. Archiv für Klinische Chirurgie, Berlin 1891; 42:545549.
  7. Woodward J W. Congenital elevation of the scapula. J Bone Jt Surg 1961: 43 – A ; 219-228.