Sprengel’s Deformity – Introduction, Pathology, Symptoms, Treatment

  • Introduction

Since decades past, disabilities have been vanquishing the healthcare industry. Disabilities come and go in the complex realities of life affecting humanity. Sometimes, it’s only when the disability manifests itself through signs and symptoms that people come to know that they actually have the disability. There are times that disabilities come to it readily. In the realms of healthcare perspective, Sprengel’s deformity constitutes a multitude of disabilities exemplifying a rare occurrence which typically influences the young generation. It specifically dysfunctions a person’s back exhibiting a higher scapula (or shoulder blade) on one side than the other.

Predominantly known as High Scapula, Sprengel’s deformity (believed to occur in early weeks of gestation) shows a shoulder that does not descend appropriately from its cervical pinnacle to its final normal position thus resulting in elevation of the scapula; hence, the name: High Scapula.

Preponderantly considered as a rare entity, it is associated with lateral spine curvature in the neck affecting the young, spina bifida, Klippel-Feil syndrome, omovertebral bone among others. It can be bilateral (both shoulders can be affected) although the left shoulder more commonly succumbs to the condition. Majority of clinical cases affects girls. Diagnosis comes around the vicinity of physical signs as well as roentgenogram.

  • Pathology

In Sprengel’s deformity, the small scapula comes around such that its lower focus gears itself towards the jurisdiction of the spine, diminishing its vertical height and upgrading its wide dimensions, causing its upper segment to loop and hook forward. This causes the cervical muscles to modify its direction due to its shortening on the affected side. This is made by the union (bone, cartilage or fibrous tissue) of which encompasses one of the lower cervical vertebrae and scapula. Then, bone extends from other structures (be it a spinous process, lamina, or transverse process) of one of more vertebrae to the upper segment of the vertebra border of the scapula, thus, forming a bony edge.

  • Signs and symptoms

Commonly, the shoulders are not identical with each other which are usually the hallmark manifestation of the condition. Shoulder movements (particularly abduction) are hugely limited. Glenohumeral rotation is intact. Torticollis and scoliosis are evident though in limited cases. In bilateral cases, the neck looks shortened.

  • Treatment

Treatments consist of interdisciplinary and multifaceted approach which includes surgery in early childhood and therapeutic interventions (especially physical therapy and rehabilitation). Postural awareness training, scapular regimens, stretching programs and strengthening protocols can activate motion. Stretching regimens (through side stretch, diagonal centripetal rotations and use of wand exercises, pulleys, and shoulder wheel – all focused on affected side) can improve muscle motion by elongating tight structures. Strengthening programs (through progressive resistive exercises, resistive diagonal centripetal rotations, resistive side bend and via therapeutic bands – all geared to affected side) can strengthen the weakened musculature. Breathing exercises expand the pulmonary machinery by increasing lung oxygenation. When motion is limited and deformity is unfounded, surgical treatment (typically done in childhood before age 5) is indicated by exposing scapula outside the periosteum, excising the supraspinatus and omovertebral bone, bringing the scapula down to desired level and repositioning muscle attachments to provide anchor and support.

Although Sprengel’s Deformity is one of the rare disabilities, how we come about managing it will depend on how we properly evaluate it and the quality of care that we give those who have it.