Surgical Treatment of Sprengel’s Deformity: A Systematic Review and Meta-Analysis

(1) Background: Sprengel’s deformity (SD) is a rare congenital anomaly caused by failure in the descent of the scapula. We aimed to systematically review the current literature reporting data from children undergoing surgery for SD, in order to explore the rate of success and complications of the different surgical techniques, possibly providing recommendations about the management of SD in children. (2) Methods: we electronically searched the literature from Ovid, MEDLINE and the Cochrane Library databases. Demographic data, surgical procedures, outcomes and complications were analyzed. We categorized surgical procedures into five groups. (3) Results: 41 articles met the inclusion criteria, showing a poor overall study quality; 674 patients (711 shoulders) were analyzed. Green’s and Woodward’s procedures, both aiming the scapular relocation in a more anatomical position, were the most commonly used techniques. We counted 168 adverse events (18 major complications). The best clinical and cosmetic results seem to be achieved when surgery is performed in children aged less than eight years. (4) Conclusions: this paper represents the first systematic review reporting qualitative and quantitative data about the surgical treatment of SD. Surgery for SD seems to be effective in increasing the shoulder’s range of motion and improving the cosmetic appearance in almost all cases, with a low rate of major complications.

High Cervical Partial Posterior Cord Cleft in a Case of Klippel-Feil Syndrome

Posterior high cervical spinal cord cleft has been rarely reported in association with Klippel - Feil syndrome (KFS). Its presence may have prognostic value in long term neurological outcome. If there is segmentation anomaly of two or more vertebrae during embryonic life mainly involving the cervical vertebrae, it can result in Klippel - Feil syndrome. It is a rare anomaly and is present with the incidence of 1 : 42000 births.1 During 2nd to 8th week of embryonic life, the segmentation of the mesodermal somites of the spine in the cervical region fails and leads to cervical spine synostosis which is also known as KFS. Although most commonly the KFS patients present with the symptoms of short length of the neck, hairline posteriorly is low and the mobility in the upper part of the spine is restricted. It is also sometimes associated with other congenital anomalies like Sprengel’s deformity, hemivertebra, basilar impression, cleft palate, and many more rare anomalies.2 Most commonly the patients having this syndrome show restriction of the motion in the neck region.3

Omovertebral bone causing traumatic compression of the cervical spinal cord and acute neurological deficits in a patient with Sprengel’s deformity and Klippel-Feil syndrome: case report

The presence of an omovertebral bone with Sprengel’s deformity and Klippel-Feil syndrome is a complex congenital anomaly that is not well understood. It most commonly manifests as cosmetic deformity, limited range of motion, and functional disability, although there are reports of the insidious development of cervical myelopathy. In this paper, the authors present the case of a 49-year-old man with acute neurological deficits after a low-energy mechanism of traumatic spinal cord compression, resulting from an impinging omovertebral bone through a traumatic laminar defect. The patient underwent resection of the omovertebral bone, laminectomy decompression of the spinal canal, and anterior stabilization. This case highlights a rarely discussed complication of undiagnosed Sprengel’s deformity and its associated conditions following even low-energy traumatic mechanisms.

INTRAOPERATIVE NEUROMONITORING DURING SURGICAL CORRECTION OF SPRENGEL’S DEFORMITY

Introduction Neuromonitoring (IOM) is a procedure for verification of the nerve impulse transmission along structures of central and peripheral nervous system during surgical procedures. Motor evoked potentials (MEPs) recordings from muscles induced with electrical pulses transcranially to motor cortex centers are especially useful during the surgery with an increased risk of iatrogenic damage to efferent nerve structures. Aim of the study The aim of this report is to present the scenario of the reversible inhibition in pathways transmitting nerve impulses during surgical correction of Sprengel’s deformity with the assessment of IOM. Material and methods Nine-year old girl was admitted to the hospital due to congenital high scapula. Corrective surgery was performed using the Woodward technique with an assessment of IOM. Results The amplitudes and latencies of the MEPs from muscles of upper right extremity were recorded as decreased and increased, respectively at about 20% during the final fixation of scapula. Thanks to these recordings surgeons could prevent the permanent damage of the brachial plexus fibers, by partial releasing of applied sutures. After surgery and subsequent rehabilitation the patient returned to the normal activity in right upper extremity. Association of electromyography and MEPs results helped with ordering and controlling the course of treatment. Conclusions The benefit of IOM relay on the safety of orthopedic surgery and decreasing the number of iatrogenic perioperative complications. This diagnostic procedure is also a strong point for argumentation in hospital administration during negotiations with lawyer representing the patient when iatrogenic complication appear.