Cervicothoracic Arthrodesis: The Best Management for Dropped Head Syndrome, A Case Series

Abstract

The authors present two cases of Dropped Head Syndrome (DHS), a condition distinguished by the chin-on-chest deformity due to weakness of the posterior muscle group of the neck. This is a unimodal syndrome and is most common among individuals in their seventh and eighth decade of life, with an average age of around 75 years. DHS is more prevalent in women, with a 3:2 ratio compared with men. Our reports document the disease's natural progression, increasing kyphosis, and resistance to initial conservative treatments. Both patients reported functional limitations, as their ability to walk and eat without assistance was hindered by loss of the horizontal gaze and dysphagia. After these treatment failures, we utilized an arthrodesis approach to target the C2-T6 segments of the cervical-thoracic spine. All previous complaints were resolved, the deformity was reduced, and the patients regained their functional independence. The surgical approach is indeed more efficient, although the combination of both methods yielded even better clinical outcomes. Arthrodesis from C2 to the upper thoracic spine segments is considered the most appropriate surgical technique for maintaining subaxial spine movement. This is due to its excellent rate of correcting deformities, preserving horizontal gaze, and improving or maintaining a neurological state.

 

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Resumo

Os autores apresentam 2 casos de síndrome da cabeça caída (SCC), um quadro destacado devido à presença da deformidade-queixo-no-peito, decorrido da fraqueza do grupo muscular do pescoço. A síndrome se apresenta de forma unimodal, sendo predominante entre a sétima e a oitava década de vida, com uma idade média de cerca de 75 anos. É mais predominante em mulheres, apresentando uma razão de 3:2 em relação aos homens. Em nossos relatos, evidenciamos a evolução natural da doença, por meio de uma cifose crescente ao longo dos anos resistente às terapias conservadoras iniciais. Ambos os pacientes apresentavam queixa de limitação funcional, devido à incapacidade de deambular e se alimentar sem auxílio, resultante da perda do olhar horizontal e da disfagia. Após essa falha, a conduta adotada foi a artrodese dos seguimentos cervico-torácicos da coluna, buscando os seguimentos C2-T6. Obteve-se resolução das queixas prévias, reduzindo a deformidade e reavendo a independência funcional dos pacientes. A abordagem cirúrgica é de fato mais eficiente, embora a combinação de ambos os métodos tenha produzido resultados clínicos ainda melhores. A artrodese de C2 até os segmentos superiores da coluna torácica é considerada a técnica cirúrgica mais adequada para manter o movimento subaxial da coluna. Isso se deve ao seu excelente índice de correção de deformidades, preservação do olhar horizontal e melhora ou manutenção do estado neurológico.

 

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Keywords

dropped head syndrome - kyphosis and surgery

Palavras-chave

síndrome da cabeça caída - cifose e cirurgia

Introduction

Dropped head syndrome (DHS) is a rare condition characterized by pathological kyphosis resulting from extreme weakness of the extensor muscles of the neck, leading to chin-to-chest deformity.[1] [2]

DHS can also be defined as scaphocephaly or anterocolis, the latest example being the result of dystonia of the cervical muscles. Currently, there is no global data available on the prevalence of dropped head syndrome. However, when associated with cervical dystonia, the incidence falls within the range of 0.06–0.08 per 100,000 individuals per year.[1] [3]

The objective of this study is to present two cases of dropped head syndrome to enhance comprehension of diagnostic aspects and treatment options.

 

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Case Report

Case One

An 87-year-old woman has had cervical kyphosis for four years. This condition has progressed over time, aggravating her previous symptoms of pain, loss of horizontal sight, and dysphagia. During the physical examination, a weakened extensor neck muscle and the uncommon chin-on-chest deformity were visible, establishing the diagnosis of drooping head syndrome. Imaging revealed a “chin-brow radiographic angle” measuring 113° and a “chin-brow vertical angle” measuring 57°, ([Figs. 1] and [2]). The presence of shortening in the tendons of the sternocleidomastoid muscle is a significant factor in determining the extent of corrective measures needed.

Fig. 1 “Chin-brow radiographic angle” of 113° on a lateral cervical radiograph.

Fig. 2 “Chin-brow vertical angle” of 57° noted on physical examination.

Initially, a conservative approach involving physiotherapy and analgesics was employed. However, no improvements were observed during this intervention. Consequently, a surgical procedure was performed later.

The surgical procedure performed was C2-T6 instrumentation with osteotomy at C5-C7. Transpedicular screws were implemented at C2 and throughout the thoracic spine, while lateral mass screws were utilized at C3-C7. Two rods were utilized due to the unavailability of the transition rod, alongside 2 reinforcing crosslinks. ([Fig. 3]) We chose to perform fixation at C2 due to the favorable alignment of the condyle/C1/C2 while preserving the anatomy and allowing recovery of the horizontal movements of the neck and the “yes-yes” movements intrinsic to the muscles attached to the condyle-C1. Before the procedure, the patient had difficulty performing daily activities independently, such as eating and walking. Following the surgery, the deformity was corrected, and the spine resumed its natural curvature. One year post-procedure, the patient revisited the clinic and reported an absence of pain and neurological issues, along with an improved level of functional independence.

Fig. 3 C2-T6 cervicothoracic arthrodesis with Schwab 1 osteotomy in C5–7 vertebrae recorded in the perioperative period.

 

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Case Two

An 87-year-old woman presented with progressive cervicothoracic kyphosis over three years. She had complaints of pain, loss of horizontal gaze, dysphagia, and walking difficulty. A physical examination revealed weakened neck extensor muscles, shortened M. sternocleidomastoid tendons, and a chin-on chest deformity. Imaging indicated a “chin-brow radiographic angle” of 147°, characteristic of drooping head syndrome.

Initially, a conservative treatment approach was attempted, which included physical rehabilitation via physiotherapy and analgesia to alleviate pain. However, this approach proved unsuccessful, and the deformity remained unresolved over time.

The second approach utilized instrumentation of the C2-C6 and T1-T6 vertebrae. Transpedicular screws were utilized at the C2 level and in the thoracic spine, while lateral mass screws were used at the levels of C3-C7 ([Fig. 4])

Fig. 4 Post-operative radiograph presenting C2-T6 cervicothoracic arthrodesis.

In both instances, we opted for fixation from C2 to preserve the horizontal movements of the neck that are intrinsic to the muscles attached to the condyle/C1. Before surgical intervention for the deformity, her main complaint was a lack of functional independence due to an inability to eat or walk unaided. One year following the procedure, the patient revisited the clinic and disclosed experiencing neither pain nor neurological complaints, while showing an increase in functional independence. ([Fig. 5])

Fig. 5 Post-op evaluation of surgical deformity correction.

 

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Discussion

Dropped head syndrome (DHS) typically occurs between the ages of 70–80, with an average age of 75 years, and is more common in women (a 3:2 female-to-male ratio). DHS is characterized by a sudden loss of neck muscle tone and subsequent head drop. Conversely, instances of pediatric DHS are rare and often attributed to genetic mutations.[1] [4]

Drooping head syndrome can be categorized as either primary/idiopathic or secondary to neurological dysfunction, supported by clinical evidence and natural history.[3]

The clinical presentation can be classified as isolated/pure or non-isolated/complex. The most common form is complex anterocollis (AC), which occurs in 7% of all cervical dystonia cases, with an incidence of 0.06–0.08/100,000 per year. Additionally, AC is often associated with dystonia in other areas of the body or with other manifestations of neurological disorders. AC can also be categorized as progressive or non-progressive, congenital, or acquired, and paroxysmal or non-paroxysmal. Pure anterocollis is less common, with an annual incidence of ∼0.008–0.275 per 100,000 individuals.[3] [5] [6]

AC is usually observed secondarily and is more prevalent among patients diagnosed with Parkinson's disease and multiple system atrophy (MSA), with a prevalence of 6% and 42% respectively. Furthermore, there may exist a genetic relationship attributable to the occurrence of movement disorders within the patient's family medical history.[7] [8]

Patients with Dropped Head Syndrome exhibit a chin-on-chest deformity, neck pain, dysphagia, loss of horizontal gaze, and some nervous system disorders. Consequently, functional independence and quality of life are significantly affected, leading to a direct impact on social relationships, as well as daily activities.[1] [8] [9] [10]

The loss of horizontal sight is a result of the imbalance and global malalignment of the spine in the sagittal and coronal planes. This is due to the compensatory mechanism of the deformity, resulting in a loss of thoracic and lumbar curvature.[10]

Another important manifestation is extreme stiffness of the neck flexor muscle group, which can be felt on palpation. There may also be weakness of the proximal appendicular muscles and, in rare cases of cervicobrachial polymyositis, of the trapezius muscle and the extensor muscles of the hand and fingers.[1] [4] [10] [11] [12]

In our reports, the patients lost the horizontal gaze, due to the deformity and rigidity of the anterior musculature, which resulted in the shortening of the M. sternocleidomastoid. These points were considered by the team to be crucial, as the degree of rigidity of these muscles can inhibit passive extension of the head, compromising surgical correction of the deformity.

Radiography (X-ray), computed tomography (CT), and magnetic resonance imaging (MRI) have a crucial role in visualizing parameters to assess the spine and soft tissues. This assessment provides valuable insight into determining the severity of the deformity and deciding on a possible therapeutic approach. Moreover, Positron Emission Tomography (PET) can be important in assessing muscle metabolism, studying the complementary effect in identifying dystonic muscles, and differentiating the forms of anterocollis: CACOL and ACAP.[3] [5] [12] [13]

Electroneuromyography (EMG) is a valuable electrodiagnostic test for identifying the involvement of muscle groups, particularly in cases of involuntary contraction and muscle weakness, and can effectively distinguish between simple and diffuse anterocollis.[3]

Muscle biopsy can reveal inflammatory muscle diseases and, when examined histopathologically, can also indicate mitochondrial myopathies caused by genetic conditions, including isolated myopathy of the extensor muscles of the neck (INEM).[12] [13] [14]

There are two approaches to treating DHS, either through surgery or conservative methods.[4] However, due to insufficient evidence, it remains uncertain which approach is the most effective for initial treatment. Therefore, conservative therapy is usually tried first, and if the results are unsatisfactory, more invasive therapies may be necessary. Therapies may be necessary.[15]

Conservative treatments comprise medication, etiological, and physical therapies, with the first focusing on the use of immunosuppressants as a form of symptom relief. Etiologic therapy aims to resolve the pathologies that could cause this syndrome. Additionally, physical therapy includes physical support, physiotherapy, and necklaces.[1]

Despite combining drug and physical therapies, results are still inconsistent. Using collars and pharmacological drugs is thus not effective in most cases.[4]

In our study, we initially opted for more conservative therapies in an attempt to optimize drug therapy and physical rehabilitation. Unfortunately, both patients exhibited worsening deformities, reduced functional independence, and worsening pain complaints. Consistent with existing literature, our findings suggest lower efficacy rates associated with conservative therapy.

Drain found that surgical therapies are significantly more effective than physical therapies, with success rates of 92.2% and 18.2%, respectively. Moreover, combining physical and surgical therapies can result in 100% effectiveness. Patients who received treatment targeted to their etiology experienced a resolution rate of 73.5%. Immunosuppressants produced a positive outcome in 78.9% of cases.[1]

The surgical procedure aims to restore the horizontal gaze, correct sagittal alignment, and decompress neural elements. There are two primary methods to perform the procedure: arthrodesis of a single segment and cervicothoracic arthrodesis.

According to Cavagnaro et al., when comparing the two approaches statistically, patients who received cervicothoracic arthrodesis had a better prognosis. Arthrodesis from C2 to the upper thoracic spine segments is the most appropriate procedure, as it preserves subaxial spine mobility and enhances the overall surgical success rate.[1] [2]

When evaluating only the restoration of horizontal gaze and the permanence or improvement of neurological status, cervical arthrodesis procedures proved successful in 91.3% of cases. In comparison, patients who underwent cervicothoracic arthrodesis had a 100% success rate, as reported by Cavagnaro et al.[2]

Our approach involved performing posterior arthrodesis of the C2-T6 levels, allowing for the fixation of two spine segments, including the cervico-thoracic junction. After the surgery, the chin-on-chest deformity was successfully reduced, leading to the restoration of horizontal gaze and relief of symptoms such as dysphagia and pain. Based on the literature, this technique is expected to achieve the highest rate of resolution while preserving the motility of the muscles attached to the C1 vertebra.

Cervicothoracic arthrodesis is a recommended approach for the treatment of this condition, which has an excellent prognosis. In our experience, most patients who undergo this therapeutic option exhibit improved functional independence, as evidenced by their ability to walk and feed themselves without assistance.[2]

 

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Conclusion

Two approaches are available for treating Dropped Head Syndrome: surgical and conservative. The surgical approach is indeed more efficient, although the combination of both methods yielded even better clinical outcomes. Arthrodesis from C2 to the upper thoracic spine segments is considered the most appropriate surgical technique for maintaining subaxial spine movement. This is due to its excellent rate of correcting deformities, preserving horizontal gaze, and improving or maintaining a neurological state. Furthermore, it is essential to emphasize the necessity for additional studies to demonstrate more clearly the circumstances that lead to the choice of the surgical approach.

 

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Conflict of Interest

None.

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Publication History

Received: 10 October 2023

Accepted: 21 October 2024

Article published online:
27 March 2025