Department of Orthopedic Surgery, Toho University Omori Medical Center, 6-11-1, Omori-Nishi, Ota-Ku, Tokyo, 143-8541, Japan.
Department of Surgical Pathology, Toho University Omori Medical Center, 6-11-1, Omori-Nishi, Ota-Ku, Tokyo, 143-8541, Japan.
BMC Musculoskelet Disord. 2021 Apr 14;22(1):353. doi: 10.1186/s12891-021-04232-6.
Acromegaly is a rare disease caused by high serum levels of growth hormone (GH) and insulin-like growth factor 1 (IGF-1), often originating from a pituitary adenoma. Spinal and peripheral joint abnormalities are caused by these hormonal hypersecretions. In particular, the response to GH is involved in the onset of ossification of the spinal ligament in vitro, especially ossification of the posterior longitudinal ligament (OPLL). However, because acromegaly and OPLL are rare diseases, we seldom encounter them in combination. To the best of our knowledge in the English-language literature, this is the first reported case of acromegaly presenting with thoracic myelopathy due to OPLL.
A 47-year-old woman presented with lower extremity weakness and paresthesia, gait disorder, and bladder disorder without any trauma. The patient's most remarkable symptom was paraplegia, and we diagnosed myelopathy due to cervical and thoracic OPLL. Furthermore, we suspected acromegaly because of the characteristic facial features, and we found a pituitary adenoma by contrast-enhanced MRI. Cervical and thoracic decompression, posterior fixation, and pituitary adenoma resection were performed.
We report a case of acromegaly that was detected after the diagnosis of OPLL. The main challenge in acromegaly is delayed in diagnosis. Even in this case, the facial features characteristic of acromegaly had appeared at least 9 years ago. Early diagnosis and treatment of acromegaly improve prognosis and reduce exposure to GH and IGF-1 through early intervention and seem to suppress the progression of ligament ossification. Orthopedic surgeons and neurosurgeons need to keep in mind that acromegaly is associated with bone/joint lesions and ossification of the spinal ligament and should aim to diagnose acromegaly early.
肢端肥大症是一种由生长激素（GH）和胰岛素样生长因子 1（IGF-1）血清水平升高引起的罕见疾病，通常源于垂体腺瘤。这些激素的过度分泌会导致脊柱和外周关节异常。特别是，GH 的反应参与了体外脊柱韧带骨化的发生，特别是后纵韧带（OPLL）的骨化。然而，由于肢端肥大症和 OPLL 是罕见疾病，我们很少同时遇到它们。据我们所知，这是英语文献中首例报道的肢端肥大症并发 OPLL 引起的胸髓病。
一名 47 岁女性因下肢无力和感觉异常、步态障碍和膀胱障碍就诊，无任何外伤史。患者最显著的症状是截瘫，我们诊断为颈椎和胸椎 OPLL 引起的脊髓病。此外，由于特征性的面部特征，我们怀疑肢端肥大症，并通过增强 MRI 发现了垂体腺瘤。我们进行了颈椎和胸椎减压、后路固定和垂体腺瘤切除术。
我们报告了一例在诊断 OPLL 后发现的肢端肥大症病例。肢端肥大症的主要挑战是诊断延迟。即使在这种情况下，肢端肥大症的特征性面部特征至少在 9 年前就已经出现。早期诊断和治疗肢端肥大症可以改善预后，并通过早期干预降低 GH 和 IGF-1 的暴露，似乎可以抑制韧带骨化的进展。骨科医生和神经外科医生需要牢记肢端肥大症与骨骼/关节病变和脊柱韧带骨化有关，应旨在早期诊断肢端肥大症。
