A rare event of perioperative spinal subarachnoid hemorrhage in mixed connective tissue disease: a case report

Introduction

Spinal subarachnoid hemorrhage (SSH) represents a rarity with only 0.05–1.5% of all cases of subarachnoid hemorrhage (SAH) (1). Causative factors can vary from trauma (including lumbar puncture), vascular malformation, neoplastic lesion, defective coagulation or systemic such as autoimmune or connective disease, the first two being the most common ones (2-11). Instead, the occurrence of idiopathic spontaneous SSH is extremely rare (12). As described in some case reports, intra-abdominal pressure (IAP) may have an impact in the occurrence of the event (12-14).

Mixed connective tissue disease (MCTD) plays a non-marginal role in the development of neurological complications, being responsible for some of the following conditions: lacunar infarctions, mononeuritis, transverse myelitis (15). MCTD refers to an overlapping condition of different autoimmune diseases such as lupus erythematosus, systemic sclerosis, rheumatoid arthritis, polymyositis and dermatomyositis. The disease is related to the expression of anti U1 ribonuceloprotein (U1-RNP) antibodies (16,17). Although connective disorders are associated with an increased risk of cerebral SAH (4), a case of SSH associated with MCTD has not yet been described.

The aim of this case report is to described the unique occurrence of SSH in a patient with MCTD, its first manifestations, symptoms and clinical signs, treatment and its evolution in order to contribute to the knowledge of this neurological complication in the literature. This case is written following the CARE reporting checklist (available at https://jss.amegroups.com/article/view/10.21037/jss-23-59/rc).

Case presentation

A 69-year-old white woman presented to the emergency room in the evening with lower abdominal pain, vomiting since the afternoon with elevated inflammatory parameters and signs of peritonism on clinical examination. Figure 1 shows the chronological timeline of the events. The abdominal computed tomography (CT) scan showed no abnormalities. Diagnostic laparoscopy was performed and then converted to laparotomy based on the evidence of small bowel ileus. One meter necrotic small bowel was removed. At the end of surgery, the patient received a transverse abdominis plane block for pain control of the anterior abdominal wall. Surgery lasted 165 minutes, while the pneumoperitoneum was maintained for 5 minutes.

Figure 1 Timeline of interventions and outcomes. MCTD, mixed connective tissue disease; U1-RNP, anti-U1 ribonucleoprotein; CT, computer tomography; MRI, magnetic resonance imaging.

The patient revealed a history of hypothyroidism and MCTD since three years prior to presentation with positive U1-RNP and anti-nuclear antibodies (ANA). MCTD was under therapy with prednisolone and rituximab for a short time in the first year due to polyarthritis and joint pain. The therapy was then stopped in the absence of symptoms. At the time of hospital admission, MCTD was quiescent.

The first postoperative day ran uncomplicated. During the second postoperative day, at midday the patient developed abdominal belt-shaped lumbar exanthema, and 2 hours later complained of sudden severe back pain radiating to both legs. Physical exam revealed the patient to be afebrile, alert, oriented and normotensive. Neurological examination revealed diminished lower extremity strength (M0–1/5) and absence of bilateral sensation, the examination was difficult to perform due to patient non-compliance. A CT-scan showed spinal canal hyperdensity. The patient was intubated and sedated because of uncontrolled pain despite high dose analgesics and to perform a magnetic resonance imaging (MRI). MRI revealed an SSH from T6 to the sacrum without signs of myelopathy (Figures 2-5). An additional subdural component could not be ruled out with certainty. There was no evidence of vascular malformations or dural fistula.

Figure 2 MRI (T2 sequence, sagittal) showing a spinal subarachnoid hemorrhage, with blood from the level of thoracic vertebrae 6 to the sacral and with ventral displacement of the spinal cord without signs of myelopathy. An additional subdural component cannot be ruled out with certainty. No evidence of a vascular malformation or dural fistula as the cause. The arrow indicates the spinal subarachnoid hemorrhage with ventral displacement of the spinal cord. MRI, magnetic resonance imaging.

Figure 3 MRI (T1 sequence, sagittal) showing a spinal subarachnoid hemorrhage, with blood from the level of thoracic vertebrae 6 to the sacral and with ventral displacement of the spinal cord without signs of myelopathy. MRI, magnetic resonance imaging.

Figure 4 MRI (T2 sequence, axial), the arrow shows the spinal subarachnoid hemorrhage compressing the spinal cord at thoracic vertebrae 12 level. MRI, magnetic resonance imaging.

Figure 5 MRI (T2 sequence, axial) showing a spinal subarachnoid hemorrhage that compress the spinal cord at lumbal vertebrae 2 level. MRI, magnetic resonance imaging.

Neurosurgery was consulted and decided against surgical intervention due to low probability of cord compression, but an urgent neurological examination was mandatory. At the completion of the diagnostic assessment, the patient was rapidly extubated and complained of pain and loss of sensitivity below the T11 dermatome bilaterally. Muscle strength showed distally left M2/5, right M3/5, and proximally M4/5, bilaterally. Sphincter tonus showed to be normal and meningismus signs were present. Conservative therapy was continued given the slight neurological improvement. During the first hours in the intensive care unit (ICU), we observed a progressive hypotension with signs and symptoms of poor perfusion. Lactate levels increased up to 6.5 mmol/L with normal hemoglobin levels. After volume resuscitation with 1,500 mL crystalloids the shock-like state slowly recovered. The next day, the strength improved, and the sensation fully recovered. Surprisingly, creatine kinase increased until a peak of 4,000 U/L. Inflammation markers (C-reactive protein) increased slightly during the first 24 hours but recovered spontaneously. The coagulation parameters were normal, and a spinal anesthesia or therapeutic anticoagulation was not performed. A follow-up MRI was not performed during the following days due to the rapid improvement in the neurological examination. After 4 days of hospital care, the patient was discharged with full recovered muscle strength and sensation with recovery of ambulation. Six months after discharge, the clinical follow-up revealed a normal neurological status and MRI showed no abnormalities.

All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Helsinki Declaration (as revised in 2013). Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

Discussion

We report a case of spontaneous SSH occurred after abdominal surgery in the context of MCTD. Only approximately 20 cases of spontaneous SSH have been described (12-14,18-22). Severe back pain and headaches are frequently observed. Some of these cases experienced stress situations or higher IAP (coughing, defecation and scuba diving). Maiti et al. [2017] (6) hypothesized that minor trauma as an increased IAP can trigger SSH, causing tearing of vessels within the subarachnoid space and their rupture. In our patient, the event manifested two days after surgery, probably caused by: first, the increased IAP through the initial pathology, the small bowel ileus; second, the minor trauma through the abdominal surgery; and third, the postoperative increased IAP due to the inflammatory edematous intra-abdominal environment.

MCTD includes connective tissue fragility, susceptible to traumatic events. This is also true for the spinal arachnoid mater. Masdeu et al. [1979] (23) postulated that an increase in IAP such as abdominal intervention, could be responsible for the rupture of the radiculomedullary vessels that cross the subdural and subarachnoid space. The fragility of the arachnoid mater together with increased IAP in our patient caused the vessels rupture within the subarachnoid space of the lumbar spine, resulting in SSH. The occurrence of a prodromal cutaneous rash, a lumbal exanthema belt-shaped, was likely due to irritation of the radicular nerves in their specific dermatomes.

Spontaneous SSH is a challenging event that may be associated with neurological deficits. The decision to perform decompressive surgery is based on neurological deterioration and myelopathy.

Komiyama et al. [1997] (1) postulated that the location of the SSH (ventral versus dorsal type) plays a role in the decision to perform surgical decompression, being the ventral-type less associated with neurological deficits and therefore, conservative treatment is favored. In our case (ventral displacement of spinal cord with dorsal SSH), a conservative management was made due to the rapid improvement of neurological functions.

MRI choice for radiological examination was essential in this case to exclude vascular malformations and other neurological complications associated with MCTD such as transverse myelitis.

A limitation of this case report is the difficulty in collecting data on the patient’s MCTD, given that the diagnosis took place outside our institution as well as the therapy, as well as its follow-up. Taking the patient’s subjective data shows another limitation of this case report, the patient was not questioned about how she experienced the event and its result.

Considering that the event and its therapy as well as the follow-up took place within the same institution shows a strength of this case report, where the information was not fragmented.

Our patient, after being diagnosed with SSH after abdominal surgery and with a positive past medical history for MCTD, was successfully treated conservatively. The diagnostics was effective and the results showed a total recovery of neurological functions over the long-term. The decision not to perform surgery was correct. For this reason, every surgeon, especially neurosurgeons must be aware that this neurological complication exists and is probably more relevant in patients with connective disorders.

Patient perspective

From patient perspective, this neurological complication was difficult to handle because after the abdominal operation and the marked improvement of the symptoms, this second event occurred, which paralyzed the patient causing her immense pain. In addition to this, the conservative therapy did not initially convince the patient given the symptoms she presented, but she trusted the physicians’ opinion and fully recovered.

Conclusions

The patient experienced an SSH triggered by the fragility of the spinal arachnoid mater, resulting in the breakdown of the radiculomedullary veins in the context of MCTD and a perioperative increase in IAP. In this report, we describe the first case of spontaneous SSH associated with MCTD.

Two main criteria are considered for intervention: neurological evolution and signs of myelopathy on MRI. A conservative treatment is recommended for patients with improving neurological status.

We recommend caution in patients with connective tissue diseases who undergo thoracic or abdominal surgery and be aware for neurosurgeons that SSH is possible although rare and that conservative treatment may be considered.

Acknowledgments

Funding: None.

Footnote

Reporting Checklist: The authors have completed the CARE reporting checklist. Available at https://jss.amegroups.com/article/view/10.21037/jss-23-59/rc

Peer Review File: Available at https://jss.amegroups.com/article/view/10.21037/jss-23-59/prf

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://jss.amegroups.com/article/view/10.21037/jss-23-59/coif). The authors have no conflicts of interest to declare.

Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Helsinki Declaration (as revised in 2013). Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.

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