According to a document from 2025, this 15-year-old female with Hurler syndrome and severe congenital kyphosis/scoliosis (with prior C1–C2 decompression laminectomies) was admitted on 4/14/2025 for operative management of spinal deformity/stenosis. The discharge summary describes the index operation as posterior spinal laminectomy, vertebral column resection (VCR), and osteotomies, with temporary lumbar stabilization placed and the planned fusion aborted after temporary loss of motor function during the case. Reported EBL ~1.2 L with intraoperative resuscitation including ~6 L crystalloid, 1 L albumin, platelets, FFP, PRBCs, and cell-saver.
The 4/14/2025 operative note details extensive deformity work including pedicle screw placement, a three-column osteotomy/VCR at L2 with cage placement, and attempted correction using rod reduction and contouring. During reduction, there were losses of MEPs in the lower extremities and anal sphincter, and the team reports implementing a “spinal cord injury protocol” including increasing MAPs, without improvement. MEPs reportedly returned to baseline after rod removal, but recurrent attempts at correction again produced MEP loss.
The surgeons then performed multilevel thoracic/lumbar laminectomies (T8–T9 through L2–L3) because the canal was described as “quite stenotic,” with concern that stenosis plus correction was driving the neuromonitoring changes; despite this, further correction attempts again caused MEP loss. An intraoperative CT reportedly showed no cord impingement or screw malposition, and the decision was made to stabilize the lumbosacral spine and abort further correction/fusion at that time.
Subsequently during the admission, the record reflects new/worsening weakness/paralysis prompting additional interventions and repeat imaging. On 4/18/2025, a halo device was applied (listed in the operative history).
On 4/22/2025, MRI of the cervical spine described cord signal abnormality (C2–C3, “may represent edema”) and an enhancing/high-proteinaceous or hemorrhagic posterior/left epidural collection from C2–3 to T5 with mass effect on the thecal sac and cord, performed for bilateral upper and lower extremity weakness/paralysis; she then underwent C5–C6 laminectomy and epidural hematoma evacuation with cervical and thoracic epidural drains (also listed in the operative history).
Follow-up MRI (4/23) continued to describe residual epidural hematoma at multiple cervical levels and thoracic canal epidural hematoma (T1–T6) with mild mass effect, along with ongoing cord T2 hyperintensity at C2–C3.
Later operative history indicates 5/2/2025 cervical re-exploration/laminoplasty with halo removal (noted in the 5/9 operative note’s summary of prior procedures), followed by 5/9/2025 completion surgery: removal/reinsertion of instrumentation and posterior spinal fusion T2–pelvis with grafting, performed in the setting of persistent severe neurologic deficit (the 5/9 note describes her as ASIA B with no motor function at that time).
Near the end of the admission, a 5/12/2025 diaphragm ultrasound documented right diaphragmatic motion with no definitive motion of the left and bilateral pleural effusions (performed for concern for diaphragmatic paralysis).
PC death on 5/13/2025 with preliminary cause acute hypoxic respiratory failure and includes quadriplegia on the problem list.
We request review of the original operative note and subsequent care to determine SOC breaches that would have led to premature death.
**Please see attached OP note for reference**
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Do you believe there might have been medical error?
This is a very high risk neurologic injury case due to several factors including Hurler's syndrome (MPS patients have much higher baseline risk of spinal stenosis and neurologic injury, and this patient already had prior cervical decompression), and severe kyphoscoliosis necessitating VCR. There isn't enough information to comment whether medical error was present based on what is given, and perhaps even unlikely from the index case. In my opinion the surgeons performed a very high neurologic risk surgery at the index procedure, followed a standard of care protocol once neuromonitoring signals were lost, and aborted further correction. Where more information is needed is to see the time course of the cervical epidural hematoma and injury. Was this during the index case (ie: was there evidence of upper extremity neuromonitoring signal loss at all rather than lower extremity)? Was the head positioned in traction at all during the index case that was a prolonged case? Was MRI of the cervical spine adequately assessed for adequate space available for the cord prior to proceeding with the index procedure? Was this new upper and lower extremity weakness after halo cervical traction was applied (were upper extremity neurologic checks documented after progressive weight addition for cervical traction) and if so, how quickly were these findings identified, and halo traction aborted and MRI obtained? These are key questions. In summary - I think the post index procedure time course is also important in this case. The neurologic signal loss in the index procedure is not unexpected for this severity of scoliosis, in patient with MPS - if the question is whether this is related to the index surgery, less likely than not if only lower extremity signals were lost. There are other factors possibly more likely.
Do you believe there might have been causation (i.e. the medical error resulted in an injury)?
To be honest, more detailed analysis is needed.
What makes you a good expert for this case?
Practicing academic pediatric spine surgeon, and my practice is >75% pediatric spinal deformity or pediatric spine conditions.
How often do you encounter cases similar to this one in your practice?
This is a relatively rare case scenario, and all cases with MPS are complex. I consult and treat several skeletal dysplasia patients a year
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