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Open access

Epidural lipomatosis with foci of hemorrhage and acute compression of the spinal cord in a child with CLOVES syndrome: illustrative case

Dmytro Ishchenko, Iryna Benzar, and Andrii Holoborodko

BACKGROUND

Congenital lipomatous overgrowth, vascular malformations, epidermal nevi, spinal/skeletal anomalies, and/or scoliosis (CLOVES) syndrome is the most recently described combined vascular anomaly characterized by congenital excessive growth of adipose tissue, vascular malformations, epidermal nevi, and skeletal deformities. This condition exhibits a significant variability in clinical manifestations and a tendency for rapid progression and affects extensive anatomical regions. Information regarding the association of epidural lipomatosis with low-flow venous lymphatic malformations is rare, with few reports in the literature.

OBSERVATIONS

The authors present a case of a 6-year-old girl who was admitted to the emergency department complaining of rapidly progressing weakness in her lower extremities and partial loss of sensation in the inguinal area. Radiologically, an extradural mass was identified at the T2–6 level, causing acute spinal cord compression. Urgent decompression and partial resection of the mass were performed. Despite satisfactory intraoperative hemo- and lymphostasis, postoperative lymphorrhea/seroma leakage was encountered as a delayed complication and was managed conservatively.

LESSONS

CLOVES syndrome is characterized by the combination of various clinical symptoms, not all of which are included in the abbreviation, as well as a progressively deteriorating course, the emergence of new symptoms, and complications throughout the patient’s life. This necessitates ongoing monitoring of such patients.

Open access

Treatment of an anterior cervicothoracic myelomeningocele together with spine deformity correction in a child: illustrative case

Hudin N Jackson, Nealen Laxpati, and David F Bauer

BACKGROUND

Anterior cervicothoracic myelomeningoceles are a rare pathology. In reported cases, treatment has included shunting, isolated resection and repair without deformity correction, or isolated deformity correction without meningocele repair. The authors describe a pediatric patient with an anterior cervicothoracic myelomeningocele presenting with progressive neurological decline, who underwent simultaneous treatment of the myelomeningocele to detether the spinal cord and achieve major correction of the scoliotic deformity.

OBSERVATIONS

A 15-year-old girl was born with C7-T1-T2 hemivertebrae and anterior cervical myelomeningocele at C7–T1. She developed progressive cervical thoracic scoliosis, left hemiparesis initially, and additional right hemiparesis eventually. She underwent surgical repair via C7, T1, and T2 corpectomies with intradural detethering of the spinal cord. The scoliosis was treated with C7–T2 Ponte osteotomies and C2–T5 posterior fixation, followed by anterior reconstruction with a titanium cage and anterior plate from C6 to T3. The myelomeningocele was adequately treated with good correction of the patient’s deformity. The patient had postoperative improvement in her strength and solid arthrodesis on postoperative imaging.

LESSONS

The authors describe the successful treatment of an anterior cervicothoracic myelomeningocele and associated scoliosis in a child. This is a unique report of a combined strategy to achieve both deformity correction and detethering of the spinal cord.

Open access

The complex treatment paradigms for concomitant tethered cord and scoliosis: illustrative case

Rose Fluss, Riana Lo Bu, Andrew J Kobets, and Jaime A Gomez

BACKGROUND

Scoliosis associated with tethered cord syndrome is one of the most challenging spinal deformities to manage. Multiple surgical approaches have been developed, including traditional staged and concomitant procedures, spine-shortening osteotomies, and individual vertebral column resections.

OBSERVATIONS

A 10-year-old female presented with congenital kyphoscoliosis with worsening curve progression, tethered spinal cord, and a history of enuresis. The scoliosis had progressed to a 26° coronal curve and 55° thoracolumbar kyphosis. Preoperative magnetic resonance imaging of the spine revealed a tethered cord between the levels of L3–4 and a large kyphotic deformity at L1. The patient underwent laminectomy, during which intraoperative motor signals were lost. A planned hemivertebrectomy at L1 was performed prior to an L4 laminectomy, untethering of the filum terminale, and posterior spinal fusion from T11 to L2. After surgery, the patient experienced transient lower-extremity weakness, with her neurological function improving from baseline over the next 2 months. Ultimately, the goal of this surgery was to halt the progressive decline in motor function, which was successfully achieved.

LESSONS

Much remains to be learned about the treatment of this complicated disease, especially in the setting of concomitant scoliosis. This case serves to exemplify the complex treatment paradigms that exist when attempting to manage this clinical syndrome and that more remains to be learned.

Open access

Presacral mature cystic teratoma associated with Currarino syndrome in an adolescent with androgen insensitivity: illustrative case

Grant Koskay, Patrick Opperman, Frank M. Mezzacappa, Joseph Menousek, Megan K. Fuller, Linden Fornoff, and Daniel Surdell

BACKGROUND

Currarino syndrome is a rare disorder that classically presents with the triad of presacral mass, anorectal malformation, and spinal dysraphism. The presacral mass is typically benign, although malignant transformation is possible. Surgical treatment of the mass and exploration and repair of associated dysraphism are indicated for diagnosis and symptom relief. There are no previous reports of Currarino syndrome in an androgen-insensitive patient.

OBSERVATIONS

A 17-year-old female patient presented with lack of menarche. Physical examination and laboratory investigation identified complete androgen insensitivity. Imaging analysis revealed a presacral mass lesion, and the patient was taken to surgery for resection of the mass and spinal cord untethering. Intraoperative ultrasound revealed a fibrous stalk connecting the thecal sac to the presacral mass, which was disconnected without the need for intrathecal exploration. The presacral mass was then resected, and pathological analysis revealed a mature cystic teratoma. Postoperatively, the patient recovered without neurological or gastrointestinal sequelae.

LESSONS

Diagnosis of incomplete Currarino syndrome may be difficult but can be identified via work-up of other disorders, such as androgen insensitivity. Intraoperative ultrasound is useful for surgical decision making and may obviate the need for intrathecal exploration during repair of dysraphism in the setting of Currarino syndrome.

Open access

Multiple-site neural tube defects complicated by multiple-site split cord malformations and thickened filum terminale: experience at a pediatric neurosurgical teaching hospital in Ethiopia. Illustrative case

Mestet Yibeltal Shiferaw, Yemisirach Bizuneh Akililu, Bethelehem Yesehak Worku, Tsegazeab Laeke T/Mariam, and Abenezer Tirsit Aklilu

BACKGROUND

Multiple-site open neural tube defects (MNTDs) and multiple-site split cord malformations (MSCMs) are extremely rare congenital anomalies that are defined by the simultaneous noncontiguous occurrence of more than one neural tube defect (NTD) and split cord malformation (SCM), respectively, in a single case with normal neural tissue in between. This work shows the cooccurrence of MNTDs and MSCMs, which has never been reported in the literature.

OBSERVATIONS

A single-stage repair for a 13-day-old female neonate with a preoperative diagnosis of MNTDs (thoracic meningocele and thoracolumbar myelomeningocele) plus an additional intraoperative diagnosis of MSCMs (type 3c) of thoracic and thoracolumbar spine, and thickened filum terminale was done with a favorable smooth postoperative course.

LESSONS

The use of intraoperative meticulous surgical technique along with preoperative skin stigmata helped for anticipation, detection, and treatment of associated complex spinal MNTDs, especially in resource-limited settings, where preoperative magnetic resonance imaging is not routinely used. Whether to repair the MNTDs as a single- versus multiple-stage procedure is mainly a function of the patient’s tolerance to the duration of anesthesia and the anticipated blood loss for the patient’s age. The overall developmental biology and long-term clinical outcome of MNTDs compared to single NTD/SCM is poorly understood and needs further study.