Adult Split Cord Malformations

Adult SCM has predominantly been reported in females (Table 9.1). Russel et al² in a review on all the case reports on adult SCM found 45 patients with a F:M ratio of 3.4:1. The cause of this gender predilection is unknown. This condition presents in a wide age group, ranging from 18 to 88 years,² with no definite peak in any decade.

Table 9.1 Management and outcomes reported in world literature on adult SCM
Author	No. of patients	Age and gender	Cord ending/type of split and level	Surgery	Complication	Follow-up/postop status
Shukla et al²	7	Not mentioned separately	2– type I, 5– type II (level not mentioned)	Detethering with removal of spur	NA	Improved or stable
Viswanathan et al²	2	67/F	L4/L2–3 type II spur, T12 syrinx	Laminectomy + removal of spur with detethering	None	5.5 years/improved
Viswanathan et al²	2	53/F	L3/type I spur at L1–2	Laminectomy + removal of spur with detethering	None	1 year/improved
Davanzo et al²	1	43/M	L5/Duplicated filum terminale	Laminectomy and detethering	Pseudomeningocele–re-exploration and repair	6 weeks/improved
Kim et al²	1	34/F	L5/C7–D11 type II spur	Laminectomy + removal of spur with detethering	None	7 years/improvement (pain and motor)
Borkar et al²	7	Not mentioned separately	M/c thoracic f/b lumbar (level not mentioned)	Laminectomy + removal of spur with detethering	7% patients had long-term deficits	Improvement/stable
Klekamp²	24	46 ± 13 years (range 23–74 years)	22– lumbar, 2– cervical	Detethering and spur removal	NA	Improvement/stable
Rahimizadeh et al²	1	72/F	C5–D3 type I spur	None	NA	18 months/stable
Conti et al²	2	87/M	Intramedullary dermoid cyst with type II spur at L1	Laminectomy and excision of tumor	None	1 month/improvement (pain and sensorimotor)
Conti et al²	2	38/F	L1–2 intramedullary teratoma with type II spur	Laminectomy and excision of tumor	None	2 months/improvement (pain)
Méndez et al²	1	88/F	L2–5 type I spur with L4 vertebral body collapse	L4 vertebroplasty	NA	Improvement (pain)
Armstrong et al²	1	37/F	D1 type II spur	NA	NA	NA
Porensky et al²	1	54/F	L4, D4–D9, D11–L3 type II spur, type I spur at D8, D7–8 extramedullary epidermoid cyst	Laminectomy + removal of spur with tumor excision	Pulmonary embolism	8 months/improvement (pain, sensorimotor, sphincter deficits with stable scoliosis)
Guilloton et al²	2	40/F	Type II spur at L2	None	NA	NA
Guilloton et al²	2	54/F	Type II spur D12–L1	None	NA	NA
Goina et al²	1	68/F	L4, type II spur at L2 with cranial syrinx	NA	NA	NA
Lewandrowski et al²	1	44/F	L1–2 type I spur	Laminectomy with removal of fusion mass and spur	Pseudomeningocele	1 year/improvement
Pallatroni et al²	1	78/F	L5, L3–4 type II spur	None	None	NA
Soni et al²	1	30/F	D7–8 type I SCM with extramedullary neuroenteric cyst	Laminectomy + excision of bony spur and cyst	None	3 month/improvement
Quinones-Hinojosa et al²	1	73/F	L3/type II spur D12–L3	Laminectomy + removal of spur with detethering	None	6 weeks/improvement (pain and motor)
Sheehan et al²	1	38/F	D1–D3 type II spur, D2–4 intramedullary epidermoid, cranial syrinx	Laminectomy + removal of spur with tumor excision	None	Improvement (pain, sensorimotor, sphincter deficits)
Hüttmann et al²	12	Not mentioned separately	Not mentioned separately	NA	NA	8 years (mean)/improvement/stable
Wenger et al²	1	38/F	L3–4 level type II spur	None	NA	Improved on conservative management
Kaminker et al²	1	38/M	L4/ type I spur L2–3	Laminectomy + extradural removal of spur	None	2 years/improvement (pain)
Iskandar et al²	13	Mean–34 years	Not mentioned separately	NA	NA	Improvement/stable
Prasad et al²	2	28/M	L4–5 type I spur	Laminectomy + removal of spur with detethering	None	Improvement (pain and sensorimotor)
Prasad et al²	2	22/F	D11–12 type I spur	Laminectomy and spur excision	None	Improvement (pain and urinary symptoms)
Pang²	8	Not mentioned separately	6–type I, 2–type II spur	NA	NA	NA
Russell et al²– review article of all previous reported cases	45	19–76 (mean–37.8 years), 3.4:1 F:M	M/C lumbar	24 patients underwent surgery	NA	23 showed improvement

Abbreviation: SCM, split cord malformation.

Clinical Features

Backache is the most common presenting complaint in adult SCM (Fig. 9.1). It may present with or without features of radiculopathy in the distribution of the involved nerve roots.²^,²^,²^,²^,²^,²^–²^,² For most adults, this may be the only complaint, with the pathology being found upon investigation into the cause of the pain. Adults present with some mild form of sensorimotor deficits or bladder symptoms. Unlike the pediatric population where these symptoms are investigated earlier, adults tend to ignore the symptoms attributing it to some other cause. The lack of knowledge about this condition, amongst patients and doctors, is the reason for some of the cases of late diagnosis of this condition in the 7th and 8th decades, despite the patient having obvious cutaneous markers or musculoskeletal deformity. Hypertrichosis²^–²^,²^,²^,² is the most common associated cutaneous marker reported in adult SCM, followed by dermal sinus.²^,² Orthopedic abnormalities like scoliosis or club foot are less commonly found in adult SCM, with most patients with these conditions being diagnosed and managed appropriately in childhood itself. Development of new deficits, or the progression of minor established ones, finally lead these patients to seek medical attention in late adulthood.²

Fig. 9.1 A 23-year-old female presented with a history of progressive low backache for the past 4 years. She had no urinary complaints and had no neurological deficits. (a) Local examination showed hypertrichosis over the lower back. (b, c) CT scan of the lumbosacral spine showed a L2-L3 bony spur. (d, e) MRI confirmed the diagnosis of Type I SCM with low lying cord at L5 (arrow). She underwent laminectomy, spur removal and detethering of the low-lying cord. (f) Intraoperative image showing the split cords with the bony spur being removed (asterisk). She had an uneventful postoperative period and is doing well at follow up.