Surgical advances in the treatment of neuromuscular scoliosis

Table 1 Incidence

Diagnosis	Incidence of scoliosis
Cerebral palsy	25% (GMFCS I and II) to 100% (GMFCS IV and V)
Charcot-Marie-Tooth disease	30%
Myelodysplasia	60% (lumbar level) to 100% (thoracic level)
Spinal muscular atrophy	70%
Friedreich ataxia	80%
Duchenne muscular dystrophy	90%1
Paralysis from spinal cord injury	100%

The incidence of neuromuscular scoliosis is variable. Among patients with neuromuscular disorders, the probability of developing scoliosis is inversely correlated with ambulatory ability, with a reported risk ranging from 80% to 100% in non-ambulatory patients.

¹Corticoids have lowered this percentage[47].

Table 2 Surgical risk

Surgical risk	Walking abilities	Weight	Cardiac Function	Respiratory function (VC)	Sleep	Comorbidities
Average	Ambulatory	> 40 kg	Normal	Normal	Normal	No
Increased	Ambulates with aid	20-40 kg	Reduced	Reduced, but > 50%	Hypersomnia
High	Non ambulatory	< 20 kg or obese	Significantly impaired	< 50%	Nocturnal hypercapnic Hypoventilation, Obstructive sleep apnea	Yes

In addition to neuromuscular pathology, factors such as walking abilities, nutritional status, cardiopulmonary function and the presence of other comorbidities, must be considered prior to surgery to minimize surgical risk. Morbidity is higher in non-ambulatory patients, with reduced weight and impaired cardiopulmonary function.

Table 3 Decade 1980-1989

Decade-Year of publication	Authors	Patients (n)	Neuromuscular condition	Instrumentation	Complications (number of patients)
1980-1989
1982	Allen et al[13]	10	Cerebral palsy	L-rod
1986	Sponseller et al[14]	34	Cerebral palsy	Interspinous process instrumentation
1988	Gersoff et al[16]	33	Cerebral palsy	L-rod	5 deep wound infections
					Complications rate: 15%
1989	Broom et al[18]	74	Various	L-rod	1 death;
					3 deep wound infections;
					2 pressure sores;
					6 sets of broken rods;
					1 distal rotation and migration of the rod
					Complications rate: 18%
1989	Boachie-Adjei et al[17]	46	Various	L-rod	3 cases of pseudarthrosis;
					3 deaths
					Complications rate: 13%

Most significant works published between 1980 and 1989.

Table 4 Decade 1990-1999

Decade-Year of publication	Authors	Patients(n)	Neuromuscular condition	Instrumentation	Complications(number of patients)	Outcome/Conclusions
1990-1999
1991	Gau et al[34]	68	Various	Luque-Galveston instrumentation	14 hardware problems;
					7 cases of pseudarthrosis;
					3 neurologic deficits
					Complications rate: 35%
1992	Hopf et al[35]	44	Various
1992	Neustadt et al[36]	18	Various	CDI of the pelvis	1 hardware failure;	Posterior spinal fusion with CDI of the pelvis is an effective treatment for patients with neuromuscular scoliosis.
					1 deep wound infection
					Complications rate: 11%
1992	Onimus et al[37]	32	Cerebral palsy		3 deaths;	Pain disappeared in 2/3 of cases;
					10 other	sitting position was acquired in all the cases at follow-up;
						motor possibilities improved in 25% of cases;
					Complications rate: 41%	associated medical pathologies were reduced in 67% of cases.
1996	Sussman et al[38]	25	Cerebral palsy	L-rod		Posterior fusion and instrumentation from the upper thoracic spine to L5 without anterior fusion provides adequate correction and control of spinal deformity for many patients with cerebral palsy
1997	Frischhut et al[39]	41	Various	29 L-rod, Luque-Galveston, CDI and ISOLA; 12 Harrington instrumentation	3 deep wound infections Complications rate: 7%
1997	Marchesi et al[40]	25	Duchenne muscular dystrophy	L-rod with sacral screws		In every patient, a good sitting balance could be restored after surgery

Most significant works published between 1990 and 1999. CDI: Cotrel-Dubousset instrumentation.

Table 5 Decade 2000-2011

Decade-Year of publication	Authors	Patients(n)	Neuromuscular condition	Instrumentation	Complications (number of patients)	Outcome/Complications
2000-2011
2000	Yazici et al[25]	47	Various	ISOLA-Galveston	2 deep wound infections;	ISOLA-Galveston instrumentation is as safe and effective as other types of instrumentation
					2 hardware removals;
					4 cases of pseudarthrosis;
					1 pseudarthrosis repair
					Complications rate: 19%
2009	Modi et al[20]	52	Cerebral palsy	U-rod and pedicle screws	2 deaths;	U-rod with pedicle screws provides good frontal and sagittal plane correction, as well as pelvic obliquity improvement (56% correction)
					1 neurologic deficit;
					17 respiratory complications (atelectasia, pneumonia, hemothorax)
					Complications rate: 38%
2010	Nectoux et al[21]	28	Cerebral palsy	Luque-Galveston, U-rod	1 case of blindness;
					1 death;
					16 respiratory complications (atelectasia, pneumonia, pneumothorax)
					Complications rate: 64%
2010	Modi et al[22]	27	Spinal muscular atrophy and Duchenne muscular dystrophy	U-rod and pedicle screws	1 death;	Although flaccid neuromuscular scoliosis can be corrected well with U-rod and posterior-only pedicle screws, there is a high rate of associated complications
					4 respiratory failure;
					2 neurological deficits;
					1 ileus;
					2 cases of atelectasia;
					3 UTIs;
					7 cases of coccydynia;
					1 rod dislodgement
					Complications rate: 77%
2011	La Rosa et al[24]	84	Cerebral palsy	Universal clamps, hooks and L-rod	5 respiratory complications
					Complications rate: 6%

Most significant works published between 2000 and 2011.