Halo gravity traction utilized in the treatment of early-onset scoliosis for patients with Prader-Willi Syndrome: A case report

Catherine M. Call; Bruce MacWilliams; Kristen Carroll; Joshua Klatt

doi:10.1016/j.jorep.2025.100553

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Case Report

2025

:4;

100553

doi:

10.1016/j.jorep.2025.100553

Halo gravity traction utilized in the treatment of early-onset scoliosis for patients with Prader-Willi Syndrome: A case report

Catherine M. Call^a, Bruce MacWilliams^b,c, Kristen Carroll^b,c, Joshua Klatt^b,c,

a Tufts University School of Medicine, Boston, MA, USA

b Shriners Children's, Salt Lake City, UT, USA

c Department of Orthopaedic Surgery, University of Utah, Salt Lake City, UT, USA

⁎Corresponding author: Joshua Klatt. Joshua.Klatt@hsc.utah.edu

Received: 2025-1-9, Accepted: 2025-1-14, Published: 2025-1-17

The Authors

Disclaimer:
This article was originally published by Reed Elsevier India Pvt. Ltd. and was migrated to Scientific Scholar after the change of Publisher.

Abstract

This report presents the cases of three pediatric patients with severe scoliosis and Prader-Willi Syndrome (PWS) who underwent halo gravity traction (HGT).

A retrospective review of three children with PWS treated with HGT application followed by growth-sparing surgery was performed. All three patients were receiving growth hormone. Patient 1 aged 13 (BMI 29), patient 2 aged 6 (BMI 19), and patient 3 aged 8 (BMI 16) were treated with HGT applied for an average duration of 24 days. Following HGT and growth-sparing surgery, the coronal curve corrected in all patients while the sagittal curve progressed in two patients. There were no HGT complications and one surgical complication of transient intraoperative neurophysiological monitoring signal loss. Longer term follow-up (average 2.3 years) showed difficulty of maintained correction. All three patients tolerated HGT well.

PWS is associated with musculoskeletal and behavioral traits that can impact scoliosis treatment planning. This report describes three patients with PWS safely treated with HGT prior to surgical intervention for severe scoliosis. HGT has been utilized for severe scoliosis to maximize preoperative health status and has been shown to decrease surgical risks by allowing for gradual curve correction. Augmentation of scoliosis treatment with HGT can be considered in patients with PWS.

Keywords

Halo gravity traction (HGT)

Prader-Willi syndrome (PWS)

Pediatric

Scoliosis

MAGEC rod

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1 Introduction

Prader-Willi syndrome (PWS) is a rare genetic disorder with characteristic musculoskeletal features including early scoliosis, obesity, joint hyperlaxity, and delayed bone age, as well as cognitive impairment and patterns of obsessive compulsive and oppositional behaviors.¹^,² Among this patient population, the prevalence of scoliosis is reported to be 15–86 %.^3–7 Management of scoliosis in patients with PWS can be challenging and notable for high surgical risk.⁸ Deferring posterior fusion until skeletal maturity is desirable, with serial casting, bracing, and growing spine instrumentation utilized for severe early-onset curves.⁹^,¹⁰ The behavioral phenotype of PWS can impact patients’ ability to tolerate casting and bracing methods, necessitating early surgical intervention. Halo gravity traction (HGT) has emerged as an adjunct therapy for treatment of patients with severe early-onset scoliosis.¹¹^,¹² HGT permits the gradual correction of spinal deformity over time in the frontal, sagittal, and axial planes, and is often utilized prior to surgical intervention.¹³ We report HGT being well-tolerated prior to growing spine instrumentation in three patients with PWS.

2 Case report

Between October 2004 and July 2021, three patients with PWS were treated with HGT at our institution. Patient 1 was male (age 13, BMI 29), patient 2 was male (age 6, BMI 19), and patient 3 was female (age 8, BMI 16). All patients were receiving growth hormone (GH), a mainstay of treatment in PWS(¹⁴,¹⁵) important for decreasing obesity and increasing vertical growth.¹⁶ GH treatment duration was unclear. All patients were referred for magnetically expandable control (MAGEC) rod placement following unsuccessful casting and bracing protocols.

All patients underwent halo pin insertion surgery under local anesthesia and sedation. Six to eight halo pins were placed and tightened to 6- to 8-inch pounds of torque to minimize loosening. Traction began immediately with a weight of 1–2 kg gradually increased at a rate of 1.0–1.5 kg daily. Goal maximum traction was 30%–50 % of the patient's body weight, consistent with literature recommendation.¹⁷^,¹⁸ Patient 1 reached a traction weight of 30 % body weight; patient 2 reach 44 %, and patient 3 reached 48 %. Neurological checks were performed 2–3 times daily by a hospitalist or pediatric spine surgeon. Pin sites were cleaned daily and evaluated for infection. HGT duration was dependent on patient tolerability, progress of curve correction, and operative planning, and monitored with clinical evaluation and radiologic measurements. The traction period for patient 1 was 31 days, while 21 days for both patients 2 and 3. There were no significant changes to body weight over this period. No HGT-related complications—nystagmus, pin loosening, pin infection, nor neurologic complications—were observed. All three patients tolerated HGT well. Patient 2 experienced transient intraoperative neurophysiological monitoring (IONM) signal loss; the surgeons were unable to place a 2nd left-sided rod at the time of surgery. There were no long-term sequalae from this event. Post-operatively, all patients recovered well. Overall, radiographic outcomes demonstrated correction of the major coronal Cobb angle following HGT and surgical intervention; patient 1 experienced a coronal curve correction from 98° to 57°, patient 2 corrected from 72° to 52°, and patient 3 corrected from 90° to 69°. In the sagittal plane, two patients experienced progression of their curve following HGT and surgical intervention. Patient 1 experienced sagittal curve progression from 83° to 92°, patient 2 corrected from 75° to 67°, and patient 3 progressed from 45° to 58°. At an average of 2.3 years follow-up, two patients had progression of their major coronal Cobb angle and two patients had progression of their sagittal Cobb angle. See Table 1.

Table 1 Demographic, curve correction, and HGT data for the three patients with PWS treated with HGT prior to growth-sparing surgery at our institution.

	Patient 1	Patient 2	Patient 3
Demographics
Sex	Male	Male	Female
Age at presentation (years)	13	6	8
BMI at presentation	29	19	16
Laterality of curve	Right	Left	Right
*Coronal curve*
At presentation (°)	98	72	90
Following HGT (°)	77	66	80
Following growth-sparing surgery (°)	57	52	69
At last post-operative follow-up, average 2.3 years (°)	73	83	65
*Sagittal curve*
At presentation (°)	83	75	45
Following HGT (°)	64	40	40
Following growth-sparing surgery (°)	92	67	58
At last post-operative follow-up, average 2.3 years (°)	90	83	75
*HGT*
Time in HGT (days)	31	21	21
Total HGT weight (lbs)	30	20	20
% HGT/Body weight	30	44	38

3 Discussion

Surgical treatment of severe scoliosis in patients with PWS is associated with high complication risk, including proximal junctional kyphosis, infection, transient paraplegia, pseudarthrosis, delayed wound healing, re-operation, and permanent spinal cord injury.⁸ Among patients with PWS undergoing growth-sparing surgery, Oore et al. found an average of 2.2 complications occurred per patient.⁹ Of our three patients treated, only one complication occurred; transient IONM signal loss, which resolved without morbidity or mortality.

Preoperative traction has been shown to improve preoperative nutritional status and pulmonary function in patients with severe scoliosis, with some clinical data supporting the hypothesis that gradual traction can also reduce the risk of neurologic damage.¹⁹^,²⁰ This growing body of literature supports that preoperative HGT can help to medically and nutritionally optimize patients and can maintain and improve the deformity correction achieved while minimizing peri- and post-operative complications.

Use of HGT in children with PWS is rare, even at our institution specializing in pediatric spine, likely out of concern for poor tolerability given the behavioral phenotype associated with PWS. Our results indicate HGT was well-tolerated and demonstrated modest curve correction prior to operative intervention. Curve progression post-HGT and post-MAGEC rods highlights the severity of scoliosis in these patients and having HGT available in the toolbox of modalities to attempt prior to fusion may be beneficial. Further research is needed to understand and maximize management of severe scoliosis in patients with Prader-Willi Syndrome.

4 Conclusion

HGT was well-tolerated in treatment of severe scoliosis in three pediatric patients with PWS. One patient experienced transient IONM signal loss during growth-sparing surgery. We highlight consideration of HGT as adjunct therapy for scoliosis treatment in patients with this condition.

CRediT authorship contribution statement

Catherine M. Call: performed data acquisition and drafted the manuscript reviewed and edited by the senior authors. All approved the version to be published. Bruce MacWilliams: All authors whose names appear on this submission made substantial contributions to warrant authorship, were involved in conception of the work. Kristen Carroll: All authors whose names appear on this submission made substantial contributions to warrant authorship, were involved in conception of the work. Joshua Klatt: All authors whose names appear on this submission made substantial contributions to warrant authorship, were involved in conception of the work.

Compliance with ethical standards

The study was conducted in accordance with the guidelines of the institutional review board (IRB) at Shriners Children's Salt Lake City. Informed consent was waived due to the retrospective, observational format of the study.

Funding

CC received funding from the University of Utah Women in Academic Orthopedics (WIAO) Summer Scholarship 2022 and Shriners Salt Lake City Helen Lemieux Summer Research Scholarship 2022 in the form of a stipend to complete research as a visiting medical student.

References

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Benarroch F., Hirsch H.J., Genstil L., Landau Y.E., Gross-Tsur V., . Prader-Willi syndrome: medical prevention and behavioral challenges. Child Adoles. Psych. Clini. North Am.. 2007;16(3):695-708.
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[Google Scholar]
Holm V.A., Cassidy S.B., Butler M.G., et al . Prader-Willi syndrome: consensus diagnostic criteria. Pediatrics. 1993;91(2):398-402.
[Google Scholar]
Gunay-Aygun M., Schwartz S., Heeger S., O'Riordan M.A., Cassidy S.B., . The changing purpose of Prader-Willi syndrome clinical diagnostic criteria and proposed revised criteria. Pediatrics. 2001;108(5):e92-e.
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[Google Scholar]
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[Google Scholar]
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Bridges N., . What is the value of growth hormone therapy in Prader Willi syndrome? Arch Dis Child. 2014;99(2):166-170.
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Rinella A., Lenke L., Whitaker C., et al . Perioperative halo-gravity traction in the treatment of severe scoliosis and kyphosis. Spine. 2005;30(4):475-482.
[Google Scholar]
Sponseller P.D., Takenaga R.K., Newton P., et al . The use of traction in the treatment of severe spinal deformity. Spine. 2008;33(21):2305-2309.
[Google Scholar]
Koller H., Zenner J., Gajic V., Meier O., Ferraris L., Hitzl W., . The impact of halo-gravity traction on curve rigidity and pulmonary function in the treatment of severe and rigid scoliosis and kyphoscoliosis: a clinical study and narrative review of the literature. Eur Spine J. 2012;21:514-529.
[Google Scholar]
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Introduction

Case report

Discussion

Conclusion

CRediT authorship contribution statement

Informed consent and compliance with ethical standards

Compliance with ethical standards

Funding

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[1] Cassidy S.B., Driscoll D.J., . Prader–willi syndrome. Eur J Hum Genet. 2009;17(1):3-13.
[Google Scholar]

[2] Benarroch F., Hirsch H.J., Genstil L., Landau Y.E., Gross-Tsur V., . Prader-Willi syndrome: medical prevention and behavioral challenges. Child Adoles. Psych. Clini. North Am.. 2007;16(3):695-708.
[Google Scholar]

[3] Nakamura Y., Murakami N., Iida T., et al . The characteristics of scoliosis in Prader–Willi syndrome (PWS): analysis of 58 scoliosis patients with PWS. J Orthop Sci. 2015;20(1):17-22.
[Google Scholar]

[4] Holm V.A., Cassidy S.B., Butler M.G., et al . Prader-Willi syndrome: consensus diagnostic criteria. Pediatrics. 1993;91(2):398-402.
[Google Scholar]

[5] Gunay-Aygun M., Schwartz S., Heeger S., O'Riordan M.A., Cassidy S.B., . The changing purpose of Prader-Willi syndrome clinical diagnostic criteria and proposed revised criteria. Pediatrics. 2001;108(5):e92-e.
[Google Scholar]

[6] Nagai T., Obata K., Ogata T., et al . Growth hormone therapy and scoliosis in patients with Prader–Willi syndrome. Am J Med Genet. 2006;140(15):1623-1627.
[Google Scholar]

[7] Kroonen L.T., Herman M., Pizzutillo P.D., MacEwen G.D., . Prader-Willi syndrome: clinical concerns for the orthopaedic surgeon. J Pediatr Orthop. 2006;26(5):673-679.
[Google Scholar]

[8] Weiss H.-R., Goodall D., . Scoliosis in patients with Prader Willi Syndrome–comparisons of conservative and surgical treatment. Scoliosis. 2009;4(1):1-8.
[Google Scholar]

[9] Oore J., Connell B., Yaszay B., et al . Growth friendly surgery and serial cast correction in the treatment of early-onset scoliosis for patients with Prader-Willi syndrome. J Pediatr Orthop. 2019;39(8):e597-e601.
[Google Scholar]

[10] van Bosse H.J., Butler M.G., . Clinical observations and treatment approaches for scoliosis in Prader–Willi syndrome. Genes. 2020;11(3):260.
[Google Scholar]

[11] Welborn M.C., Krajbich J.I., D'Amato C., . Use of magnetic spinal growth rods (MCGR) with and without preoperative halo-gravity traction (HGT) for the treatment of severe early-onset scoliosis (EOS) J Pediatr Orthop. 2019;39(4):e293-e297.
[Google Scholar]

[12] Iyer S., Duah H.O., Wulff I., et al . The use of halo gravity traction in the treatment of severe early onset spinal deformity. Spine. 2019;44(14):E841-E845.
[Google Scholar]

[13] McIntosh A.L., Ramo B.S., Johnston C.E., . Halo gravity traction for severe pediatric spinal deformity: a clinical concepts review. Spine Deform.. 2019;7:395-403.
[Google Scholar]

[14] Dykens E.M., Roof E., Hunt‐Hawkins H., . Cognitive and adaptive advantages of growth hormone treatment in children with Prader‐Willi syndrome. JCPP (J Child Psychol Psychiatry). 2017;58(1):64-74.
[Google Scholar]

[15] Bridges N., . What is the value of growth hormone therapy in Prader Willi syndrome? Arch Dis Child. 2014;99(2):166-170.
[Google Scholar]

[16] Tauber M., . Effects of growth hormone treatment in children presenting with Prader-Willi syndrome: the KIGS experience. 2007:377-388.
[Google Scholar]

[17] Rinella A., Lenke L., Whitaker C., et al . Perioperative halo-gravity traction in the treatment of severe scoliosis and kyphosis. Spine. 2005;30(4):475-482.
[Google Scholar]

[18] Sponseller P.D., Takenaga R.K., Newton P., et al . The use of traction in the treatment of severe spinal deformity. Spine. 2008;33(21):2305-2309.
[Google Scholar]

[19] Koller H., Zenner J., Gajic V., Meier O., Ferraris L., Hitzl W., . The impact of halo-gravity traction on curve rigidity and pulmonary function in the treatment of severe and rigid scoliosis and kyphoscoliosis: a clinical study and narrative review of the literature. Eur Spine J. 2012;21:514-529.
[Google Scholar]

[20] Garabekyan T., Hosseinzadeh P., Iwinski H.J., et al . The results of preoperative halo-gravity traction in children with severe spinal deformity. J Pediatr Orthop B. 2014;23(1):1-5.
[Google Scholar]

Halo gravity traction utilized in the treatment of early-onset scoliosis for patients with Prader-Willi Syndrome: A case report

Abstract