Dystrophic Scoliosis in Neurofibromatosis

type 1 and Rib-head Resection: A Case

Report

Misbahuddin*, R. Andhi Prijosedjati **

*Resident of Orthopaedic & Traumatology Faculty of Medicine, Sebelas Maret University** Division
of MusculoSkeletal Tumor, Faculty of Medicine Sebelas Maret University- Moewardi Hospital,

Prof. Dr. dr. R. Soeharso Orthopaedic Hospital,Surakarta

Abstract

Neurofibromatosis is often associated with a dysplastic progressive scoliosis.1,2 Enlargement of the

neural foramina by erosion of adjacent neurfibromas occasionally predisposes to the adjacent
attached rib entering the spinal canal.3–8 We describe such a case in which partial rib excision and
later posterior spinal fusion. There is still no consensus on the management of severe intracanal RH
dislocation in neurofibromatosis type 1 dystrophic kyphoscoliosis. This study notes the early cord
function impairment signs, reports a serious complication in a susceptible cord, identifies possible
mechanisms of injury, and discusses the management of intracanal RH dislocation presented in the
literature.

Introduction

Neurofibromatosis type 1 (NF1), also known as von Recklinghausen’s disease, is a single gene
hamartomatous disease inherited by the autosomal dominant trait [1, 2]. The relentless
deterioration of the short dystrophic curves, which leads to acute kyphosis and possible vertebral
subluxation, mandates surgical stabilization [1, 3]. Intracanal rib head (RH) dislocation at the convex
of the dystrophic curve may impinge on the cord and constitute another cause of neurology [3–9].
The “painful rib hump” sign caused by the RH dislocation has recently been described [8]. Even
though there are recent reports of retraction of the RH away from the cord along with curve
correction [2, 10, 11], there is still no consensus among authors that the excision of the dislocated
RHs is indicated in symptomatic NF1- dystrophic curves [5, 8, 12, 13].The preoperative identification
of a “cord at risk,” the susceptibility of the cord to intraoperative manipulations, and the sequence of
an iatrogenic injury are not fully described yet.

CASE REPORT

A 13-year-old premenarchal girl presented with back pain due to dystrophic NF1 left thoracic
kyphoscoliosis (Figure 1). She feel weakness and it’s difficult to walk without assistance. She had
bilateral brisk patellar reflexes and a left four beat ankle clonus. She presented to our scoliosis clinic,
a left-sided prominent rib hump associated with a 60° thoracic scoliosis was seen.
Figure 1. Presenting clinical feature neurofibromatosis type 1 in a 13-year-old girl,

Radiologic examination demonstrated a right thoracic curve from T7 to T11, measuring 60° by the
Cobb method (Fig. 1). Preoperative MRI revealed a compression of spinal cord to the right side at the
level T8/T9-T10-T11 . at the apex of the kyphosis with a penetrating
left 9th-T10, RH adjacent to the cord (Figures 1(b)–1(d)).
Figure 2. Preoperative anteroposterior roentgenogram showing a sharp thoracic curve measuring
60° by the Cobb method. angled thoracal scoliosis;

Three dimensional CT scan revealed protusion of rib head at the level of T 9 –T 10 about 1.9 cm
cause stenosis of canal spinal. the cephalad end of the ninth rib on the convex side of the curve
through an enlarged intervertebral foramen into the spinal canal, where it was severely compressing
the spinal cord (Fig. 2, left).

Figure 3. axial CT image of the apical vertebra showing rib head penetration into the spinal canal
touching the spinal cord

After thorough evaluation, A combined fusion and pesterior rib head incission to decompress of the
protruding right ninth rib into the thoracic canal. We had planned to excise the whole posterior third
of the rib.
Figure 4. 3-dimensional CT image demonstrating rib heads through the neural foramen;

The rib was adherent to the cord. Because of this, the small intraspinal segment of the rib was left in
situ, and a 5-cm segment starting at the nerve-root foramina was excised with the rib periosteum.
Figure 4. T2-weighed MRI where protrusion of the ninth rib on the convex side of the curve
impinging on the spinal cord

Fifteen days postoperatively the patient was ambulatory with crutch. Postoperative radiographs
evidenced a main thoracic curve of 36∘ (60% correction). Following a laminectomy, the dislocated
RH, which was not adherent to the dura but was impinging on the cord, was excised.

Wake-up test showed no spontaneous movement in the lower limbs with good upper limbs
movement. Postoperative neurological examination showed grade 4 muscle power in all muscle
groups of the both lower limb. Fine touch and proprioception remained intact bilaterally.
Figure 5. T2 weighed MRI revealed a compression of spinal cord to the right side at the level T8/T9-T10-
T11 . at the apex of the kyphosis with a penetrating left 9th-T10, RH adjacent to the cord
Discussion
A short-segment and sharply angulated curve normally used for describe dystrophic
scoliosis, and the vertebral bodies is associated wedging and scalloping. And
dystrophic scoliosis could be accompanied by the phenomena including vertebral
body rotation, widening of the intervertebral foramina and penciling of rib head; and it
could tend to develop a severe deformity. 1

In young patients with progressive dystrophic deformities, it is believed that only

applying posterior spinal fusion is contraindicated. In accordance with the literature
report, after applying posterior spinal fusion alone, pseudarthrosis is occurred to be
up to 60%. In spite that some surgeons may perform posterior fusion with abundant
12

autologous bone graft and pedicle screw instrumentation, which is of perfect long-
term results, anterior and posterior spinal fusion with segmental instrumentation and
bone grafting are the more predictable and successful procedure. What's more,
13–15

based on the acknowledged natural history of certain progression for this type, the
young child with dystrophic scoliosis greater than 40° is applicable to be fused. And
4,8

because of poor growth potential of the involved segments, minimal stunting of

growth is caused by an early fusion. 2

There is a question raised in severe scoliosis, that is, will posterior surgery alone is
adequate or both anterior and posterior surgeries are a necessity. As the
anterior/posterior approach is concerned, the approach combines anterior distraction
with posterior compression of the vertebrae; as a result, it causing a more
comprehensive correction of the deformity possible. The more extensive operation is
necessary, but the operation involves complications with higher risk, including
reduced respiratory function and excessive bleeding. In the contrast, the posterior
approach only is of fewer invasions, while it has been associated with high rates of
instrumentation failure, pseudarthrosis, and postoperative progression of scoliosis. 16,17
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