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Multiple split cord malformation with dysplasia of the hip joints: A rare association or part of a sydromic presentation

1 Department of Neurosurgery, PGIMER, Chandigarh, India
2 Department of Orthopedics, PGIMER, Chandigarh, India

Date of Submission19-Jun-2021
Date of Decision26-Jan-2022
Date of Acceptance12-Mar-2022
Date of Web Publication30-Jan-2023

Correspondence Address:
Sushanta K Sahoo,
Department of Neurosurgery, PGIMER, Sector 12, Chandigarh 1600l2
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jpn.JPN_136_21



Background: Multiple level split cord malformations (SCMs) with associated skeletal abnormality are rare. Here we discuss a 3-year-old male child with double-level SCM type 1 and developmental dysplasia of the hip joints. Case: A 3-year-old male child presented with chronic constipation since birth with urinary incontinence. There was gait abnormality and he could not able stand without support. Radiology showed SCM type 1 at the level of D9-11 and L4. X-ray showed left dysplastic hip joints. Surgically both the bony spurs were resected and dural sac was repaired along with detethering of the low-lying filum. Orthopedic intervention was done after 3 months. At 18-month follow-up the child has improved bowel and bladder symptoms and could able to walk independently. Conclusion: Though rare, skeletal abnormalities like dysplastic hip joints may be associated with SCM. Early diagnosis and management of both conditions have better outcomes.

Keywords: Dysplasia of hip joint, split cord malformation, tethered cord

How to cite this URL:
Sahoo SK, Kumar A, Siroliya A, Rangasamy K, Gopinathan NR. Multiple split cord malformation with dysplasia of the hip joints: A rare association or part of a sydromic presentation. J Pediatr Neurosci [Epub ahead of print] [cited 2023 Jun 3]. Available from: https://www.pediatricneurosciences.com/preprintarticle.asp?id=368797

   Introduction Top

Split cord malformations (SCMs) are congenital anomalies that account for up to 5% of spinal cord malformation.[1] Multiple level or composite SCM is uncommon. They usually present with pain, progressive sensory-motor deficit, autonomic dysfunction, or may remain clinically silent till adult age.[1],[2] Association of multiple SCM with other congenital skeletal anomalies is unusual. Here we discuss a case of 3-year-old male child with clinical features of double-level type 1 SCM and developmental dysplasia of the hip joints.

   Case Report Top

A 3-year-old male child presented to us with features of constipation and difficulty in standing. There was also history of chronic constipation since birth with no urinary control till that time. Neurological examination revealed normal power of both the lower limbs and the left lower limb was shorter than the right one. There was no feature of skin patches or tuft of hair at the back.

For chronic constipation and difficulty in standing he was evaluated outside. He underwent barium study that showed dilated sigmoid colon with narrowing at the rectum and congenital megacolon was kept as a differential. In addition, X-ray pelvis showed left side femoral head lying outside the acetabulum suggestive of developmental dysplasia of hip (DDH) [Figure 1]. In our center, magnetic resonance imaging (MRI) of the whole spine was advised to rule out other spinal anomalies. MRI showed SCM at the level of D9-11 and L4 levels [Figure 2]. There was also low-lying filum attached to S2 level. Computed tomogram (CT) spine showed bony spur at the level of D9-11 and L4 level. The reconstructed CT images showed the bony spurs that arises from the vertebral body and extend up to the posterior elements completely dividing the spinal canal at both levels [Figure 3]. The child was planned for surgery for the splitcord malformation. There was two separate cord structure with two separate dural sac at both the dorsal and lumbar level. The bony septum was resected at both levels and dural sac was reconstructed to a single tube. Filum was cut at its lower attachment. The child recovered uneventfully. He was being managed by the orthopedics team for the dysplasia of hip joint. The child underwent open surgical reduction of the left hip along with pelvic (Salters) osteomy and proximal femoral varus derotational osteotomy. The iliac crest graft used in Salter’s osteotomy was stabilized with two threaded Kirschner wires, and the proximal femoral osteotomy was stabilized using an external fixator across the hip joint. After fixation, intraoperatively the hip joint was found to be stable. Postoperatively, the child developed pin tract infection of the external fixator and was treated with antibiotics (amoxicillin and clavulanic acid combination tablets for 3 weeks). Later, 3 months following surgery, the external fixator was removed, and clinically hip joint was found to be stable.
Figure 1: (A) Barium study showing dilated sigmoid colon with narrowing at the rectum. (B) X-ray pelvis showing bilateral hypoplasia of the acetabulum and the head of femur in a dislocated position

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Figure 2: (A) T2W MRI sagittal cut showing bony anomalies at the level of D9-11 and L4 level. The T1W axial image at the level of D10 (B) and T2W axial image at L4 (C) showing two cords with intervening bony septum

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Figure 3: (A and B) Reconstructed CT images and sagittal CT section showing hypertrophy of the posterior elements at the D9-11 and L4 level. Note the defect in spinous process and lamina both above and below the deformity. (C and D) Axial CT images showing the bony spur extending from the vertebral body to the posterior elements completely dividing the spinal canal at both the dorsal (C) and lumbar level (D)

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An 18-month follow-up radiograph [Figure 4] showed the femoral head located well inside the acetabulum with satisfactory reduction. The child is able to walk without any pain, with the shoe heel raised on the left lower limb to compensate for shortening.
Figure 4: Two-year follow-up Antero-posterior view radiograph of pelvis with both hip joints showing left-sided hip is reduced well in the acetabulum socket and having pelvic obliquity. The two Kirschner wires used to stabilize Salter’s osteotomy needs to be removed

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   Discussion Top

SCMs are congenital abnormalities where the cord is split sagittally by a bony or fibrous band. Pang et al.[3] have proposed the unified theory of embryogenesis to explain these developmental anomalies. The presence of an accessory neuroenteric canal between the yolk sac and amniotic cavity through the midline embryonic disc is considered the basic embryological error. SCMs may present in association with other malformations depending upon the persistence of the accessory neuroenteric canal. As the mesenchymal cells invest upon the neuroenteric canal, it may result in a pure bony septum with two cords and dural sacs (type 1 SCM) or a cartilaginous partition with two cords and a single dural sac (type 2 SCM). Again partial regression of the neuroenteric canal can also explain the coexistence of a dermal sinus or neuroenteric cyst.[4]

Multiple level SCM or composite SCM are rare. These cases accounts for less than 1% of all SCM. Presence of more than one accessory neuroenteric canal is responsible for such rare congenital malformations.[5] Alzhrani et al.[1] have proposed a new classification to include various types of multiple level SCM and multiple level bony septum were subgrouped under SCM IIIA. However, presence of multiple level bony septum (SCM 1) is reported only in few cases.[1],[2] Spinal deformities were also observed in association with several anorectal malformations.[6] These associations may be a part of a syndrome-like VACTERL (vertebral anomalies, anal atresia, cardiac malformations, tracheoesophaseal fistula, renal and limb anomalies), OEIS (omphalocele, extrophy, imperforated anus, spinal defects). These multisystem anomalies are usually seen with trisomy 13, 18, and 21; deletion of 22q.[6] Such dual pathology was also reported in the past. Primary ossification center for vertebra appears at eighth week of gestation and the hip joint is differentiated from the musculoskeletal system at this time. Any defect in the embryogenesis will give rise to skeletal abnormality like DDH, sacral agenesis along with neural defect like tethered cord and SCM.[7] As the caudal portion of the notochord and paraxial mesoderm differentiates in a similar time frame, some embryological insult at this period has resulted in DDH and SCM in our patient.

Clinically these patients may present with features of pain, progressive sensory or motor deficit of the lower limbs with or without bladder and bowel involvement secondary to tethering of the cord. In our case the child has lower limb length discrepancy. To our surprise, he had also DDH and multilevel SCM 1. Both these conditions can explain this clinical feature but the overall growth of one limb may be less than the other one in SCM. Additionally chronic constipation since birth also raised the possibility of some congenital anorectal malformation like congenital megacolon as supported by the barium study. SCM was not considered the first differential due to absence of apparent kyphoscoliosis or any cutaneous stigma. The screening MRI only revealed the multiple level SCM with low-lying tethered cord. Then the child underwent surgery for SCM first. The clinical improvement in the bowel and bladder symptoms suggested the cord tethering was responsible for these dysfunctions. The gait abnormality was probably due to both SCM and dysplasia of hip joint. After orthopedic intervention and with physiotherapy the child could able to walk independently at 18-month follow-up.

   Conclusion Top

Multiple level SCM is uncommon and can be associated with other skeletal abnormalities like developmental dysplasia of the hip joint. Screening radiology of the hip joints with spine should always be considered in pediatric patients having gait abnormality with bowel and bladder dysfunction as early treatment of both the conditions has a better outcome.

Compliance with ethical standards

The parents of the patient have consented for the publication of this case.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Alzhrani GA, Al-Jehani HM, Melançon D Multi-level split cord malformation: do we need a new classification? J Clin Imaging Sci 2014;4:32.  Back to cited text no. 1
Borkar SA, Mahapatra AK Split cord malformations: a two years experience at AIIMS. Asian J Neurosurg 2012;7:56-60.  Back to cited text no. 2
Pang D, Dias MS, Ahab-Barmada M Split cord malformation: part I: a unified theory of embryogenesis for double spinal cord malformations. Neurosurgery 1992;31:451-80.  Back to cited text no. 3
Sahoo SK, Salunke P, Randhawa MS, Hc P, Chatterjee D, Vincent JV Neurenteric cyst masquerading as acute flaccid paralysis in a 2-month-old infant. World Neurosurg 2020;142:385-7.  Back to cited text no. 4
Akay KM, Izci Y, Baysefer A, Timurkaynak E Composite type of split cord malformation: two different types at three different levels: case report. J Neurosurg 2005;102:436-8.  Back to cited text no. 5
Alamo L, Meyrat BJ, Meuwly JY, Meuli RA, Gudinchet F Anorectal malformations: finding the pathway out of the labyrinth. Radiographics 2013;33:491-512.  Back to cited text no. 6
Morimoto T, Sonohata M, Mawatari M Ipsilateral hip dysplasia in patients with sacral hemiagenesis: a report of two cases. Case Rep Orthop 2015;2015:854151.  Back to cited text no. 7


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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