La tunisie Medicale - 2017 ; Vol 95 ( n°07 ) : 505-506
[ 800 times seen ]
Article

Case: A 41 week male infant, was born to a 28 year-old, gravida 1 para 1 mother who pregnancy was uncomplicated. He was born by cesarean section performed for fetal distress. No resuscitation was required. Apgar scores were 9 and 10 at 1 and 5 minutes, respectively. Birth weight, occipital circumference and length were all in 25th percentile. He was admitted in our unit at birth for posterior spinal dysraphism with a right lumbar hernia.
Physical examination indicated a soft reductible mass at right lateral abdominal wall prominent on crying (Fig 1) with a 3x3 cm open lumbosacral myelomeningocele.
The neurological examination of lower limbs was unremarkable. Anal tone was normal.
Cranial ultrasound showed an Arnold Chiari malformation with triventricularhydrocephalus.
Thoraco-abdominal X- ray revealed hemivertebras at D7 to D10 level with homolateral right rib agenesis. There was a thoraco lumbar scoliosis with convexity to the left (Figure 2).
Magnetic resonance imaging revealed a lumbo-costovertebral syndrome with diastematomyelia and myelomeningocle. The patient was then referred to neurosurgery clinic with an orthopedic follow up.
Conclusion: Lumbocostovertebral syndrome includes hemi-vertebra, congenital absence of ribs, anterior myelomeningocoele and abdominal wall muscular hypoplasia. Our case is the first reporting the combination with a posterior spinal dysraphism and a diastematomyelia.


Figure 1: Right-sided congenital lumbar hernia


Figure 2: Digital X- ray showing a soft tissue shadow under the right costal margin, hemivertebras at D7 to D10 level with homolateral right rib agenesis and a lumbar scoliosis with convexity to the left.

Reference
  1. Singh G, Ahuja S, Kumar R, Chandra A, Ojha B, Singh C, Gupta S. Posterior spinal dysraphism with lumbocostovertebral syndrome.Br J Neurosurg. 2010 ; 24:216-8.
  2. Mamta S, Vivek M, Anup M, Vishesh J, Swarup D. Intercostal variant of lumbar hernia in lumbocostovertebral syndrome: our experience with 6 cases. J PediatrSurg2011 ; 46 :1974-77.
  3. Emrah S, Tuğrul T, Halil A , Aysegül C, Tevfik G. Lumbo-costovertebral syndrome with diastematomyelia. PediatrInt2008 ; 50 : 600-2.
Login
E-mail :
Password :
Remember Me Forgot password? Sign UP
Archives
2018
January
February
March
April
May
June
July
August
September
October
November
December
Keywords most used
Child treatment surgery diagnosis prognosis Tunisia Children Crohn’s disease Breast cancer screening Cancer epidemiology Ulcerative colitis Risk factors obesity
Newsletter
Sign up to receive our newsletter
E-mail :
Stay in Touch
Join Us! !