American Journal of Medical Genetics 43:751-752 (1992)

Brief Clinical Report Paraplegia and Congenital Contractures as a Consequence of Intrauterine Trauma Leah K. Weyerts, Marilyn C. Jones, and Hector E. James Divisions of Dysmorphology and Clinical Genetics (L.K.W.,M.C.J.) and Neurosciences (H.E.J.), Children’s Hospital and Health Center, San Diego, California We present a newborn infant with paraplegia and contractures of the lower limbs, consistent with neurologic injury rather than malformation. The mother was involved in a severe motor vehicle accident during the sixth month of pregnancy. We propose that this infant’s injuries are a result of that accident. 0 1992 Wiley-Liss, Inc.

KEY WORDS fetal trauma, trauma during pregnancy, congenital contractures INTRODUCTION Fetal injuries as a result of maternal trauma are relatively rare. However, cranial injury [Crosby, 19741 and fractures of the clavicle and long bones [Pearlman et al., 19901have been reported. Fetal spinal cord injury as a result of maternal trauma has not been documented.

CLINICAL REPORT We evaluated an infant who was noted a t birth to have paraplegia of the lower limbs. She was the 3,180 g product of a 40-week-gestation born in vertex position to a 35-year-old G4P3SAB1 woman. At birth, the infant had a normal cranium and neuromuscular exam of the upper limbs. The hips were held in flexion and there was minimal motor function of the lower limbs. However, the movement of the toes was somewhat preserved. There was a pes cavus deformity on the left and a metatarsus adductus on the right, with wasting of the muscle mass of both legs. The hips were asymmetric with mild decrease in muscle mass of the buttocks. Radiographic studies showed a dysplastic right hip with a shallow acetabulum, and a superiorly dislocated right femoral head. Asymmetric ossification of the femoral heads was noted. The lumbosacral spine was Received for publication July 11,1991; revision received November 1, 1991. Address reprint requests to Marilyn C. Jones, M.D., Director, Dysmorphology and Clinical Genetics, Children’s Hospital and Health Center, 8001 Frost Street, San Diego, CA 92123.

0 1992 Wiley-Liss, Inc.

normal with no evidence of sacral agenesis or dysplasia. Magnetic resonance imaging of the lumbosacral spine was also unremarkable. Most importantly, the distal spinal cord was normal in size and shape with a wellformed conus. An EMG was done at age 5 weeks and was compatible with chronic neuropathic damage. There was preservation of the motor response of the foot muscles. This pattern was thought to be more consistent with damage to the sciatic nerve or paraspinus muscles than with a polyneuropathy. The pregnancy was unremarkable until 24 weeks, when the mother was in a motor vehicle accident. She was a passenger in an automobile which was hit broadside by another vehicle.The mother states she was wearing a lap-belt restraint but was thrown to one side, sustaining a blow t o her head and abdomen. She was taken to an emergency room where a head laceration was sutured and her cervical spine evaluated. The fetal heart tones were recorded as 160 beatstminute but no ultrasound study was done. There were no recorded concerns of placental abruption and the mother denies any bleeding. No Kleihauer-Bettke test was performed. Her cervical spine and skull films were normal, and she was diagnosed as having lumbosacral strain. She was reevaluated the next day because of headaches, but no new findings were made. The infant’s mother recalls that after she felt the impact, the baby felt as though it became “stuck” in the upper right quadrant. She also states that fetal activity diminished strikingly after the motor vehicle accident. Since birth, the infant has been followed in spinal defects clinic and has shown some progress in her ability to move her lower limbs. The infant has had an open reduction and medial capsulorrhaphy of the right hip with an adductor transfer to the ischium and a psoas tenotomy. She has also undergone serial casting of her left foot for the clubfoot deformity but will most likely require surgery to correct this. She has not had any urologic problems and has a normal bladder and kidneys by ultrasonography. Except for her lower limbs, her social and neurologic development has been normal.

DISCUSSION Reported cases of injury t o the fetus throughmaternal trauma are rare. This is thought to be due t o the protec-

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Fig. 1. Patient. Note the asymmetric hip alignment and pes cavus deformity.

tive nature of the maternal soft tissues, uterus and amniotic fluid. In addition, during the first 12 weeks of pregnancy, the uterus remains a pelvic organ and therefore protected by the mother’s bony pelvis. However, in the case of severe maternal trauma such as motor vehicle accidents, shearing forces between the placenta and the wall of the uterus occur that may cause abruption of the placenta. This is the most common cause of fetal death in cases of maternal trauma [Pearlman et al., 19901. Later in pregnancy when the uterus is more exposed, the fetus is vulnerable to more direct trauma. Fetal death has also been reported as a result of cranial injuries to the fetus due to maternal pelvic fracture a t the end of the third trimester when the fetal head is engaged [Pearlman et al., 19901. In fetuses that survive maternal trauma, fractures are the most frequently reported injury. These include

fractures of the clavicle, femur, tibia, and humerus [Crosby, 19741. Spinal cord injury as a consequence of maternal trauma in pregnancy has not been reported. However, spinal cord injury from the torquing forces placed upon an infant’s spine during a traumatic birth have been documented. [Painter and Bergman, 19821. In addition, in vitro studies in animals have demonstrated spinal cord injury as a result of blunt trauma [Balentine et al., 19881. In the case presented here, significant paraplegia with subsequent atrophy and deformation of the lower limbs was present at birth without evidence of a malformation of the vertebral column or spinal cord. The only significant event during the pregnancy was severe trauma during the sixth month with an abrupt decrease in fetal movement. We propose that the nerve injuries found in this infant were a direct consequence of the motor vehicle accident. Two possible mechanisms are proposed: (1)Due to the nature of the accident and the position of the lap-belt, torquing of the fetal spinal cord may have occurred with consequent paraplegia. (2) Vascular compromise of the artery of Adamkiewicz may have occurred, although spinal atrophy was not demonstrated on imaging. More cases need to be evaluated to explore the validity of this association.

REFERENCES Balentine JD, Greene WB, Bornstein M (1988): In vitro spinal cord trauma. Lab Invest 58:92-100. Crosby WM (1974): Trauma during pregnancy: Maternal and fetal injury. Obst & Gynecol Surv 29:683-699. Painter MJ, Bergman I(1982): Obstetrical trauma to the neonatal central and peripheral nervous system. Sem Perinat 6:89-104. Pearlman MD, Tintinalli JE, Lorenz RP (1990):Blunt trauma during pregnancy. NEJM 323:1609-1613.

Paraplegia and congenital contractures as a consequence of intrauterine trauma.

We present a newborn infant with paraplegia and contractures of the lower limbs, consistent with neurologic injury rather than malformation. The mothe...
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