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Department of Plastic and Reconstructive Surgery, Wake Forest University School of Medicine, Medical Center Boulevard, Winston-Salem, NC 27157-1075, USA
Department of Plastic and Reconstructive Surgery, Wake Forest University School of Medicine, Medical Center Boulevard, Winston-Salem, NC 27157-1075, USA
Department of Plastic and Reconstructive Surgery, Wake Forest University School of Medicine, Medical Center Boulevard, Winston-Salem, NC 27157-1075, USA
Department of Plastic and Reconstructive Surgery, Wake Forest University School of Medicine, Medical Center Boulevard, Winston-Salem, NC 27157-1075, USA
The presence of a myelomeningocele at multiple levels along the spinal column is a rare event. There are only a few cases of double myelomeningocele reported in the world's literature. Large myelomeningoceles present surgical closure challenges due to the size and location of these defects. We report the case of a newborn female who had two myelomeningoceles with one at the thoracic level and the other at the lumbar level. Surgical closure must provide durable and stable coverage for the spinal cord. Stable coverage was provided for this patient using bilateral paraspinous myofascial turnover flaps. This unusual case report as well as a review of the literature is presented.
The patient was delivered by cesarean section at 39 weeks gestation. The birth weight was 3120 g (55th percentile) and the head circumference measured 34 cm (60th percentile). The mother, who denied any substance abuse, had received appropriate prenatal care. An intrauterine ultrasound had revealed an apparent neural tube defect in addition to possible ventriculomegaly. After birth, examination revealed two fluid filled sacs along the spinal column. The sacs were not bulging or erythematous and no leakage of fluid was evident. The larger of the two defects, measuring approximately 6 cm in length and 6 cm in width, was in the lumbosacral region at the level of L1 and extended inferiorly toward the sacrum. The other lesion, measuring approximately 4 cm in length and 4 cm in width, was over the thoracic region at the level of T8–T9. Neurologically the patient was normal. She was active and alert with good muscle tone and spontaneous motion of all four extremities.
On the day of birth, the patient was taken to the operating room for immediate repair of both myelomeningoceles (Fig. 1) . Closure of the neural placode and dural repair was performed by our paediatric neurosurgeon. Bilateral paraspinous myofascial turnover flaps were used to close the defects. Beginning with the thoracic defect, the paraspinal muscles were elevated laterally and turned over the dural placode medially and approximated, covering the dura completely, using a series of (Vicryl) simple interrupted sutures (Fig. 2(A–C)) . Next, the overlying fasciocutaneous layer was dissected out to the lateral wall of the abdomen bilaterally. These flaps were advanced to the midline and the deep subcutaneous tissue was approximated using 5/0 Vicryl interrupted buried suture followed by closure of the skin with running locked 5/0 polyglyconate suture. No drains were used. A sterile protective dressing was placed over the closed wounds (Fig. 3) .
Figure 1Infant at time of operative procedure showing two separate defects.
Figure 22(A–C) Diagrammatic illustration of myofascial turnover flap. (A) Myelomeningocele defect after dural cord closure. (B) Paraspinous muscles are elevated from medial to lateral. (C) Paraspinous myofascial flaps are approximated in the midline.
Following the operation, the patient remained hospitalised in the newborn intensive care unit and step down unit for seven days. During this time she had an uneventful recovery from the surgical operation. A cranial ultrasound performed on the date of birth revealed mild colpocephaly and a Chiari II malformation, but no hydrocephalus. A follow-up cranial ultrasound on the sixth day of life revealed no significant progression of the colpocephaly. She did not require a ventriculoperitoneal shunt during the initial hospitalisation. The patient was discharged and then followed as an outpatient.
The patient continues to do well and follow-up is now more than 2 years. The patient never did develop hydrocephalus and her neurosurgical follow-up has been uneventful. The patient has nearly full function of her legs with the ability to crawl and stand to a pull; however, active plantar flexion is absent and she has been using ankle-foot orthosis. She is also followed by the urology service for periodic ultrasounds to evaluate for minimal pelviectasis.
2. Discussion
Among congenital defects of the central nervous system, myelomeningocele is the most common. The incidence of myelomeningocele in the United States is between 6000 and 11 000 cases per year, or one in 800–2000 live births.
The occurrence of myelomeningocele is thought to stem from defective closure of the caudal neural tube between 26 and 28 days gestation, a process known as neurulation.
While the aetiology is probably multifactorial in nature, dietary folate supplementation for expectant mothers is effective in reducing the incidence of spinal dysraphism.
The presence of a double myelomeningocele is an extremely rare event. A thorough review of the literature reveals only seven reported cases of double myelomeningoceles. Two of these cases were associated with the presence of type 1 neurofibromatosis.
Bailey IC. Double meningocele. Arch Dis Child 1971;46:549–50. Potter EI. Pathology of the foetus and infant. Chicago: Year Book Medical Publishers; 1962. p. 571.
Bertan and Wilson described a double myelomeningocele at the thoracic and lumbosacral levels that first presented to the neurosurgery service nearly 2 weeks after birth with infection and cerebrospinal fluid discharge from the defects.
This patient also had hydrocephalus and an accompanying Arnold–Chiari malformation. In addition, lower extremity neurological deficits were noted. The patient underwent three operations including the placement of a ventriculoperitoneal shunt for hydrocephalus. Nearly 3 months after birth the patient died due to pneumonia related septicemia. Bailey also reported a thoracic and lumbosacral myelomeningocele.
Bailey IC. Double meningocele. Arch Dis Child 1971;46:549–50. Potter EI. Pathology of the foetus and infant. Chicago: Year Book Medical Publishers; 1962. p. 571.
This patient underwent operations to close the thoracic and lumbosacral defects at 8 and 14 weeks, respectively. Both operations resulted in wound infections that resolved. The child was reportedly free of neurological deficits in the lower extremities and hydrocephalus did not develop. More recently, Durmaz, et al reported the case of a double myelomeningocele at the thoracic and lumbar levels.
No congenital anomalies or hydrocephalus was observed in this patient. The defects were closed simultaneously with the use of subcutaneous fascia and normal skin.
As with our patient, in every case previously described the myelomeningoceles are at the thoracic and lumbosacral levels. Neurological function of the lower extremities was compromised in only one patient. Furthermore, only one of the cases describes the need to place a ventriculoperitoneal shunt. Most of these cases are in the minority, as it is reported in the literature that greater than 80% of patients with hydrocephalus will require VP shunting.
Central nervous system anomalies associated with meningomyelocele, hydrocephalus, and the Arnold–Chiari malformation: reappraisal of theories regarding the pathogenesis of posterior neural tube closure defects.
Surgical or wound infections were noted in two of the patients. The infant requiring the VP shunt eventually died from pneumonia related septicemia as a result of an infected VP shunt. Another case reported surgical site infections associated with both operations needed for closure of the myelomeningoceles. Infection in these cases is probably due to the delayed closure of these defects. Earlier closure is thought to significantly reduce this infection rate.
In our case there were no perioperative complications associated with closure of the patient's defect and the patient has never required placement of a VP shunt. Our patient probably benefited from immediate surgical intervention. Early closure of the neural placode has been shown to decrease risk of infection.
Good long term clinical outcomes for patients born with myelomeningocele is enhanced with a reliable and stable closure technique. The use of a paraspinous myofascial turnover flap was chosen as a result of our experience with its effectiveness in closing large myelomeningocele defects.
Bailey IC. Double meningocele. Arch Dis Child 1971;46:549–50. Potter EI. Pathology of the foetus and infant. Chicago: Year Book Medical Publishers; 1962. p. 571.
Central nervous system anomalies associated with meningomyelocele, hydrocephalus, and the Arnold–Chiari malformation: reappraisal of theories regarding the pathogenesis of posterior neural tube closure defects.
2100 Hollinswood Ave, Winston-Salem, NC 27103, USA
Steven Glazier MD
Department of Neurosurgery
Louis C. Argenta MD, Professor and Chairman
Lisa R. David MD, Assistant Professor
Department of Plastic and Reconstructive Surgery Wake Forest University Baptist Medical CenterMedical Center BoulevardWinston-Salem, NC 27157-1075, USA
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