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Year : 2013  |  Volume : 61  |  Issue : 6  |  Page : 692--693

Anterior cervical meningomyelocele in a neonate

G Srihari, Hosmani Sharan Basappa, Bada Vijay Kumar, PN Harish 
 Department of Neurosurgery, Narayana Hrudayalaya Hospital, Hyderabad, Andhra Pradesh, India

Correspondence Address:
G Srihari
Department of Neurosurgery, Narayana Hrudayalaya Hospital, Hyderabad, Andhra Pradesh

How to cite this article:
Srihari G, Basappa HS, Kumar BV, Harish P N. Anterior cervical meningomyelocele in a neonate.Neurol India 2013;61:692-693

How to cite this URL:
Srihari G, Basappa HS, Kumar BV, Harish P N. Anterior cervical meningomyelocele in a neonate. Neurol India [serial online] 2013 [cited 2020 Nov 27 ];61:692-693
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Full Text


Anterior cervical meningomyelocele (ACM) is rare. The patient expired in the follow up after 2 months. We report this extremely rare neural tube defect in a 1-month baby girl and the challenges and complications we faced in its management. This case is the only one described in neonate.

A month-old baby girl presented with repeated respiratory tract infections at another facility. Chest X-ray revealed right lung haziness. Suspecting right pleural effusion, inadvertently right intercostal drain (ICD) was placed and clear cerebrospinal fluid (CSF) was drained. Subsequently, the child was referred to our center. Examination in the emergency department the child was conscious, moving all limbs, oxygen saturation 90% and pus was draining at the ICD site. Magnetic resonance imaging of the entire spine showed large ACM at C6 level extending inferiorly into the posterior mediastinum and causing right lung collapse and hydrothorax [Figure 1] and [Figure 2]. The C6 vertebral body was incompletely formed in the midline and Ryles tube was displaced to the right side. A dysplastic neural placode [Figure 1] was seen at the neck of the myelomeningocele sac and the cord showed a short segment syrinx [Figure 1] at the same level. Upper dorsal scoliosis due to hemivertebrae was seen. Other relevant investigations were normal.{Figure 1}{Figure 2}

Considering her age and poor general condition, she was planned for a simple ligation of the sac and disconnection of the distal sac as dissection of sac would risk an injury to trachea/esophagus/nerves/vessels. She underwent anterior cervical approach on the right side, neck of meningomyelocele sac was exposed at C6 level, sac was opened and inspected for neural tissue at C6 level and inferiorly the sac was extending into the posterior mediastinum and engulfing the trachea/great vessels. The neck of the sac was ligated in flush with vertebral body defect with mersilk and disconnected from the rest of the sac. Fibrin glue was applied to the cut end of the ligated neck of the sac. Distal sac was not closed. The neural placode was left undisturbed. Ryles tube was not put preop and it was a mistake as we realized it in the postoperative period, because esophagus would have been identified better with Ryles tube in situ as it was displaced to the right side. Post-operatively on breastfeeding, milk leaked from the wound suggestive of esophageal tear, which was re-explored the following day. The esophageal tear was repaired by a surgical gastroenterologist and ligated neck of the sac was found to be intact with no CSF leak. However, esophageal tear leaked again and was managed thereafter conservatively. There was no CSF leak after primary surgery. Immediate post-operatively she was on the ventilator for 3 days. Ryles tube feeding was on for 3 weeks. Cefoperazone was instituted for ICD site infection as culture showed Klebsiella pneumoniae sensitive to the drug. Blood culture grew Candidiasis for which fluconazole was started. After extended stay in intensive care unit for 4 weeks, esophageal fistula and ICD site fistula closed spontaneously. However she had repeated episodes of breathlessness and fall in saturation, which were due to aspiration/infections and managed with bronchodilators, antibiotics and chest physiotherapy. Thoracotomy and evacuation of loculated collection was planned to favor lung expansion, however cardiothoracic surgeons were reluctant in view of her poor general condition. She expired 2 months after discharge presumably due to hypoxia/aspiration/sepsis.

Posterior spinal meningocele is seen in the lumbosacral region, accounting for 80% of cases. Anterior spinal meningocele is rarely seen in the sacral region and very rarely in the cervicodorsal spine. Posterior meningocele is due to defect in primary neurulation while anterior meningocele is due to incomplete development of mesoderm around the neural tube. [1] Any insult that interferes with this process is responsible for development of anterior meningocele. [1] Our patient was not properly evaluated prenatally and had normal natal/perinatal events. She progressively developed breathlessness due to aspiration and right hydrothorax was probably due to rupture of the meningocele sac. Such a presentation was reported with anterior dorsal meningocele. [2] Multiple and unusual meningocele are seen in neurofibromatosis. [3] There were no findings of neurofibromatosis in our case. Management was difficult and we could do simple disconnection of meningomyelocele at C6 level as it was close to nearby vital structures. Cystoperitoneal shunt is a simple and reliable modality of treatment in a large single loculated anterior dorsal meningocele. [3],[4] In anterior dorsal meningocele of small size, laminectomy and duroplasty can be done while in large size lesions, thoracotomy can be planned. [2],[5] Whether laminectomy and duroplasty as done for anterior dorsal meningocele [2],[5] can be tried in the cervical region is doubtful. Anterior cervical approach seems to be the most direct approach for addressing the anterior cervical meningoceles. Review of the literature [Table 1] showed only three cases of anterior meningomyelocele in the cervical region reported earlier, with limited knowledge regarding its course and management. All three cases were in adults and in females. The present case demonstrates the challenges in the management of this rare clinical entity when encountered in an infant. Esophageal tear could have been avoided with Ryles tube placed pre-operatively, meticulous dissection and left sided anterior cervical approach.{Table 1}[8]


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