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CASE REPORT |
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Year : 2013 | Volume
: 6
| Issue : 1 | Page : 102-104 |
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A case of double meningomyelocoele with hydrocephalous in a four months' infant
Anuja A Goyal, Vangapuram Raghavachari Rangachari, Chhaya Suryavanshi
Department of Anaesthesiology and Critical Care, Padmashree Dr. D. Y. Patil Medical College, Hospital & Research Centre, Dr. D. Y. Patil Vidyapeeth, Pimpri, Pune, India
Date of Web Publication | 14-Mar-2013 |
Correspondence Address: Anuja A Goyal Department of Anaesthesiology and Critical Care, Padmashree Dr. D. Y. Patil Medical College, Hospital & Research Centre, Dr. D. Y. Patil Vidyapeeth, Pimpri, Pune India
Source of Support: None, Conflict of Interest: None | Check |
DOI: 10.4103/0975-2870.108665
Meningomyelocoele is a developmental congenital disorder caused by failure of the neural tube to close during the first month of embryonic development thus presenting with herniation of neural elements along with meninges. Myelomeningocoeles most commonly occur in the lumbosacral region, but can also occur at any level in the neuraxis, including occipital/suboccipital areas or nasally (encephalocoele).We report a rare case of double meningomyelocoele in a 4-month-old infant (cervical as well as thoracic meningocele) with weakness in the left lower limb (power grade 0) posted for resection with difficult intubation because of obstructive hydrocephalus, fear of rupture of neuroplaque, positioning and care of blood loss and temperature. The infant was discharged within 15 days, and his hospital stay was uneventful. Keywords: Blood loss, double meningomyelocoele, hydrocephalus, hypothermia, prone position
How to cite this article: Goyal AA, Rangachari VR, Suryavanshi C. A case of double meningomyelocoele with hydrocephalous in a four months' infant. Med J DY Patil Univ 2013;6:102-4 |
Introduction | | |
Meningomyelocoele [1] is a developmental congenital disorder caused by incomplete closure of the embryonic neural tube.Anesthetic considerations include airway management (especially in lesions of the head and neck), coexisting disease (hydrocephalus), age-related pathophysiology, positioning, protection of the neuroplaque from rupture, volume status (high third-space losses from the skin defect + accurate assessment of blood loss) and potential for hypothermia due to exposure of a large surface area. We report a rare case of cervical and thoracic meningomyelocoele posted for resection and discuss its anesthetic implications.
Case Report | | |
A 4-month-old female child weighing 4kg presented with two swellings on the back over the cervical (2cm × 2 cm) and thoracic regions (6cm × 7cm) with left lower limb weakness (power grade 0). [2] The baby was a full-term normal delivery, weighing 2.5 kg at the time of birth. Vitals were within normal limits. The cardiovascular and respiratory system had no abnormality detected. No other congenital abnormality was detected. A thorough pediatric reference was done. Magnetic resonance imaging (MRI) of the back showed large spina bifida at the D10-L5 level with widening of interpedincular distance with a large meningomyelocoele with syrinx/syringomyelia in dorsal spinal cord with scoliosis of thoracic spine and gross obstructive hydrocephalus (head circumference: 40cm). [3] All other investigations were normal. In our case, the problems we anticipated were difficult intubation [4],[5] due to presence of hydrocephalus, positioning of the baby for intubation and prevention of rupture oftwo neuroplaque and then the prone position, probability of hypothermia. [6] [Figure 1], [Figure 2], [Figure 3] and [Figure 4] show the MRI findings. | Figure 2: Magnetic resonance imaging scan showing two meningomyelocoeles
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| Figure 3: Magnetic resonance imaging scan showing the axial view of the defect
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| Figure 4: Showing both the swellings with positioning in prone position on two bolsters
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Anesthetic Management
As the patient had double meningomyelocoele, i.e. two swellings thatwere present in the thoracic and cervical regions, two rings were made with proper padding with cotton of the size of the swelling so that the patient could be made supine for intubation, and the rest of the body was supported on folded sheets. As the patient had gross hydrocephalus, good extension at the neck was given. A precordial pediatric stethoscope was placed. The case was induced with oxygen:nitrous oxide 40:60 mixture, sevoflurane 7.5% [5] and then premedicated with inj atropine 0.04mg, inj fentanyl 5μg and inj Rocuronium 2.5 mg; then, after 90s, the infant was intubated with a 3.5 uncuffedsouthpole RAE endotracheal in the first attempt, followed by a throat pack being done and maintained on oxygen 33%, nitrous oxide 66%, isoflurane (0.2-0.4%) and controlled respiration. The patient was then made prone on two bolsters so that the abdomen was free for respiration and extreme care was taken to prevent accidental extubation of the tube. Intraoperatively, surgery was carried out for 3.5 h, incremental dose of inj Rocuronium 0.6mg was given four times and intra-operative fluid management was done after calculating fluid for the first and second hours (first hour 70 mL and second hour 30 mL). As there was blood loss of 120 mL, intra-operatively, 100 mL blood was given. Total intra-operative urine output was 25 mL. Surgical duration remained uneventful. The patient was reversed with inj atropine 0.08mg and inj neostigmine 0.2 mg. The throat pack was removed, proper suctioning was done and the patient was extubated in lateral position, only after the patient was fully awake, crying and moving both the upper limbs. The patient was given 100% O 2 and was then shifted to the Pediatric Intensive Care Unit (PICU) for further monitoring.
Discussion | | |
A meningoencephalocele is caused by failure of the neural tube to close during the first month of embryonic development thus presenting with herniation of neural elements along with meninges. Incidence of meningomyelocoele is 1-2 per 1000 population, more predominant in females. Meningomyelocoele is more common in the lumbosacral region, although it may occur in any region of the neuraxis. Menigomyelocoeleis caused mainly due to folic acid deficiency and some drugs like oral hypoglycaemics, anticonvulsants, obesity, etc. Meningomyelocoele repair should be done as soon as possible as delay in surgery will lead to more neurological deficits [7] as they present with varying degrees of sensory and motor deficits as well as increased risk of infection. They are also associated with many congenital defects (club foot, hydrocephalus, atrophy of bladder, prolapsed uterus, Kippel-Fiel syndrome, congenital cardiac effects, etc.). First of all, the positioning and minimal handling of neck is very important in such cases as there is always a chance of rupture of neuroplaque;therefore, we had made a well-padded head ring of the size of the swelling. In approximately 90% of the cases with meningomyelocoele, hydrocephalus will be present because the displaced cerebellum intervenes with the normal flow of cerebrospinal fluid, causing excess fluid to accumulate. [8] Becauseof the presence of gross hydrocephalus, good extension was given at the neck. Asouthpole RAE endotracheal tube was used to give us the benefit of its curve so that there is no kink in the tube leading to negligible resistance. Also, the infant was made prone on two bolsters, one under the shoulders and the other below the anterior superior iliac spine, such that the abdomen was free for respiration; also, there would be no pressure on the inferior vena cava leading to back pressure on the vertebral veins and thus leading to pressure on the epidural veins and more bleeding. Prone position itself has its own problems due to risks of accidental extubation and ventilation perfusion (V/Q) mismatch; therefore, continuous monitoring of airway and other vitals was done. Infant's head and extremities were rolled in cotton pads to prevent hypothermia, and were kept in a relaxed position. Meticulous monitoring of blood loss was done and replaced immediately. Post-operatively, good oxygenation was done to prevent any post-op hypoxia. The patient was then shifted to the PICU with warmer to prevent hypothermia.
Conclusion | | |
Meningomyelocoele case requires thorough pre-operative anesthetic assessment to rule out the congenital defects present, if any. Also, care of airway and prevention of rupture of neuroplaqueare important aspects. Proper intra-operative and post-operative care is must. The baby was discharged from the hospital in 15 days.
References | | |
1. | Available from: http: //en.wikipedia.org/wiki/Spina_bifida as on.[Last accessed on 2012 Feb 03]. |
2. | Leech R. Myelodysplasia, Arnold Chiari malformation and hydrocephalus.In: Leech R, Brumbaric R, editors.Hydrocephalus: Current clinical concepts. St. Louis: Mosky Yearbook; 1991. p. 129. |
3. | Blanco G, Melman E, Curain V, Moyao D, Ortiz-Monastero F. Fiberoptic nasal intubation in children with anticipated and unanticipated difficult intubation. Pediatr Anesth 2001;11:49-53. |
4. | Bissonette B, Sadeghi P. Anaesthesia for neurosurgical procedures. In: Gregory GA, editro. Pediatric Anesthesia. Vol. 393, 4 th ed. Edinburgh: Churchill Living stone: 2002. p. 4411-3. |
5. | Wheeler M. Management strategies for the difficult pediatric airway. Anesthesiol Clin North Am 1998;16:753. |
6. | Hooper VD, Chard R, Clifford T, Fetzer S, Fossum S, Godden B, et al. ASPAN's evidence-based clinical practice guideline for the promotion of perioperative normothermia. J Perianesth Nurs 2009;24:271-87. [PUBMED] |
7. | Ahmed NK, Ahmed M, Mendolkar S, Abdul Majeed G. Malhadar; Lateral Thoracic Meningocele: Anaesthetic Implications. AI Ameen J Med Sci 2008; 1:136-8. |
8. | Foster MR, Lorenzo CT. Jun 7 2012 Spina Bifida. Available fromhttp://emedicine.medscape.com/article/311113-overview. [Last accessed on 2012 Aug 24]. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4]
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