Peer-Review Reports

1 59
2 60
3 Delayed Repair of Myelomeningoceles 61
4 Q6 Joseph C. Watson, Gary Tye, John D. Ward 62
5 63
6 64
7 65
8 Key words - OBJECTIVE: Myelomeningocele is a defect that typically is repaired surgi- 66
9 - Myelomeningocele 67
cally within the first few days of life in developed countries to minimize the risk
10 - Pediatric neurosurgery 68
- Spina bifida
of meningitis. If left unrepaired, these children may survive to have their
11 69
12 - Spinal dysraphism meningocele sac epithelialize. The surgical reduction and closure of an epi- 70
13 thelialized myelomeningocele represents a unique challenge for the neurosur- 71
Abbreviation and Acronym
14 geon because it requires a modification of the typical closure technique. 72
MMC: Myelomeningocele
15 - METHODS:
73
Our 10 years’ experience in 97 patients with the delayed (>6
16 Harold F. Young Neurosurgical Center, 74
Department of Neurosurgery, Virginia months) repair of myelomeningoceles formed the basis of this report.
17 75
Commonwealth University, Falls Church, Virginia, USA
18 - RESULTS: We present repair techniques in a child with a myelomeningocele 76
To whom correspondence should be addressed:
19 that was not repaired at birth and presented a surgical challenge. 77
Joseph C. Watson, M.D.
20 [E-mail: jwatson4@vcu.edu] 78
21 - CONCLUSION: Delayed closure of myelomeningoceles is facilitated by 79
Citation: World Neurosurg. (2013).
22 http://dx.doi.org/10.1016/j.wneu.2013.01.022 lessons learned from our surgical experience during a medical missions to 80
23 Journal homepage: www.WORLDNEUROSURGERY.org Guatemala. 81
24 Available online: www.sciencedirect.com
82
25 83
1878-8750/$ - see front matter ª 2013 Elsevier Inc.
26 All rights reserved. 84
27 has decreased (7), attributed in part to up the majority of the children who pre- 85
28 improved nutrition, the administration of sented for neurosurgical evaluation and 86
29 INTRODUCTION vitamins prenatally, and in some instances treatment at our clinic in Guatemala. This 87
30 Myelomeningoceles (MMCs) affect approx- to improved prenatal testing. clinic was sponsored by the Pediatric 88
31 imately 2500e6000 children annually in the Neurulation is the process of the forma- Foundation of Guatemala and the Interna- 89
32 United States (5). Internationally, the rate tion and closure of the neural tube, which tional Hospital for Children. The delayed 90
33 Q1 varies from a low of 0.1 per 1000 in African takes place during days 18e26; failure of presentation of these children required that 91
34 children to 12.5 per 1000 among Celtic chil- closure of the posterior neuropore leads to we adopt techniques that allowed us to 92
35 dren (7). In Guatemala, where we have made the development of a MMC. The normal reproduce the type of closure that has 93
36 our past medical missions, in the year 2000 structures (spinal cord, arachnoid, dura proven most successful in neonates with 94
37 the incidence was 2.34 per 1000 (2). Many mater, and skin) do develop but simply fail MMCs. These techniques are illustrated in 95
38 children do not have ready access to medical to approximate. The lack of skin and dura this report. Delayed closure of a small series 96
39 or neurosurgical care. A great many live leaves the neural tissue exposed to the of epithelialized MMCs has been published 97
40 in remote areas in which their mothers environment. previously (3), but the authors did not 98
41 have limited resources and may not receive The primary treatment of MMCs is early specifically address the technical chal- 99
42 prenatal care or vitamin supplementation. surgical closure of the defect to prevent lenges we faced or the solutions that we 100
43 These are the children in whom we see meningitis and to protect the neural pla- found most useful. 101
44 delayed presentations of open neural tube code. In addition, closure of the neural 102
45 defects during our screening clinic. placode is performed in hopes of prevent- 103
46 MMCs are formed by failure of closure of ing tethering of the exposed surface and an PATIENTS AND METHODS 104
47 the neural tube dorsally, leaving a mal- attempt to preserve more proximal spinal We began seeing patients in Guatemala 105
48 formed open neural placode that fuses with cord function by restoring its normal envi- City in 1996 on an annual or biannual 106
49 the skin. Most defects are in the thor- ronment. Delayed closure is associated basis. Of those patients, we treated 108 107
50 acolumbar region. The etiology is thought to with an increased incidence of meningitis children for MMC. Data were available for 108
51 be multifactorial, and includes a genetic after 72 hours. 97 of these patients. The average age at 109
52 basis, but is strongly linked to hypofunction When access to surgical treatment is treatment was 9.9 months. The age range 110
53 of a folic acidedependent process during unavailable, these children may survive, was from 1 day to 11 years of age. Children 111
54 early embryonic development (week 4). allowing their MMC sacs, including the younger than 3 weeks of age (n ¼ 4) were 112
55 There is an association with other nutritional placode, to epithelialize. Lorber and Sal- excluded form the analysis. There were 113
56 deficiencies, such as folate and zinc, and field (4) report a 100% rate of mortality if 13 thoracic and five cervical MMC repairs 114
57 some medications, especially anticonvul- untreated; however, some children survived performed. The remainder were thor- 115
58 sants (5). Overall, the incidence worldwide longer than 6 months. These patients make acolumbar or lumbar sacral MMCs. We 116

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 PEER-REVIEW REPORTS
JOSEPH C. WATSON ET AL. DELAYED REPAIR OF MYELOMENINGOCELES

117 were unable to close two of the MMCs placode can be identified near the center 175
118 because of the size of the defect. The of the sac, although compared with its 176
119 majority of these children also required perinatal appearance, the epithelialized 177
120 concomitant shunting. placode margins are less well-defined 178
121 defined, looking more like a keloid than 179
122 Surgical Technique exposed neural tissue. An incision is out- 180
123 MMCs that have not been closed at birth lined with a sterile marking pen starting in 181
124 will epithelialize if the child survives. A the normal skin midline 1 cm above the 182
125 typical example is represented in Figure 1. sac and extending at the perceived junc- 183
126 Just as with primary closure cases, careful tion of normal skin and epithelialized 184
127 consideration must be given to the size of arachnoid on each side of the sac. Infil- 185
Figure 3. Intraoperative photographs of
128 tration with 1/4 % lidocaine and epineph- Q2 186
the lesion and associated bony deformity (A) the placode being prepared for sharp
129 to judge the need for the ability to perform rine 1:400k solution was used for pain dissection of epithelium off of neural tissue, 187
130 control and hemostasis under the area of proximal spinal cord (SC) seen leading to the 188
a primary closure. There is no magic placode; and inset (B) epithelial layer
131 number for the size of the defect that can normal skin above the sac. removed from the placode.
189
132 be closed because the ability to perform Incisions were made into the sac cir- 190
133 a successful primary closure is multifac- cumferentially, usually at the top of the sack 191
134 torial and includes the infant’s size, the near the junction of the presumed placode 192
135 and the epithelialized arachnoid, with very freed from the placode. The placode was 193
amount of viable skin, and the ability to
136 careful attention paid to hemostasis then be closed (neurulated) suture (we used 194
correct associated bone deformity, espe- Q3
137 because significant skin vessels may be 5 or 6.0 PROLENE; Ethicon, Somerville, 195
cially kyphosis. Data from plastic surgery
138 encountered. Once the sac was opened, the New Jersey, USA). Inclusion of epithelium 196
suggest that the minimal-sized defect
139 anatomy became clearer as the proximal into the deep closure risks epidermoid 197
referred for plastic surgery collaboration is
140 spinal cord was then identified rostrally formation in the future, so great care is 198
18 cm2 (6).
141 leading to the placode (Figure 2). In all taken in the dissection of the placode. Just 199
A standard sterile preparation is per-
142 cases, we attempted to preserve nerve roots as in perinatal repair, closure of the raw, 200
formed because an epithelialized surface
143 arising from the placode, although the exposed surface of the placode will help 201
presents no special concerns for iodine
144 sacrifice of roots, particularly distal ones minimize scar formation and diminish the 202
intoxication (Figure 1). For children with
145 from the placode, may be done without new potential for retethering. 203
open MMC, iodine preparations are avoi-
146 deficit. At this point, closure was more stan- 204
ded for the theoretical risk of iodine-
147 At this point the surgery typically became dard. We freed the dural edges from their 205
induced hypothyroidism (1) as well as the
148 quite different from a perinatal repair; the fusion to the skin by finding the epidural 206
potential damage to neural tissue. The
149 skin was removed from the residual placode plane rostrally and proceeding circum- 207
150 by sharp dissection (Figure 3). The tech- ferentially with a sharp scissors by placing 208
151 nique requires that a plane between skin one blade in the epidural space and the 209
152 and placode be found. It was started by other in the sac. The epidural fat was our 210
153 gently retracting the top of the ellipse of guide for staying in the correct plane, as 211
154 skin and identifying the neural placode, the dorsal paraspinal fascia may be a false 212
155 which will appear gray and friable, whereas layer of dissection. The dura was then 213
156 the skin that is firm and pale. We developed mobilized toward the spinal cord. Multiple 214
157 this plane sharply with a scalpel or sharp perforating arteries needed to be coagu- 215
158 scissors until the entire skin ellipse was lated and divided to accomplish this, but, 216
159 in general, no roots needed be sacrificed. 217
160 The dura was then closed in a watertight 218
161 fashion. The skin edges were mobilized as 219
162 needed by undermining over the fascia. 220
163 Occasional facial relaxing incisions were 221
164 used. In several cases of significant bony 222
print & web 4C=FPO

165 kyphosis, removal of the kyphus with 223

166 small rongeurs was required. Rotational 224
167 skin flaps or skin grafts for larger defects 225
168 may be needed, but are the exception, not 226
169 the rule, and we had only two in the series 227
Figure 1. A 7-month-old patient with an that could not be closed primarily.
170 epithelialized myelomeningocele (MMC). 228
171 (A) photograph of the MCC. (B) Artist 229
172 rendering to emphasize the subtle Figure 2. Intraoperative photograph Technical Outcomes 230
distinction between the placode and demonstrating the proximal spinal cord (SC)
173 epithelialized arachnoid. leading to the placode (PL).
The neurological outcomes after delayed 231
174 repair were no different from our 232

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JOSEPH C. WATSON ET AL. DELAYED REPAIR OF MYELOMENINGOCELES

233 experience in the United States where the require techniques that differ from those 3. Ersahin Y, Yurtseven T: Delayed repair of large 267
234 myelomeningoceles. Childs Nerv Syst 20:427-429, Q 4 268
children are repaired acutely: no new used for perinatal MMC closure because
235 2004. 269
236 proximal deficits were encountered in our they are epithelialized and may have 270
237 series. We did have one postoperative death undergone changes associated with 4. Lorber J, Salfield SA: Results of selective treatment 271
238 from a gram-negative infection within 36 previous infection. Despite the increased of spina bifida cystica. Arch Dis Child 56:822-830, 272
239 1981. 273
240 hours of closure. Our main complication difficulty associated with dissecting 274
241 was cerebrospinal fluid leak requiring epithelium away from the spinal placode, 275
5. McLone DG: Care of the neonate with a myelome-
242 a second surgery seen in five cases. As it may be accomplished without causing ningocele. Neurosurg Clin N Am 9:111-120, 1998.
276
243 277
244
mentioned previously, there were two new deficits. We do worry, however, that 278
245 children in whom closure was not possible there will be an increased incidence of 6. Ozcelik D, Yildiz KH, Is M, Dosoglu M: Soft tissue 279
246 because of the size of the defect. epidermoid tumors, but long-term data on closure and plastic surgical aspects of large dorsal 280
247 myelomeningocele defects (review of techniques). 281
these children are lacking. Closure of the Neurosurg Rev 28:218-225, 2005.
248 282
249 MMC is indicated to prevent sac rupture, 283
CONCLUSIONS
250 correct deformity, and to untether the 7. Shurtleff DB, Lemire RJ: Epidemiology, etiologic 284
251 Delayed repair of spinal MMCs is spinal cord. The surgical technique of factors, and prenatal diagnosis of open spinal 285
252 encountered in environments in which dysraphism. Neurosurg Clin N Am 6:183-193, 1995. 286
253
closing these MMCs has been outlined 287
254
access to neurosurgical care is limited or with special attention to the removal of 288
255 impaired. Such a situation has been skin from the placode. 289
Conflict of interest statement: The authors declare that the
256 encountered in our experience of 10 years article content was composed in the absence of any
290
257 in Guatemala City. The primary goal of 291
commercial or financial relationships that could be construed
258 Q5
REFERENCES 292
259 immediate, perinatal treatment of MMCs as a potential conflict of interest.
293
1. Barakat M, Carson D, Hetherton AM, Smyth P,
260 is to prevent meningitis, protect the neural Received 15 March 2012; accepted 4 January 2013 294
Leslie H: Hypothyroidism secondary to topical
261 placode, and to prevent spinal cord teth- Citation: World Neurosurg. (2013). 295
iodine treatment in infants with spina bifida. Acta
262 296
263
ering at the placode. Children that do not Paediatr 83:741-743, 1994. http://dx.doi.org/10.1016/j.wneu.2013.01.022
297
264 have the option of immediate perinatal Journal homepage: www.WORLDNEUROSURGERY.org 298
265 repair may survive to present for delayed Available online: www.sciencedirect.com 299
266 2. Beltranena FC, C, Polanco GH: Honor’s thesis: the 300
closure and often have large, disfiguring neural tube defect’s dilemma in Guatemala. Bull 1878-8750/$ - see front matter ª 2013 Elsevier Inc.
sacs. The surgical repair of these sacs Res Abstr 14, 2003. All rights reserved.

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