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SURGICAL OPTIONS FOR THE CLOSURE OF LARGE MYELOMENINGOCELES

Dov C. Goldenberg, MD'; Nivaldo Alonso, MD, PhD2; Marcus C. Ferreira, MD, PhD3 There are two options for the surgical treatment of myelomeningoceles: primary closure and flap repair. Since the latter technique is more complex and is used in larger lesions, the presence of a plastic surgeon may be important for an adequate evaluation and for the definition of which flaps should be used. This study aimed at defining the role of plastic surgery in the repair of large myelomeningoceles and at standardizing the use of specific surgical flaps (fasciocutaneous or musculocutaneous). From January 1999 to June 2000, 31 patients presentingmyelomeningoceles were selectedat Hospitalde Clinicas da Faculdade de Medicina da Universidade de Sao Paulo, Brazil. All cases were evaluated by the neurosurgery and plastic surgery teams for treatment planning. Flap repair was performed exclusively by the plastic surgery team in 20 patients (PS group); primary soft tissue closure was carried out by the neurosurgery team in I 1patients (NS group). In the PS group, two patients undewent only primary closure. Fasciocutaneous simple rhomboid flaps were used in three cases, double rhomboidflaps in 10 cases, and musculocutaneouslatissimus dorsi flaps in five cases. The mean area of defects was lower in the NS group than in the PS group. Incidence ofpostoperative hydrocephalus and need for ventriculoperifoneal shunt were higher in the PS group (p<0.05). In sum, this multidisciplinary approach allowedfor increasedsafety and quality of treatment and may potentially reduce complication and failure rates. KEY WORDS: Myelomeningoceles; congenital defects; surgery.

Congenital deformities of the neural tube affect approximately one in every 1,000 newborns. They can vary from simple skin birthmarks to large and complex defects. Myelomeningocelescorrespond to 85% of all neural tube defects and are the most common cases of neurological malformation referred to pediatric neurosurgeons (1-3). Nowadays it is possible to obtain a survival rate of 90% for patients with myelomeningoceles. Of these, 75% have normal intelligence, 80% achieve urinary and fecal continence when using proper medication, and 80% can walk with assistance (1,4,5). In view of these good results, the performance of a safe and stable surgical procedure aimed at repair is of paramount importance.

Assistant Physiclan. Division of Plastic Surgery and Burn. Hospital de Clinicas da Faculdade de Medicina da Universidade de SBa Paulo. Brazil. Member. Socledade Bras~lelrade Cirurga PBstica. Brarll. Associate Member, Socldade Brasileira de Clrurgia Craniomaxilofacial. Brarll. correspondence to: RUB pedro de T O I ~ ~9801124. 04039~002.SBO O Paulo. SP. Braril. E-maii: didov@terra.com.br. 2 Assistant Physician. Division of Plastic Surgery and Burn. Hospital de Clin>casda Faculdade de Medlcina da Unlveisidade de SBa Paulo, Brazil. Member. Sociedade Brasiieira de Cirurgia Plds~ica.Brazil. President. sacledade ~~~~~i~~~~ clrurgla de cranlomaxlofacai.B ~ ~ Z , I . 3 Professor. Plastic Surgery. School of Medicine. Un~versidade de SBa Paulo. BfazI. Head. Dlvislon of Plastic Surgery and Burn. Hosptta de Cin8cas da Faculdade de Medicina da Universldade de SBo Paulo. Bra281

In myelomeningoceles,the skin, the subcutaneous layer, the lumbodorsal fascia, and dural and neural tissues are affected. Small defects may be easily treated with primary closure of all layers, that is, medular reconstruction, dural closure, and repair of the lumbodorsalfascia,subcutaneous tissue, and skin. However, in more severe cases, soft tissue closure may require a complex reconstructive procedure. Inthe surgicalkeafmentof large myelomeningoceles, the presence of a plasticsurgeon may be importantforan adequate reconstruction. Depending on the patient's stature and the anatomic location and size of the lesion, complex techniques may be necessary. Unsuccessfulattempts of primary closure of large myelomeningoceles by neurosurgeons affect the surgical planning offlap repair and increasefailure rates. So, the most difficult aspect in the treatment of myelomeningoceles is to define limits for the use of either primary closure or flap repair. Several authors have tried to determine these limits, D~ chalain et al, (4) reported that defects larger than 25 cm2should be submitted to plastic surgical treatment using flaps. Lanigan (6) used the transverse diameter to determine the performance of either primary or flap closure. Another important aspect in the surgical management these lesions is the definition Of which flaps should
Braz J Craniomaxillofac Surg 2000;3(21 21

. Figure 2. and the donor area was primarily closed (figures4-6). sex of the newborn. surgery was performed exclusively by the plastic surgery team.lumbar. were used in cases of larger defects. all patients born at Hospital de Clinicas da Faculdadede Medicina da Universidade de SLo Paulo. In these cases. the purposes of the present study were: 1) to define the role of plastic surgery in the repair of large myelomeningoceles. . All cases were evaluated using a multidisciplinary approach. Brazil. and skin (figures 2 and 3). donor areas were grafted or left open with dressings for spontaneous secondary healing (figure 7). Soft tissue defects were primarily closed by either the neurosurgeon or the plastic surgeon. At 6 rhorncolumbar. and 2) to standardize the use of specific surgical flaps based on the defect dimensions. Fasciocutaneous flaps were designed as single or double rhomboidflaps (Limberg's flaps). €1 sacral. Surgicalflaps were divided into fasciocutaneous and musculocutaneous. local skin conditions. performed by theneurosurgeon orthe plasticsurgeon. all cases were evaluated by the neurosurgery and plastic surgery teams with the aim of planning treatment. 22 Braz J Craniomaxillofac Surg 2000.obtained by means of lateral incisions.f myelomeningocele. the second stage consisted of soft tissue closure.3121 . Figure I . Lesion location and size (area) were analyzed. were also recorded. and presenting myelomeningoceles in thoracic. The time interval between birth and surgery. by determining limits for either primary or flap closure. A total of 31 newborns presented the iesion (figure 1).andlor sacral regions were included in the study. Primary sofl tissue closure was done in three or four layers. In view of these aspects. Treatment was divided into two stages: the first stage consisted of dissection of the neural tissue and closure of the dura and the lumbodorsalfascia. Latissimus dorsi musculocutaneousflaps. and the occurrence of preoperative rupture was recorded. and newborn weight and height.and postoperative hydrocephalus and need for ventriculoperitoneal shunt were evaluated. MATERIALSAND METHODS From January 1999 to June 2000. Ct kmbar. they were closed in two layers (fascia and skin). When flaps were necessary. Dt 1 lumhosacral. including the dura. Incidence of pre. carried out by t& neurosurgeon. Prior to surgery. Primary closure <.Goldenberg et al be used. prenatal diagnosis and follow-up. as well as information regarding surgical options. Data collected included mother's age. Anatomk lorarion of rnyelornen~r~goceles: Ihorac~c. subcutaneous tissue. based on the size of defects and other quantitativeparameters. and birthweight. lurnbodorsal fascia. Surgical decision was based on the area and location of the defect.

Single fasciocutaneous rhomboid flap Rimberg's flap). Primary closure of myelomeningocele.Surgical treatment of myelomeningoceles rtgure 3. Braz J Craniomaxillofac Surg 2000. Figure 4.3/21 23 .

Single fasciocutaneous rhomboid flap (Limberg's flapl. Double fasciocutaneous rhomboid flaps (Limberg's flapl.Goldenberg et al. Figure 5. Bilateral latissimus dorsi musculocutaneous flap obtained b y means of lareral incisions. 24 Braz J Craniomaxillofac Surg 2000.3/21 . Figure 6. Figure 7. without skin grafts. The arrow indicates late postoperative secondary heaihg of lateral incisions.

and patients operated by the neurosurgery and the plastic surgery teams (PS group). grams) Mother's age (mean. Epidemiological data and surgical results were analyzed comparing patients operated exclusively by the neurosurgery team (NS group). Lesion location and occurrence of rupture were not statistically different.4 days). In 20 patients. Complications in the PS and the NS groups can be observed in table 3. with flap closure of soft tissue defects. Clinical data in the PS and NS groups Plasticsuraerv Number of patients Sex Male Female Birthweight(mean.3/21 25 Neurosuraerv Total . Fisher's exact test and Wilcoxon's test. undergoing primary closure of soft tissue defects.05). Complications included dehiscence. infection. group). Comparison between the two groups showed no differences in terms of sex and birthweight.05). In the remaining 11 patients. The mean area of defects in the NS group was 25. surgery was performed in the first 72 hours of life. and need for reoperation. RESULTS Thirty-one patients presenting rnyelorneningoceles at birth were surgically treated between January 1999 and June 2000. days of life) Occurrenceof preoperative rupture Surgical procedure Primary closure Use of single rhomboid flap Use of double rhomboid flap Use of latissimus dorsi flap Braz J Craniomaxillofac Surg 2000. soft tissue closure was performed by the plastic surgery team (PS Table 1. In 40% of cases. Mothers in the PS group were younger than those in the NS group (p<0.11 cm2.Surgical treatment o f myelomeningoceles Cases were followed after surgery and observed in terms of surgical results. Results for the PS group and the NS group are shown in table 1. primary closure was performed by neurosurgeons (NS group) after plastic surgery evaluation. Statistical analysis was performed using Student's t test.which is statistically lower than the mean area observed in the PS group (p<0. When mean area of the defects was compared to flap options (table 2). that is. complications and need for ventriculoperitoneal shunt. Surgical procedure was performed between 18 hours and 10 days after birth (mean = 4. cerebrospinal fluid (CSF) leak. no statistically significant difference was observed. years) Prenataldiagnosis Hydrocephalus Dimensionof the defect (cm2) Minimum Maximum Mean Defect location Lumbar Sacral Thorawlumbar Lumbosacral Time elapsed until surgery (mean.

the neurosurgeon closes any type of lesion. lncidence is variable.Goldenberg et al. being likely to face several complications in more complex cases. The location and dimension of the defect in relation to the patient's stature are of paramount importance for treatment decision. Minor dehiscence was more frequent in the PS group (p<0.2 72. Single rhomboid fasciocutaneous flap Number of patients Size of the defect (cm2) Minimum Maximum Mean 3 20.mainly due to the risk for rupture. and social care professionals.2 Table 3.9 Latissimus dorsi musculocutaneousflap 5 50. DISCUSSION Myelorneningoceles (or spina bifida) is the most common malformation of the central nervous system seen by pediatric neurosurgeons.05). (9) recommend surgery during the first 48 hours of life. They require a stable sofl tissue coverage of the exposed neural tissue (73) in order to provide a better quality of life. According to the classical approach. In addition. pediatricians. The choice of the closure technique by plastic surgeons potentially decreases complication rates or at least the incidence of unsuccessful attempts at primary repair. On the other hand.0 38. Patients in the PS group had lesions predominantly in the thoracolurnbar and lumbosacral regions.0 70. Table 2. including neurosurgeons. Therefore. simultaneous conditions may delay the procedure. attempts to use flap repair afler several unsuccessful experiences of primary repair with massivedissection of tissues increasesflap loss and dehiscence. Plastic surgery Number of patients Needfor ventriculoperitonealshunt Complication Minor dehiscence with no need for reoperation Major dehiscence with need for reoperation Surgical infection Cerebrospinal fluid leak 20 20 (100%) 7 (35%) 1 (5%) I(5%) 3 (15%) Neurosurge~y 11 6 (55%) 2 (18%) 1 (9%) 1 (9%) - Total 31 26 (84%) 9 (29%) 2 (6%) 2 (6%) 3 (9%) lncidence of postoperative hydrocephalus and need for ventriculoperitonealshunt were higher in the PS group (p<0. 40% of the patients in the NS group presented sacral lesions. . geneticists.4 Double rhomboid fasciocutaneous flap 10 20. If all locations are considered. but no differences were observed in infection or reo~eration rates. the participation of plastic surgery professionals in the treatment of myelomeningoceles is highly justified.0 59. sacral lesions are more easily treated.neurologists.2 32. plasticsurgeons. urologists. Complications in the PS and NS groups. orthopedists. Seidel et al.and was adopted in this study.3/2) Early surgical treatment is recommended in the literature. Defect areas in the PS group. However. Sacral lesions occurred in only 15%. Treatment of myelorneningoceles requires a multidisciplinary approach. from 1:200 to 1:1000 newborns. The impact of unsuccessful repair on morbidity and mortality is high.0 50. 26 Braz J Craniomaxi/lofac Surg 2000. Improvement in prenatal diagnosis and neonatal intensive care have increased survival rates for patients with multiple congenital malformations.05). Patientswith myelomeningoceles are included in this group.

In 41 patients. lQ in hydrocephalus and m y e l o m e n i n g o c e l e Implication o f surgical treatment.5711):44-50. rather than as a routine surgical option. In ourstudy. 1990. In the present study.B.3/21 27 . primary closure was indicated only when tension-free closure was possible prior to sofl tissue undermining. We observed an increase in the number of minor dehiscence in the PS group when compared to the NS group. Spina bifida. fasciocutaneous flaps were used in 65%. Cohen M . In our study.9 cm2in defects receiving fasciocutaneous flaps. The first flap was elevated. but cases requiring more complex flaps presented higher defect areas.2 cm2. De Chalain (4) found a rate of 24. In: McCarthy. Da Silva PP. 39(3):522-6. Saunders. Rizzo AML.Surgical treatment of myelomeningoceles The rate of involvement of plastic surgery teams In the treatment of myelomeningoceles is variable in the literature. Silva IM. the defects in which this type of flap was used had a mean area of 59.against 38. (10) reinforced the use of fasciocutaneous flaps in myelomeningocele closure. the higher the chance for hydrocephalus in the postoperative period. 4. this type of flap was considered as the last option for the treatment of large defects. Plastic surgery. Buchman SR. Rhomboid flaps seem to be the most adequate ones. The absence of statistical significance may be explained by the different relationships between defect area and patient stature. with no distress about soft tissue coverage. Philadelphia: W. All patients in the PS group had hydrocephalus prior to surgery or developed hydrocephalus after closure and the need for ventriculoperitonealshunt. and therefore a higher rate of complications was expected in this group. Zide BM. a rate of 64. REFERENCES 1. Complication rates in this study were similar to those reported by other authors (4. No statistically significant difference was observed between defect areas and flap options.9). but major complications were similar. Boydston WR. Problems directly related to treatment as well as secondary infectionsare the leading causes of mortality.8% in 65 patients. Flap design is currently being discussed in the literature. Braz J Craniomaxillofac Surg 2000. a second previously designed flap was elevated. This aspectwas statistically different in the NS group. Ann Plast Surg 1995. (9). In: Bentz ML. The literature suggests the use of musculocutaneous flaps in cases of larger defects (14). The final result was not significantly altered by this parameter. O'Brien MS. Fasciocutaneous flaps have been shown to present some advantages when compared to musculocutaneous flaps. Murazko KM. In the present study.3513):272-8. Teixeira CE. the rate was of 25. Bilateral latissimus dorsi flaps are associated with a high morbidity rate when compared to fasciocutaneous flaps. which is more familiar to this type of problem. since fasciocutaneous vessels and musculocutaneous perforators can be preserved in fasciocutaneousflaps. need for skin grafting in some cases and functional impairment are common problems associated with latissimus dorsi flaps. Increased blood loss. Measurement of the defect size helps surgical planning and flap choice. Stamford: Appleton & Lange. 2. 3. The multidisciplinary approach allows for a good-quality treatment in terms of safety and quality of soft tissue coverage. Radhaksihnan J. et al. One of these advantages refers to the distal blood supply. Fobe JL. This may potentially reduce complication and failure rates. Pediatric plastic surgery. It is important to note that cases in the PS group were more complex. Ramasastry SS. Soft tissue coverage in complex defects is a responsibility to the plastic surgery team. Fiala TGS.4% of cases referred to plastic surgery for the closure of soft tissue defects. Arq Neuropsiquiatr 1999. Ohtsuka et al. options for the use of single or double flaps were defined during the procedure. In our series. Congenital back defects. Cohen SR. p.The more severe the myelomeningocele. 757-82. De Chalain TMB. This index surpasses expectations and illustrates the effectiveness of the multidisciplinary approach. Hudgins RJ.5% of plasticsurgical treatment of soft tissue defects was observed. In the study of Seidel et al. Decision making in primary surgical repair of myelomeningoceles. Hydrocephalus seems to be the most important prognostic factor in myelomeningoceles. p. and if it was not enough for tension-free closure. 1998. In our cases requiring flap repair. It reduces the incidence of unsuccessful attempts at surgical closure and allows the neurosurgeon to correct the dural defect with more safety. Use of lumbar periosteal turnover flaps in myelomeningocele closure. Neurosurgery 1996. 5. Burstein FD. De Souza AM. since they present a good width-tolength ratio and sutures are not located exactly over the dural sutures (11-13). 3780-96.

Fantino AG. Modified Limberg flap for lurnbosacral meningornyelocele. Ann Plast Surg 1996. Modified bilateral advancement flap: the slide in flap. Zide BM.Back closure with a latissimus dorsi musculocutaneous flap. Yada K. Childs Nerv Syst 1998: 14(3):120-3. 7. Outcome of patients with rnyelorneningocele: the Ege University experience. 10. Kapucu MR. Orthop Clin North Am 1993. Dudkiewica 2 . Ohtsuka H. Ulusoy MG. 37(3):310-6. Soff-tissue coverage of the neural elements after rnyelomeningocele repair. 8. Technical note. 14. Kayahan A.3(21 . 28 Brar J Craniomaxillofac Surg 2000.Goldenberg et al. 13. Coverage of the chest wall and spine. J Neurosurg 1991. Optimal wound closure after tethered cord correction. Shioya N. Lodzinski K.42(5):545-8. Grotting JC. 6. Gardner PM. Ann Plast Surg 1995:31(6):514-21. Seidel SB.8 Suppl 1: 52-4. Stephens DR. Surgical repair of myelomeningocele. 9.24(3):449-60. 11. Lanigan MW. Casari EF. Epastein FJ. J a w o r s k i S. A longitudinal study of cognitive abilities and achievement status of children with myelomeningocele and their relationship with clinical types. Mutluer S. Akan IM. J Pediatr Surg 1992. 12.74(4):673-6. Lenkiewicz T. Mirzai H. Ann Plast Surg 1999.3:114. S o f t tissue reconstruction. RsahinY. Bilen BT. Ann Plast Surg 1979. 27(1):74-5. Eur J Pediatr Surg 1998. Wisoff J. Howard PS.