J Nenrosurg 72:726-73 l, 1990
Experience in the management of myelomeningocele in Puerto Rico RICARDO H . BRAU, M.D., RAFAEL RODRiGUEZ, M.D., MAYRA VERA RAMiREZ, M.D., M.P.H., ROSARIO GONZALEZ, M.D., AND VIRGINIA MARTiNEZ, M . P . H .
Departments o f Neurosurgery, Pediatrics, and Biostatistics, University of Puerto Rico School of Medicine, San Juan, Puerto Rico
~" The medical records of 128 children with myelomeningocele who were treated at the Pediatric University Hospital, Puerto Rico Medical Center, from January, 1980, to July, 1985, were reviewed retrospectively. The medical and surgical management during the first hospitalization of these children was studied in detail for predefined parameters. The average age at the time of myelomeningocele repair was 6.6 days. Statistical analysis showed that repair of the myelomeningocele defect before 48 hours of age did not reduce the occurrence of ventriculitis. The incidence of ventriculitis secondary to the management of the myelomeningocele lesion was 12.5%. Complications after repair of the myelomeningocele (including skin flap necrosis, cerebrospinal fluid leaks, and wound infection) were present in 43.8% of the patients who developed ventriculitis and in 19.0% of those who did not. This observation is statistically significant (p = 0.03) and indicates that complications of healing after myelomeningocele repair represent the most significant risk factor for the development of ventriculitis. KEy WORDS
M
9
myelomeningocele
ANAGEMENT o f the newborn with myelomeningocele has stirred great controversy, 1'3~4'6' 9-12,14,15,20,23-25,27-29,35,39,40 the most critical issue being the identification o f which patients will benefit from treatment. This controversy involves medical, legal, ethical, moral, economic, social, and humanitarian issues. Our policy has been to operate in all cases. Having adopted this policy, we could concentrate on dealing with the neurosurgical problems of these children. Some of the questions that arise are: what is the optimal timing for myelomeningocele closure? which is the best technique? what is the best treatment of ventriculitis? what is the ideal t r e a t m e n t of hydrocephalus? and, finally, when is it best to institute cerebrospinal fluid (CSF) shunting? Some published studies have discussed the incidence of infection in newborns with myelomeningocele 28'37 and the associated risk factors. Although m a n y reports have been published assessing the long-term prognosis of children afflicted with myelomeningocele, l, l l, 16,21,27.35, 36,38there are few publications that comprehensively analyze the parameters 6'18'37 a n d the immediate outcome in these patients. For this reason, we decided to review our experience in the m a n a g e m e n t of newborns with myelomeningocele during the initial hospitalization. 726
9 ventriculitis
9 spina bifida
9 neonate
Summary of Cases
The medical records of all children with myelomeningocele treated at the Pediatric University Hospital, Puerto Rico Medical Center, between January, 1980, and July, 1985, were studied retrospectively. Almost all of the children born with myelomeningocele on the island of Puerto Rico and the United States Virgin Islands are referred to this institution for medical and surgical management. The hospital records o f 128 patients were identified. These records were reviewed and analyzed for the following parameters: sex, birth weight, location of the lesion, size of the myelomeningocele, presence of CSF leakage from the lesion, head size at birth, head size at the time of CSF shunting, presence of hydrocephalus, timing of closure of the myelomeningocele defect, duration of the operation for the closure of a spinal bifida cystica defect, the use o f prophylactic antibiotics prior to the myelomeningocele repair, complications in the healing of the myelomeningocele repair, presence of ventriculitis before or after the shunting procedure, the organism producing ventriculitis, type of shunting procedure used, timing of the shunting procedure, duration of the shunting procedure, use of prophylactic antibiJ. Neurosurg. / Volume 72/May, 1990
Management of myelomeningocele in Puerto Rico otics at the t i m e o f t h e s h u n t i n g procedure, incidence o f ventriculitis after s h u n t i n g , o r g a n i s m s p r o d u c i n g the infection, m o r t a l i t y , a n d t h e total hospitalization time. These children were m a n a g e d following a s t a n d a r d t r e a t m e n t protocol. M o s t p a t i e n t s were b o r n elsewhere a n d transferred to t h e P e d i a t r i c University H o s p i t a l after they were stable e n o u g h for t r a n s p o r t a t i o n . T h e y were a d m i t t e d to the p e d i a t r i c n e w b o r n service w a r d or the intensive care u n i t d e p e n d i n g o n the general condition o f the infant. T h e y were k e p t in a p r o n e p o s i t i o n a n d the m y e l o m e n i n g o c e l e defect was covered with s a l i n e - m o i s t e n e d gauze. P r o p h y l a c t i c antibiotics were not used while the c h i l d was being stabilized or waiting for surgery b u t were u s e d liberally if i n d i c a t e d for o t h e r conditions. P r o p h y l a c t i c a n t i b i o t i c s were a d m i n i s t e r e d prior to surgery in t h e o p e r a t i n g r o o m according to the surgeon's preference. T h e m y e l o m e n i n g o c e l e was closed urgently (as s o o n as possible) b u t n o t on an e m e r g e n c y basis. M y e l o m e n i n g o c e l e r e p a i r was carried out using s t a n d a r d t e c h n i q u e s j 3~19-21.32.34.42t a i l o r e d to the individual case. H e a d g r o w t h was carefully m o n i t o r e d a n d a diagnosis o f h y d r o c e p h a l u s was c o n f i r m e d with a h e a d c o m p u t e r i z e d t o m o g r a p h y (CT) scan. A shunt system for h y d r o c e p h a l u s c o n t r o l was p l a c e d after the child was assessed to be w i t h o u t active m e d i c a l p r o b l e m s , usually after the m y e l o m e n i n g o c e l e repair was n o t e d to be healing properly. Statistical analysis o f the d a t a was p e r f o r m e d with analysis o f v a r i a n c e u s i n g a statistical c o m p u t e r software package for the social sciences.* A p r o b a b i l i t y o f p < 0.05 was c o n s i d e r e d significant. Results There were 62 m a l e s (48.4%) a n d 66 females (51.6%) in this series (Table 1). T h e average birth weight was 6.6 lbs (range 2.3 to 10.4 lbs). T h e defect was located in the t h o r a c i c a r e a in t h r e e cases (2.3%), in the t h o r a c o l u m b a r area in 41 cases (32.0%), in the l u m b a r a r e a in 28 (21.9%), a n d in the l u m b o s a c r a l area in 56 (43.8%). T h e size o f t h e m y e l o m e n i n g o c e l e lesion was recorded in 50 cases: t h e average area was 29.9 sq c m (range 3.0 to 100.0 sq cm). A C S F leak after birth was described in 12 cases (9.4%). T h e m y e l o m e n i n g o c e l e defect was r e p a i r e d in 126 p a t i e n t s (98.4%) at an average age o f 6.6 d a y s (range < 24 h o u r s to 52.3 days). T h e average d u r a t i o n o f t h e m y e l o m e n i n g o c e l e repair, i n c l u d i n g the anesthesia, i n t u b a t i o n , a n d e x t u b a t i o n time, was 3.4 h o u r s a n d p r o p h y l a c t i c antibiotics at surgery were used in 73 cases (57.0%). Back w o u n d healing c o m p l i c a t i o n s , i n c l u d i n g skin necrosis, C S F leak, a n d w o u n d i n f e c t i o n , o c c u r r e d in 26 cases (20.3%). Ventriculitis p r i o r to s h u n t p l a c e m e n t develo p e d in 16 cases (12.5 %). T h e m o s t c o m m o n organisms in o r d e r o f f r e q u e n c y were Klebsiella, Acinetobacter,
* Statistical package SPSSx obtained from SSPS, Inc., Chicago, Illinois; IBM 4361-3 system obtained from International Business Machines, Boca Raton, Florida.
J. Neurosurg. / Volume 7 2 / M a y , 1990
TABLE 1
Myelomeningocele assessed parameters in 128 patients* Parameter Value sex (M:F) 62 (48.4%): 66 (51.6%) average birth weight 6.6• 1.41bs location of lesion thoracic 3 cases (2.3%) thoracolumbar 41 cases (32.0%) lumbar 28 cases (21.9%) lumbosacral 56 cases (43.8%) average size of lesion (50 cases) 29.9 sq cm CSF leak before repair 12 cases (9.4%) average age at time of repair 6.6 • 7.3 days duration of repair surgery 3.4• 1.1 hrs prophylactic antibiotics at repair 73 cases (57.0%) complications of repair 26 cases (20.3%) nonshunt-related ventrieulitis 16 cases (12.5%) average hospital stay 42.2 • 26.3 days mortality 0 (O%) * Mean values are given • standard deviation. CSF = cerebrospinal fluid.
TABLE 2
Parameters assessed in 97 patients with hydrocephalus* Parameter Value shunt placement 97 cases head circumference at birth 36.1 - 4.9 cm head circumference at shunt placement 39.4 • 4.5 cm average age at shunt placement 30.4 • 17.7 days prophylactic antibiotics given at shunt placement 68 cases (70.1%) duration of shunting procedure 2.3 • 0.8 hrs shunt-related ventriculitis 5 cases (5.2%) * Mean values are expressed _+ standard deviation.
Escherichia coli, P s e u d o m o n a s , Serratia, Staphylococcus epidermidis, Enterococcus, a n d Enterobacter species. T h e average l e n g t h o f h o s p i t a l i z a t i o n t i m e for the 128 p a t i e n t s was 42.2 d a y s (range 4 to 170 days). T h e r e were n o d e a t h s d u r i n g t h e i n i t i a l h o s p i t a l i z a t i o n in this series. H y d r o c e p h a l u s was d i a g n o s e d d u r i n g the initial hosp i t a l i z a t i o n in 97 cases (75.8%), a n d all were t r e a t e d with the i n s e r t i o n o f t h e v e n t r i c u l o p e r i t o n e a l s h u n t systems. A s u m m a r y o f f i n d i n g s in t h e h y d r o c e p h a l i c p a t i e n t s is given in T a b l e 2. T h e a v e r a g e age at s h u n t p l a c e m e n t was 30.4 d a y s . T h e a v e r a g e h e a d c i r c u m f e r ence at b i r t h was 36.1 c m a n d at t h e t i m e o f s h u n t p l a c e m e n t was 39.4 c m . T h e a v e r a g e t i m e for the s h u n t ing p r o c e d u r e , i n c l u d i n g i n t u b a t i o n a n d e x t u b a t i o n t i m e , was 2.3 h o u r s . P r o p h y l a c t i c a n t i b i o t i c s were u s e d in 68 cases (70.1%) at t h e t i m e o f s h u n t surgery. V e n triculitis was d i a g n o s e d in five p a t i e n t s (5.2%) after s h u n t insertion. T h e o r g a n i s m s i d e n t i f i e d as t h e c a u s e o f ventriculitis after s h u n t p l a c e m e n t were Staphylo-
coccus epidermidis, Streptococcus, Pseudomonas, Alcaligenes faecalis, a n d Citrobacter.
72-/
R. H. Brau, et al. TABLE 3
Timing of myelomeningocele repair
Age at Repair (days) 0-2 3-6 6-12 > 12
Nonshunt-Related Ventriculitis ( 16 cases) 31.3% 37.5% 6.3% 25.0%
No Ventriculitis ( 110 cases) 26.0% 44.0% 16.0% 14.0%
Discussion
The incidence o f myelomeningocele in the United States is 1 to 2 per 1000 live births. 19'32 A significant variation in this incidence has been reported for different ethnic groups; however, this has been estimated to be the incidence of myelomeningocele in the Puerto Rican population (unpublished data). The defect in embryogenesis that leads to the formation of the myelomeningocele is usually thought to occur during the 4th week o f gestation. 2"~3 The mechanism that triggers the derangement o f the complex synchronized steps of spinal cord f o r m a t i o n is not known. In 1982, M c L o n e and associates 28 published a study in which they implicated central nervous system (CNS) infection as the m a j o r factor limiting the intelligence of children afflicted with myelomeningocele. They found that ventriculitis reduced the intelligence quotient (IQ) by 25% (average IQ o f 72 in the ventriculitis group vs. an average IQ of 95 in the group that had never been so infected). In a m o r e recent article, McLone 2v stated that almost 80% o f children with myelomeningocele should have n o r m a l intelligence. Convinced that a n o r m a l intelligence is critical to assure an independent and productive life for patients with myelomeningocele, we elected to examine in detail the different p a r a m e t e r s between patients who developed ventriculitis a n d those who did not. Ventriculitis was diagnosed at different stages during the care of these patients and was identified either before myelomeningocele repair, after repair but before shunt insertion, or after shunt placement. The association of ventriculitis with shunt insertion has been studied extensively; 5"8"j3"~9"22"2s3~ however, the connection between ventriculitis and myelomeningocele repair itself has not received m u c h attention. 6"j3374~ Therefore, this study e x a m i n e d in detail all cases in which ventriculitis developed. Special emphasis was given to those in which the infectious process was not directly associated to the shunting procedure itself. The group of patients who developed ventriculitis before shunt placem e n t will be t e r m e d the "nonshunt-related ventriculitis group." Ventriculitis I n c i d e n c e
The incidence o f ventriculitis before shunt placement in this study was 12.5% (16 cases); the incidence re728
ported in the literature varies from 10% to 2 5 % . 6"13"37"40 The organisms found in these patients were usually gastrointestinal tract or skin colonizers. Five patients were infected with m o r e than one organism. The organisms isolated from the ventricular fluid in our patients followed a similar pattern to those found in the series reported by Charney, et al. 6 Timing o f Surgery One of the most controversial management variables is the optimal timing of myelomeningocele repair. The myelomeningocele should be closed within 48 hours of birth according to some authors, j~ Other reports indicate that closure can be performed after 48 hours without detrimental effects to the child. 1.6.JJ.14.37.4o In this series, the average age at the time of the myelomeningocele repair was 6.6 days. In two patients, myelomeningocele repair was not performed during the initial hospitalization; however, all patients with documented ventriculitis underwent closure of the defect during the initial hospitalization. The average age at the time of repair of the 16 patients who developed ventriculitis was 9.7 days, and the average age of those who did not develop infection was 6.2 days (Table 3). This correlation is not statistically significant (p = 0.07). Of the 16 children who developed ventriculitis, five (31.3%) were operated on within 48 hours of birth, six (37.5%) were operated on between 3 and 6 days of age, and the remaining five patients underwent repair at age 12 days (one case), 16 days (one case), 20 days (two cases), and 52 days (one case). In the group of 110 children who did not develop CNS infection, 26% underwent myelomeningocele repair within 48 hours, 44% between 3 and 6 days, 16% between 6 and 12 days, and 14% after 12 days (the two patients without surgery were excluded). O f the 16 cases with nonshunt-related ventriculitis, three were diagnosed as having ventriculitis before the myelomeningocele was repaired and 13 after surgery. In the three patients in w h o m surgery was performed after the diagnosis of ventriculitis, the repair was carried out after the CNS infection was totally eradicated. Two of these underwent surgery at the age of 20 days and the third at 52 days. If these three cases are eliminated, the average age at surgery for the group that developed CNS infection was 4.7 days. In this series, the recommended period of 48 hours after birth as the optimal timing for myelomeningocele defect closure, as mentioned by some reports, ~~ was not found to produce a significant difference in the overall incidence of ventriculitis. Size o f M y e l o m e n i n g o c e l e The size of the myelomeningocele was compared between both groups. The average size of the defect was recorded in 50 cases, nine (56.3%) of the 16 cases with nonshunt-related ventriculitis and 41 (37.2%) o f the 110 cases without. The average size of the lesion was 29.9 sq cm for the total group, 32.3 sq cm for the J. Neurosurg. / Volume 72/May, 1990
Management of myelomeningocele in Puerto Rico ventriculitis group, and 28.8 sq cm for the group without infection (Tables 1 and 4). There was no statistical difference between the groups (p = 0.58). Cerebrospinal F l u i d L e a k In a recent report, Hubballah and Hoffman ~8pointed out that children who are born with overt hydrocephalus associated with a myelomeningocele are at higher risk of CSF leakage and CNS infection than other myelomeningocele patients. We compared the head circumferences at birth in our target groups. The average head circumference at birth for the entire population was 36.1 cm, for the group with the nonshuntrelated CNS infection it was 35.0 cm, and for the group without ventriculitis it was 36.2 cm. There was no statistical correlation between the groups (p = 0.36). Therefore, overt hydrocephalus was not a risk factor in the development of ventriculitis in this series. Active leaking of CSF through the myelomeningocele defect was documented in 12 cases (10.2%): three patients (18.8%) developed nonshunt-related ventriculitis and nine (8.0%) did not develop ventriculitis (no statistical correlation; p = 0.40). This finding is interesting because it does not agree with previous r e p o r t s . 9"32'33 We have no explanation for this finding. Surgical Variables Surgical complication rates may be associated with different surgeons, the duration of the procedure, or other documented parameters. 4~ With this in mind, we investigated whether the surgeon or the time required to perform the surgery contributed to the incidence of ventriculitis in this series. In our institution, most myelomeningocele closures are performed by the senior resident staff. There was no correlation between the incidence of CNS infection and the surgeon performing the procedure (p = 0.29). There was no statistical difference between the groups in the overall time in the operating room for myelomeningocele repair (p = 0.17). The use of systemic prophylactic antibiotics prior to myelomeningocele closure did not lower the incidence of nonshunt-related ventriculitis (p = 0.16). Also, low birth weight was found not to increase the incidence of ventriculitis or surgical complications (Table 4). Wound-Healing P r o b l e m s Information regarding wound-healing complications after myelomeningocele repair was recorded in 116 of the 126 surgical cases. Data were available for all patients who developed nonshunt-related ventriculitis. Back wound-healing problems were defined as skin necrosis, CSF leakage, and gross wound infection. There were 26 cases (22.4%) with these problems and 90 cases (77.6 %) with no healing complications after myelomeningocele repair. In the nonshunt-related ventriculitis group, seven patients (43.8%) developed back woundhealing problems, while only 21 (19.0%) of the patients who did not develop ventriculitis had myelomeningoJ. Neurosurg. / Volume 7 2 / M a y , 1990
TABLE 4 Nonshunt-related ventriculitis group versus group without ventriculitis NonshuntRelated Ventriculitis no. of cases 16 birth weight 6.64 lbs average size of defect (50 cases) 32.3sq cm head circumference at birth 35.0 cm CSF leak before repair 18.8% duration of repair 3.2 hrs prophylactic antibiotics 81.3% wound-healing problems 43.8% * CSF = cerebrospinal fluid. I"Statistically significant value. Parameter*
No Ventri- P culitis Value 1l0 6.57 lbs 28.8 sq cm 36.2 cm 8.0% 2.9 hrs 59.4% 19.0%
0.89 0.58 0.36 0.40 0.17 0.16 0.03t
cele repair healing problems (statistically significant; p = 0.03). In the group with nonshunt-related ventriculitis, three patients developed skin necrosis, one a CSF leak, and three gross skin infection. Ventriculitis Versus W o u n d H e a l i n g The relationship between healing problems of the myelomeningocele repair and ventriculitis have been recorded sporadically in the literature. In 1973, Foltz, et al., j~ reported a 14% incidence of myelomeningocele repair breakdown and stated that this incidence could be reduced to between 3% and 5% if hydrocephalic patients received shunts before myelomeningocele repair. Guthkelch and associates ~6 reported a 7% incidence of CSF leaks after myelomeningocele repair with fasciomuscular flaps and aggressive use of ventriculostomies. Lehman and collaborators, z~ conscious about the importance of wound necrosis and dehiscence, suggested using nitroglycerin ointment to enhance cutaneous blood flow in myelomeningocele closure. Other authors have reported different techniques using skin flaps with the purpose of avoiding tension at the skin edges and skin necrosis. 7"~7 Our study strongly associated the incidence of ventriculitis with healing complications of the myelomeningocele closure. We are not aware of any other study that correlated these two variables so definitely. Different repair techniques that might minimize these complications should be considered. Different techniques for myelomeningocele repair have been reported, 7"13"17"2~ and a controlled trial between them may provide useful information about which method is best. Hydrocephalus There were 97 patients (75.6%) diagnosed as having hydrocephalus during their first hospitalization. All were treated with the insertion o f a ventriculoperitoneal shunt. The incidence o f ventriculitis after shunt insertion in this series was 5.2% or five of the 97 cases (Table 2). The complication of ventriculitis after shunt insertion and the different parameters and variables 729
R. H. Brau, et al. that i n f l u e n c e it h a v e b e e n discussed p r e v i o u s l y ? "8"t3" 19,22,28,30,32,33,36,39 A l t h o u g h i n - d e p t h analysis o f our five cases w o u l d n o t p r o d u c e a n y c o n c l u s i v e observations, a survey o f t h e p a r a m e t e r s s t u d i e d b e t w e e n t h e group with s h u n t - r e l a t e d ventriculitis a n d the r e m a i n i n g p a tients failed t o s h o w a statistical r e l a t i o n s h i p for: birth weight, h e a d size at the t i m e o f s h u n t i n s e r t i o n , age at s h u n t p l a c e m e n t , d u r a t i o n o f s h u n t surgery, a n d the use o f p r o p h y l a c t i c antibiotics.
Mortality A few p u b l i c a t i o n s have e x a m i n e d the survival o f the n e w b o r n w i t h m y e l o m e n i n g o c e l e . T h e g r o u p f r o m the C h i l d r e n ' s H o s p i t a l at P h i l a d e l p h i a r e p o r t e d no m o r tality b y 10 m o n t h s o f age in 52 p a t i e n t s with early spina b i f i d a cystica repair, a n d a 6% m o r t a l i t y rate in the g r o u p w i t h late surgery. 4~ G r o s s a n d associates ~4 r e p o r t e d 100% survival in t h e i r g r o u p t h a t received active m e d i c a l t h e r a p y (one p a t i e n t d i e d at age 14 m o n t h s in a m o t o r - v e h i c l e accident). T h e r e were no d e a t h s in o u r series.
Acknowledgments The authors are indebted to Dr. Nathan Rifkinson and Dr. Humberto Ortiz for editorial advice. References 1. Ames MD, Schut L: Results of treatment of 171 consecutive myelomeningoceles - - 1963 to 1968. Pediatrics 50: 466-470, 1972 2. Angevine JB: Clinical relevant embryology of the vertebral column and spinal cord. Clin Neurosurg 20:95-113, 1973 3. Barden GA, Meyer LC, Stelling FH: Myelodysplasia - fate of those followed for twenty years or more. J Bone Joint Surg (Am) 57:643-647, 1975 4. Black PM: Selective treatment of infants with myelomeningocele. Neurosurgery 5:334-338, 1979 5. Borges LF: Cerebrospinal fluid shunts interfere with host defenses. Neurosurgery 10:55-59, 1982 6. Charney EB, Weller SC, Sutton LN, et al: Management of the newborn with myelomeningocele: time decisionmaking process. Pediatrics 75:58-64, 1985 7. Cruz NI, Ariyan S, Duncan CC, et al: Repair of lumbosacral myelomeningoceles with double Z-rhomboid flaps. Technical note. J Neurosurg 59:714-717, 1983 8. Djindjian M, Fevrier M J, Otterbein G, et al: Oxaciltin prophylaxis in cerebrospinal fluid shunt procedures: results of a randomized open study in 60 hydrocephalic patients. Surg Neurol 25:178-180, 1987 9. Epstein F: Myelomeningocele - - "pitfalls" in early and late management. Clin Neurosurg 30:366-384, 1983 10. Foltz E: Myelomeningocele: selection for treatment, in O'Brien MS (ed): Pediatric Neurological Surgery. Seminars in Neurological Surgery. New York: Raven Press, 1978, pp 105-124 11. Foltz EL, Kronmal R, Shurtleff DB: To treat or not to treat: a neurosurgeon's perspective of myelomeningocele. Clin Neurosurg 20:147-163, 1973 12. Freeman JM: To treat or not to treat: ethical dilemmas of treating the infant with a myelomeningocele. Clin
730
Neurosurg 20:134-146, 1973 13. French BN: Midline fusion defects and defects of formation, in Youmans JR (ed): Neurological Surgery, ed 2. Philadelphia: WB Saunders, 1982, Vol 3, pp 1236-1380 14. Gross RH, Cox A, Tatyrek R, et al: Early management and decision making for the treatment of myelomeningocele. Pediatrics 72:450-458, 1983 15. Guthkelch AN: The indications and contraindications for early operation in myelomeningocele, in Morley TP (ed): Current Controversies in Neurosurgery. Philadelphia: WB Saunders, 1976, pp 147-154 16. Guthkelch AN, Pang D, Vries JK: Influence of closure technique on results in myelomeningocele. Childs Brain 8:350-355, 1981 17. Habal MB, Vries JK: Tension free closure of large myelomeningocele defects. Surg Neurol 8:177-180, 1977 18. Hubballah MY, Hoffman H J: Early repair of myelomeningocele and simultaneous insertion of ventriculoperitoneal shunt: technique and results. Neurosurgery 20: 21-23, 1987 19. Humphreys RP: Spinal dysraphism, in Wilkins RH, Rengachary SS (eds): Neurosurgery. New York: McGrawHill, 1985, Vol 3, pp 2041-2052 20. Lehman RAW, Page RB, Saggers GC, et al: Technical note: the use of nitroglycerin ointment after precarious neurosurgical wound closure. Neurosurgery 16:701-702, 1985 21. Lindseth RE, Stelzer L: Vertebral excision for kyphosis in children with myelomeningocele. J Bone Joint Snrg (Am) 61:699-704, t979 22. Liptak GS, Masiulis BS, McDonald JV: Ventricular shunt survival in children with neural tube defects. Acta Neuroehir 74:113-117, 1985 23. Lorber J: Results of treatment of myelomeningocete: an analysis of 524 unselected cases, with special reference to possible selection for treatment. Dev Med Child Neurol 13:279-303, 1971 24. Lorber J: Spina bifida cystica. Arch Dis Child 47: 854-873, 1972 25. MacKeith RC: New look at spina bifida aperta. Dev Med Child Neurol 13:277-278, 1971 (Editorial) 26. McLone DG: Results of treatment of children born with a myelomeningocele. Clin Neurosurg 30:407-412, 1983 27. McLone DG: Treatment of myelomeningocele: arguments against selection. Clin Neurosurg 33:359-370, 1986 28. McLone DG, Czyzeswki D, Raimondi A J, et al: Central nervous system infection as a limiting factor in the intelligence of children with myelomeningocele. Pediatrics 70: 338-342, 1982 29. Menzies RG, Parkin JM, Hey EN: Prognosis for babies with myelomeningocele and high lumbar paraplegia at birth. Lancet 2:993-995, 1985 30. Odio C, McCracken GH, Nelson JD: CSF shunt infections in pediatrics. A seven-year experience. Am J Dis Child 138:1103-1108, 1984 31. Raimondi A J, Soare P: Intellectual development in shunting hydrocephalus children. Am J Dis Child 127: 664-672, 1974 32. Reigel DH: Spina bifida, in Section of Pediatric Neurosurgery of the American Association of Neurological Surgeons (eds): Pediatric Neurosurgery. New York: Grune & Stratton, 1982, pp 23-47 33. Rekate HR: To shunt or not to shunt: hydrocephalus and dysraphism. Clin Neurosurg 32:593-607, 1985 34. Shillito JS Jr: Surgical approaches to spina bifida and myelomeningocele. Ciin Neurosurg 20:114-133, 1973 35. Shurtleff DB, Hayden PW, Loeser JD, et al: Myelodys-
J. Neurosurg. / Volume 72/May, 1990
Management of myelomeningocele in Puerto Rico
36. 37. 38. 39.
40.
plasia: decision for death or disability. N Engl J Med 291: 1005-1010, 1974 Shurtleff DB, Kronmal R, Foltz EL: Follow-up comparison of hydrocephalus with and without myelomeningocele. J Neurosurg 42:61-68, 1975 Smith K, Smith BD: Selection for treatment in spina bifida cystica. Br Med J 4:189-197, 1973 Spindel MR, Bauer SB, Dyro FM, et al: The changing neurologic lesion in myelodysplasia. JAMA 258: 1630-1633, 1987 Stark GD, Drummond ME, Poneprassert S, et al: Primary ventriculo-peritoneal shunt in treatment of hydrocephalus associated with myelomeningocele. Arch Dis Child 49:112-117, 1974 Sutton LN, Charney EB, Bruce DA, et al: Myelomeningocele - - the question of selection. Clin Neurosurg 33:
J. Neurosurg. / Volume 7 2 / M a y , 1990
371-381, 1986 41. Till JS, Toole JF, Howard VJ, et al: Declining morbidity and mortality of carotid endarterectomy, the Wake Forest University Medical Center Experience. Stroke 18: 823-829, 1987 42. Venes JL: Surgical considerations in the initial repair of myelomeningocele and the introduction of a technical modification. Neurosurgery 17:111-113, 1985
Manuscript received October 3, 1988. Accepted in final form November 20, 1989. Address reprint requests to." Ricardo H. Brau, M.D., Department of Surgery, Section of Neurological Surgery, University of Puerto Rico, Medical Sciences Campus, G.P.O. Box 5067, San Juan, Puerto Rico 00936.
733
Experience in the management of myelomeningocele in Puerto Rico RICARDO H . BRAU, M.D., RAFAEL RODRiGUEZ, M.D., MAYRA VERA RAMiREZ, M.D., M.P.H., ROSARIO GONZALEZ, M.D., AND VIRGINIA MARTiNEZ, M . P . H .
Departments o f Neurosurgery, Pediatrics, and Biostatistics, University of Puerto Rico School of Medicine, San Juan, Puerto Rico
~" The medical records of 128 children with myelomeningocele who were treated at the Pediatric University Hospital, Puerto Rico Medical Center, from January, 1980, to July, 1985, were reviewed retrospectively. The medical and surgical management during the first hospitalization of these children was studied in detail for predefined parameters. The average age at the time of myelomeningocele repair was 6.6 days. Statistical analysis showed that repair of the myelomeningocele defect before 48 hours of age did not reduce the occurrence of ventriculitis. The incidence of ventriculitis secondary to the management of the myelomeningocele lesion was 12.5%. Complications after repair of the myelomeningocele (including skin flap necrosis, cerebrospinal fluid leaks, and wound infection) were present in 43.8% of the patients who developed ventriculitis and in 19.0% of those who did not. This observation is statistically significant (p = 0.03) and indicates that complications of healing after myelomeningocele repair represent the most significant risk factor for the development of ventriculitis. KEy WORDS
M
9
myelomeningocele
ANAGEMENT o f the newborn with myelomeningocele has stirred great controversy, 1'3~4'6' 9-12,14,15,20,23-25,27-29,35,39,40 the most critical issue being the identification o f which patients will benefit from treatment. This controversy involves medical, legal, ethical, moral, economic, social, and humanitarian issues. Our policy has been to operate in all cases. Having adopted this policy, we could concentrate on dealing with the neurosurgical problems of these children. Some of the questions that arise are: what is the optimal timing for myelomeningocele closure? which is the best technique? what is the best treatment of ventriculitis? what is the ideal t r e a t m e n t of hydrocephalus? and, finally, when is it best to institute cerebrospinal fluid (CSF) shunting? Some published studies have discussed the incidence of infection in newborns with myelomeningocele 28'37 and the associated risk factors. Although m a n y reports have been published assessing the long-term prognosis of children afflicted with myelomeningocele, l, l l, 16,21,27.35, 36,38there are few publications that comprehensively analyze the parameters 6'18'37 a n d the immediate outcome in these patients. For this reason, we decided to review our experience in the m a n a g e m e n t of newborns with myelomeningocele during the initial hospitalization. 726
9 ventriculitis
9 spina bifida
9 neonate
Summary of Cases
The medical records of all children with myelomeningocele treated at the Pediatric University Hospital, Puerto Rico Medical Center, between January, 1980, and July, 1985, were studied retrospectively. Almost all of the children born with myelomeningocele on the island of Puerto Rico and the United States Virgin Islands are referred to this institution for medical and surgical management. The hospital records o f 128 patients were identified. These records were reviewed and analyzed for the following parameters: sex, birth weight, location of the lesion, size of the myelomeningocele, presence of CSF leakage from the lesion, head size at birth, head size at the time of CSF shunting, presence of hydrocephalus, timing of closure of the myelomeningocele defect, duration of the operation for the closure of a spinal bifida cystica defect, the use o f prophylactic antibiotics prior to the myelomeningocele repair, complications in the healing of the myelomeningocele repair, presence of ventriculitis before or after the shunting procedure, the organism producing ventriculitis, type of shunting procedure used, timing of the shunting procedure, duration of the shunting procedure, use of prophylactic antibiJ. Neurosurg. / Volume 72/May, 1990
Management of myelomeningocele in Puerto Rico otics at the t i m e o f t h e s h u n t i n g procedure, incidence o f ventriculitis after s h u n t i n g , o r g a n i s m s p r o d u c i n g the infection, m o r t a l i t y , a n d t h e total hospitalization time. These children were m a n a g e d following a s t a n d a r d t r e a t m e n t protocol. M o s t p a t i e n t s were b o r n elsewhere a n d transferred to t h e P e d i a t r i c University H o s p i t a l after they were stable e n o u g h for t r a n s p o r t a t i o n . T h e y were a d m i t t e d to the p e d i a t r i c n e w b o r n service w a r d or the intensive care u n i t d e p e n d i n g o n the general condition o f the infant. T h e y were k e p t in a p r o n e p o s i t i o n a n d the m y e l o m e n i n g o c e l e defect was covered with s a l i n e - m o i s t e n e d gauze. P r o p h y l a c t i c antibiotics were not used while the c h i l d was being stabilized or waiting for surgery b u t were u s e d liberally if i n d i c a t e d for o t h e r conditions. P r o p h y l a c t i c a n t i b i o t i c s were a d m i n i s t e r e d prior to surgery in t h e o p e r a t i n g r o o m according to the surgeon's preference. T h e m y e l o m e n i n g o c e l e was closed urgently (as s o o n as possible) b u t n o t on an e m e r g e n c y basis. M y e l o m e n i n g o c e l e r e p a i r was carried out using s t a n d a r d t e c h n i q u e s j 3~19-21.32.34.42t a i l o r e d to the individual case. H e a d g r o w t h was carefully m o n i t o r e d a n d a diagnosis o f h y d r o c e p h a l u s was c o n f i r m e d with a h e a d c o m p u t e r i z e d t o m o g r a p h y (CT) scan. A shunt system for h y d r o c e p h a l u s c o n t r o l was p l a c e d after the child was assessed to be w i t h o u t active m e d i c a l p r o b l e m s , usually after the m y e l o m e n i n g o c e l e repair was n o t e d to be healing properly. Statistical analysis o f the d a t a was p e r f o r m e d with analysis o f v a r i a n c e u s i n g a statistical c o m p u t e r software package for the social sciences.* A p r o b a b i l i t y o f p < 0.05 was c o n s i d e r e d significant. Results There were 62 m a l e s (48.4%) a n d 66 females (51.6%) in this series (Table 1). T h e average birth weight was 6.6 lbs (range 2.3 to 10.4 lbs). T h e defect was located in the t h o r a c i c a r e a in t h r e e cases (2.3%), in the t h o r a c o l u m b a r area in 41 cases (32.0%), in the l u m b a r a r e a in 28 (21.9%), a n d in the l u m b o s a c r a l area in 56 (43.8%). T h e size o f t h e m y e l o m e n i n g o c e l e lesion was recorded in 50 cases: t h e average area was 29.9 sq c m (range 3.0 to 100.0 sq cm). A C S F leak after birth was described in 12 cases (9.4%). T h e m y e l o m e n i n g o c e l e defect was r e p a i r e d in 126 p a t i e n t s (98.4%) at an average age o f 6.6 d a y s (range < 24 h o u r s to 52.3 days). T h e average d u r a t i o n o f t h e m y e l o m e n i n g o c e l e repair, i n c l u d i n g the anesthesia, i n t u b a t i o n , a n d e x t u b a t i o n time, was 3.4 h o u r s a n d p r o p h y l a c t i c antibiotics at surgery were used in 73 cases (57.0%). Back w o u n d healing c o m p l i c a t i o n s , i n c l u d i n g skin necrosis, C S F leak, a n d w o u n d i n f e c t i o n , o c c u r r e d in 26 cases (20.3%). Ventriculitis p r i o r to s h u n t p l a c e m e n t develo p e d in 16 cases (12.5 %). T h e m o s t c o m m o n organisms in o r d e r o f f r e q u e n c y were Klebsiella, Acinetobacter,
* Statistical package SPSSx obtained from SSPS, Inc., Chicago, Illinois; IBM 4361-3 system obtained from International Business Machines, Boca Raton, Florida.
J. Neurosurg. / Volume 7 2 / M a y , 1990
TABLE 1
Myelomeningocele assessed parameters in 128 patients* Parameter Value sex (M:F) 62 (48.4%): 66 (51.6%) average birth weight 6.6• 1.41bs location of lesion thoracic 3 cases (2.3%) thoracolumbar 41 cases (32.0%) lumbar 28 cases (21.9%) lumbosacral 56 cases (43.8%) average size of lesion (50 cases) 29.9 sq cm CSF leak before repair 12 cases (9.4%) average age at time of repair 6.6 • 7.3 days duration of repair surgery 3.4• 1.1 hrs prophylactic antibiotics at repair 73 cases (57.0%) complications of repair 26 cases (20.3%) nonshunt-related ventrieulitis 16 cases (12.5%) average hospital stay 42.2 • 26.3 days mortality 0 (O%) * Mean values are given • standard deviation. CSF = cerebrospinal fluid.
TABLE 2
Parameters assessed in 97 patients with hydrocephalus* Parameter Value shunt placement 97 cases head circumference at birth 36.1 - 4.9 cm head circumference at shunt placement 39.4 • 4.5 cm average age at shunt placement 30.4 • 17.7 days prophylactic antibiotics given at shunt placement 68 cases (70.1%) duration of shunting procedure 2.3 • 0.8 hrs shunt-related ventriculitis 5 cases (5.2%) * Mean values are expressed _+ standard deviation.
Escherichia coli, P s e u d o m o n a s , Serratia, Staphylococcus epidermidis, Enterococcus, a n d Enterobacter species. T h e average l e n g t h o f h o s p i t a l i z a t i o n t i m e for the 128 p a t i e n t s was 42.2 d a y s (range 4 to 170 days). T h e r e were n o d e a t h s d u r i n g t h e i n i t i a l h o s p i t a l i z a t i o n in this series. H y d r o c e p h a l u s was d i a g n o s e d d u r i n g the initial hosp i t a l i z a t i o n in 97 cases (75.8%), a n d all were t r e a t e d with the i n s e r t i o n o f t h e v e n t r i c u l o p e r i t o n e a l s h u n t systems. A s u m m a r y o f f i n d i n g s in t h e h y d r o c e p h a l i c p a t i e n t s is given in T a b l e 2. T h e a v e r a g e age at s h u n t p l a c e m e n t was 30.4 d a y s . T h e a v e r a g e h e a d c i r c u m f e r ence at b i r t h was 36.1 c m a n d at t h e t i m e o f s h u n t p l a c e m e n t was 39.4 c m . T h e a v e r a g e t i m e for the s h u n t ing p r o c e d u r e , i n c l u d i n g i n t u b a t i o n a n d e x t u b a t i o n t i m e , was 2.3 h o u r s . P r o p h y l a c t i c a n t i b i o t i c s were u s e d in 68 cases (70.1%) at t h e t i m e o f s h u n t surgery. V e n triculitis was d i a g n o s e d in five p a t i e n t s (5.2%) after s h u n t insertion. T h e o r g a n i s m s i d e n t i f i e d as t h e c a u s e o f ventriculitis after s h u n t p l a c e m e n t were Staphylo-
coccus epidermidis, Streptococcus, Pseudomonas, Alcaligenes faecalis, a n d Citrobacter.
72-/
R. H. Brau, et al. TABLE 3
Timing of myelomeningocele repair
Age at Repair (days) 0-2 3-6 6-12 > 12
Nonshunt-Related Ventriculitis ( 16 cases) 31.3% 37.5% 6.3% 25.0%
No Ventriculitis ( 110 cases) 26.0% 44.0% 16.0% 14.0%
Discussion
The incidence o f myelomeningocele in the United States is 1 to 2 per 1000 live births. 19'32 A significant variation in this incidence has been reported for different ethnic groups; however, this has been estimated to be the incidence of myelomeningocele in the Puerto Rican population (unpublished data). The defect in embryogenesis that leads to the formation of the myelomeningocele is usually thought to occur during the 4th week o f gestation. 2"~3 The mechanism that triggers the derangement o f the complex synchronized steps of spinal cord f o r m a t i o n is not known. In 1982, M c L o n e and associates 28 published a study in which they implicated central nervous system (CNS) infection as the m a j o r factor limiting the intelligence of children afflicted with myelomeningocele. They found that ventriculitis reduced the intelligence quotient (IQ) by 25% (average IQ o f 72 in the ventriculitis group vs. an average IQ of 95 in the group that had never been so infected). In a m o r e recent article, McLone 2v stated that almost 80% o f children with myelomeningocele should have n o r m a l intelligence. Convinced that a n o r m a l intelligence is critical to assure an independent and productive life for patients with myelomeningocele, we elected to examine in detail the different p a r a m e t e r s between patients who developed ventriculitis a n d those who did not. Ventriculitis was diagnosed at different stages during the care of these patients and was identified either before myelomeningocele repair, after repair but before shunt insertion, or after shunt placement. The association of ventriculitis with shunt insertion has been studied extensively; 5"8"j3"~9"22"2s3~ however, the connection between ventriculitis and myelomeningocele repair itself has not received m u c h attention. 6"j3374~ Therefore, this study e x a m i n e d in detail all cases in which ventriculitis developed. Special emphasis was given to those in which the infectious process was not directly associated to the shunting procedure itself. The group of patients who developed ventriculitis before shunt placem e n t will be t e r m e d the "nonshunt-related ventriculitis group." Ventriculitis I n c i d e n c e
The incidence o f ventriculitis before shunt placement in this study was 12.5% (16 cases); the incidence re728
ported in the literature varies from 10% to 2 5 % . 6"13"37"40 The organisms found in these patients were usually gastrointestinal tract or skin colonizers. Five patients were infected with m o r e than one organism. The organisms isolated from the ventricular fluid in our patients followed a similar pattern to those found in the series reported by Charney, et al. 6 Timing o f Surgery One of the most controversial management variables is the optimal timing of myelomeningocele repair. The myelomeningocele should be closed within 48 hours of birth according to some authors, j~ Other reports indicate that closure can be performed after 48 hours without detrimental effects to the child. 1.6.JJ.14.37.4o In this series, the average age at the time of the myelomeningocele repair was 6.6 days. In two patients, myelomeningocele repair was not performed during the initial hospitalization; however, all patients with documented ventriculitis underwent closure of the defect during the initial hospitalization. The average age at the time of repair of the 16 patients who developed ventriculitis was 9.7 days, and the average age of those who did not develop infection was 6.2 days (Table 3). This correlation is not statistically significant (p = 0.07). Of the 16 children who developed ventriculitis, five (31.3%) were operated on within 48 hours of birth, six (37.5%) were operated on between 3 and 6 days of age, and the remaining five patients underwent repair at age 12 days (one case), 16 days (one case), 20 days (two cases), and 52 days (one case). In the group of 110 children who did not develop CNS infection, 26% underwent myelomeningocele repair within 48 hours, 44% between 3 and 6 days, 16% between 6 and 12 days, and 14% after 12 days (the two patients without surgery were excluded). O f the 16 cases with nonshunt-related ventriculitis, three were diagnosed as having ventriculitis before the myelomeningocele was repaired and 13 after surgery. In the three patients in w h o m surgery was performed after the diagnosis of ventriculitis, the repair was carried out after the CNS infection was totally eradicated. Two of these underwent surgery at the age of 20 days and the third at 52 days. If these three cases are eliminated, the average age at surgery for the group that developed CNS infection was 4.7 days. In this series, the recommended period of 48 hours after birth as the optimal timing for myelomeningocele defect closure, as mentioned by some reports, ~~ was not found to produce a significant difference in the overall incidence of ventriculitis. Size o f M y e l o m e n i n g o c e l e The size of the myelomeningocele was compared between both groups. The average size of the defect was recorded in 50 cases, nine (56.3%) of the 16 cases with nonshunt-related ventriculitis and 41 (37.2%) o f the 110 cases without. The average size of the lesion was 29.9 sq cm for the total group, 32.3 sq cm for the J. Neurosurg. / Volume 72/May, 1990
Management of myelomeningocele in Puerto Rico ventriculitis group, and 28.8 sq cm for the group without infection (Tables 1 and 4). There was no statistical difference between the groups (p = 0.58). Cerebrospinal F l u i d L e a k In a recent report, Hubballah and Hoffman ~8pointed out that children who are born with overt hydrocephalus associated with a myelomeningocele are at higher risk of CSF leakage and CNS infection than other myelomeningocele patients. We compared the head circumferences at birth in our target groups. The average head circumference at birth for the entire population was 36.1 cm, for the group with the nonshuntrelated CNS infection it was 35.0 cm, and for the group without ventriculitis it was 36.2 cm. There was no statistical correlation between the groups (p = 0.36). Therefore, overt hydrocephalus was not a risk factor in the development of ventriculitis in this series. Active leaking of CSF through the myelomeningocele defect was documented in 12 cases (10.2%): three patients (18.8%) developed nonshunt-related ventriculitis and nine (8.0%) did not develop ventriculitis (no statistical correlation; p = 0.40). This finding is interesting because it does not agree with previous r e p o r t s . 9"32'33 We have no explanation for this finding. Surgical Variables Surgical complication rates may be associated with different surgeons, the duration of the procedure, or other documented parameters. 4~ With this in mind, we investigated whether the surgeon or the time required to perform the surgery contributed to the incidence of ventriculitis in this series. In our institution, most myelomeningocele closures are performed by the senior resident staff. There was no correlation between the incidence of CNS infection and the surgeon performing the procedure (p = 0.29). There was no statistical difference between the groups in the overall time in the operating room for myelomeningocele repair (p = 0.17). The use of systemic prophylactic antibiotics prior to myelomeningocele closure did not lower the incidence of nonshunt-related ventriculitis (p = 0.16). Also, low birth weight was found not to increase the incidence of ventriculitis or surgical complications (Table 4). Wound-Healing P r o b l e m s Information regarding wound-healing complications after myelomeningocele repair was recorded in 116 of the 126 surgical cases. Data were available for all patients who developed nonshunt-related ventriculitis. Back wound-healing problems were defined as skin necrosis, CSF leakage, and gross wound infection. There were 26 cases (22.4%) with these problems and 90 cases (77.6 %) with no healing complications after myelomeningocele repair. In the nonshunt-related ventriculitis group, seven patients (43.8%) developed back woundhealing problems, while only 21 (19.0%) of the patients who did not develop ventriculitis had myelomeningoJ. Neurosurg. / Volume 7 2 / M a y , 1990
TABLE 4 Nonshunt-related ventriculitis group versus group without ventriculitis NonshuntRelated Ventriculitis no. of cases 16 birth weight 6.64 lbs average size of defect (50 cases) 32.3sq cm head circumference at birth 35.0 cm CSF leak before repair 18.8% duration of repair 3.2 hrs prophylactic antibiotics 81.3% wound-healing problems 43.8% * CSF = cerebrospinal fluid. I"Statistically significant value. Parameter*
No Ventri- P culitis Value 1l0 6.57 lbs 28.8 sq cm 36.2 cm 8.0% 2.9 hrs 59.4% 19.0%
0.89 0.58 0.36 0.40 0.17 0.16 0.03t
cele repair healing problems (statistically significant; p = 0.03). In the group with nonshunt-related ventriculitis, three patients developed skin necrosis, one a CSF leak, and three gross skin infection. Ventriculitis Versus W o u n d H e a l i n g The relationship between healing problems of the myelomeningocele repair and ventriculitis have been recorded sporadically in the literature. In 1973, Foltz, et al., j~ reported a 14% incidence of myelomeningocele repair breakdown and stated that this incidence could be reduced to between 3% and 5% if hydrocephalic patients received shunts before myelomeningocele repair. Guthkelch and associates ~6 reported a 7% incidence of CSF leaks after myelomeningocele repair with fasciomuscular flaps and aggressive use of ventriculostomies. Lehman and collaborators, z~ conscious about the importance of wound necrosis and dehiscence, suggested using nitroglycerin ointment to enhance cutaneous blood flow in myelomeningocele closure. Other authors have reported different techniques using skin flaps with the purpose of avoiding tension at the skin edges and skin necrosis. 7"~7 Our study strongly associated the incidence of ventriculitis with healing complications of the myelomeningocele closure. We are not aware of any other study that correlated these two variables so definitely. Different repair techniques that might minimize these complications should be considered. Different techniques for myelomeningocele repair have been reported, 7"13"17"2~ and a controlled trial between them may provide useful information about which method is best. Hydrocephalus There were 97 patients (75.6%) diagnosed as having hydrocephalus during their first hospitalization. All were treated with the insertion o f a ventriculoperitoneal shunt. The incidence o f ventriculitis after shunt insertion in this series was 5.2% or five of the 97 cases (Table 2). The complication of ventriculitis after shunt insertion and the different parameters and variables 729
R. H. Brau, et al. that i n f l u e n c e it h a v e b e e n discussed p r e v i o u s l y ? "8"t3" 19,22,28,30,32,33,36,39 A l t h o u g h i n - d e p t h analysis o f our five cases w o u l d n o t p r o d u c e a n y c o n c l u s i v e observations, a survey o f t h e p a r a m e t e r s s t u d i e d b e t w e e n t h e group with s h u n t - r e l a t e d ventriculitis a n d the r e m a i n i n g p a tients failed t o s h o w a statistical r e l a t i o n s h i p for: birth weight, h e a d size at the t i m e o f s h u n t i n s e r t i o n , age at s h u n t p l a c e m e n t , d u r a t i o n o f s h u n t surgery, a n d the use o f p r o p h y l a c t i c antibiotics.
Mortality A few p u b l i c a t i o n s have e x a m i n e d the survival o f the n e w b o r n w i t h m y e l o m e n i n g o c e l e . T h e g r o u p f r o m the C h i l d r e n ' s H o s p i t a l at P h i l a d e l p h i a r e p o r t e d no m o r tality b y 10 m o n t h s o f age in 52 p a t i e n t s with early spina b i f i d a cystica repair, a n d a 6% m o r t a l i t y rate in the g r o u p w i t h late surgery. 4~ G r o s s a n d associates ~4 r e p o r t e d 100% survival in t h e i r g r o u p t h a t received active m e d i c a l t h e r a p y (one p a t i e n t d i e d at age 14 m o n t h s in a m o t o r - v e h i c l e accident). T h e r e were no d e a t h s in o u r series.
Acknowledgments The authors are indebted to Dr. Nathan Rifkinson and Dr. Humberto Ortiz for editorial advice. References 1. Ames MD, Schut L: Results of treatment of 171 consecutive myelomeningoceles - - 1963 to 1968. Pediatrics 50: 466-470, 1972 2. Angevine JB: Clinical relevant embryology of the vertebral column and spinal cord. Clin Neurosurg 20:95-113, 1973 3. Barden GA, Meyer LC, Stelling FH: Myelodysplasia - fate of those followed for twenty years or more. J Bone Joint Surg (Am) 57:643-647, 1975 4. Black PM: Selective treatment of infants with myelomeningocele. Neurosurgery 5:334-338, 1979 5. Borges LF: Cerebrospinal fluid shunts interfere with host defenses. Neurosurgery 10:55-59, 1982 6. Charney EB, Weller SC, Sutton LN, et al: Management of the newborn with myelomeningocele: time decisionmaking process. Pediatrics 75:58-64, 1985 7. Cruz NI, Ariyan S, Duncan CC, et al: Repair of lumbosacral myelomeningoceles with double Z-rhomboid flaps. Technical note. J Neurosurg 59:714-717, 1983 8. Djindjian M, Fevrier M J, Otterbein G, et al: Oxaciltin prophylaxis in cerebrospinal fluid shunt procedures: results of a randomized open study in 60 hydrocephalic patients. Surg Neurol 25:178-180, 1987 9. Epstein F: Myelomeningocele - - "pitfalls" in early and late management. Clin Neurosurg 30:366-384, 1983 10. Foltz E: Myelomeningocele: selection for treatment, in O'Brien MS (ed): Pediatric Neurological Surgery. Seminars in Neurological Surgery. New York: Raven Press, 1978, pp 105-124 11. Foltz EL, Kronmal R, Shurtleff DB: To treat or not to treat: a neurosurgeon's perspective of myelomeningocele. Clin Neurosurg 20:147-163, 1973 12. Freeman JM: To treat or not to treat: ethical dilemmas of treating the infant with a myelomeningocele. Clin
730
Neurosurg 20:134-146, 1973 13. French BN: Midline fusion defects and defects of formation, in Youmans JR (ed): Neurological Surgery, ed 2. Philadelphia: WB Saunders, 1982, Vol 3, pp 1236-1380 14. Gross RH, Cox A, Tatyrek R, et al: Early management and decision making for the treatment of myelomeningocele. Pediatrics 72:450-458, 1983 15. Guthkelch AN: The indications and contraindications for early operation in myelomeningocele, in Morley TP (ed): Current Controversies in Neurosurgery. Philadelphia: WB Saunders, 1976, pp 147-154 16. Guthkelch AN, Pang D, Vries JK: Influence of closure technique on results in myelomeningocele. Childs Brain 8:350-355, 1981 17. Habal MB, Vries JK: Tension free closure of large myelomeningocele defects. Surg Neurol 8:177-180, 1977 18. Hubballah MY, Hoffman H J: Early repair of myelomeningocele and simultaneous insertion of ventriculoperitoneal shunt: technique and results. Neurosurgery 20: 21-23, 1987 19. Humphreys RP: Spinal dysraphism, in Wilkins RH, Rengachary SS (eds): Neurosurgery. New York: McGrawHill, 1985, Vol 3, pp 2041-2052 20. Lehman RAW, Page RB, Saggers GC, et al: Technical note: the use of nitroglycerin ointment after precarious neurosurgical wound closure. Neurosurgery 16:701-702, 1985 21. Lindseth RE, Stelzer L: Vertebral excision for kyphosis in children with myelomeningocele. J Bone Joint Snrg (Am) 61:699-704, t979 22. Liptak GS, Masiulis BS, McDonald JV: Ventricular shunt survival in children with neural tube defects. Acta Neuroehir 74:113-117, 1985 23. Lorber J: Results of treatment of myelomeningocete: an analysis of 524 unselected cases, with special reference to possible selection for treatment. Dev Med Child Neurol 13:279-303, 1971 24. Lorber J: Spina bifida cystica. Arch Dis Child 47: 854-873, 1972 25. MacKeith RC: New look at spina bifida aperta. Dev Med Child Neurol 13:277-278, 1971 (Editorial) 26. McLone DG: Results of treatment of children born with a myelomeningocele. Clin Neurosurg 30:407-412, 1983 27. McLone DG: Treatment of myelomeningocele: arguments against selection. Clin Neurosurg 33:359-370, 1986 28. McLone DG, Czyzeswki D, Raimondi A J, et al: Central nervous system infection as a limiting factor in the intelligence of children with myelomeningocele. Pediatrics 70: 338-342, 1982 29. Menzies RG, Parkin JM, Hey EN: Prognosis for babies with myelomeningocele and high lumbar paraplegia at birth. Lancet 2:993-995, 1985 30. Odio C, McCracken GH, Nelson JD: CSF shunt infections in pediatrics. A seven-year experience. Am J Dis Child 138:1103-1108, 1984 31. Raimondi A J, Soare P: Intellectual development in shunting hydrocephalus children. Am J Dis Child 127: 664-672, 1974 32. Reigel DH: Spina bifida, in Section of Pediatric Neurosurgery of the American Association of Neurological Surgeons (eds): Pediatric Neurosurgery. New York: Grune & Stratton, 1982, pp 23-47 33. Rekate HR: To shunt or not to shunt: hydrocephalus and dysraphism. Clin Neurosurg 32:593-607, 1985 34. Shillito JS Jr: Surgical approaches to spina bifida and myelomeningocele. Ciin Neurosurg 20:114-133, 1973 35. Shurtleff DB, Hayden PW, Loeser JD, et al: Myelodys-
J. Neurosurg. / Volume 72/May, 1990
Management of myelomeningocele in Puerto Rico
36. 37. 38. 39.
40.
plasia: decision for death or disability. N Engl J Med 291: 1005-1010, 1974 Shurtleff DB, Kronmal R, Foltz EL: Follow-up comparison of hydrocephalus with and without myelomeningocele. J Neurosurg 42:61-68, 1975 Smith K, Smith BD: Selection for treatment in spina bifida cystica. Br Med J 4:189-197, 1973 Spindel MR, Bauer SB, Dyro FM, et al: The changing neurologic lesion in myelodysplasia. JAMA 258: 1630-1633, 1987 Stark GD, Drummond ME, Poneprassert S, et al: Primary ventriculo-peritoneal shunt in treatment of hydrocephalus associated with myelomeningocele. Arch Dis Child 49:112-117, 1974 Sutton LN, Charney EB, Bruce DA, et al: Myelomeningocele - - the question of selection. Clin Neurosurg 33:
J. Neurosurg. / Volume 7 2 / M a y , 1990
371-381, 1986 41. Till JS, Toole JF, Howard VJ, et al: Declining morbidity and mortality of carotid endarterectomy, the Wake Forest University Medical Center Experience. Stroke 18: 823-829, 1987 42. Venes JL: Surgical considerations in the initial repair of myelomeningocele and the introduction of a technical modification. Neurosurgery 17:111-113, 1985
Manuscript received October 3, 1988. Accepted in final form November 20, 1989. Address reprint requests to." Ricardo H. Brau, M.D., Department of Surgery, Section of Neurological Surgery, University of Puerto Rico, Medical Sciences Campus, G.P.O. Box 5067, San Juan, Puerto Rico 00936.
733
