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Symposium on myelomeningoceles and their complications
592
INTERNATIONAL
AN ASSESSMENTOF THE TREATMENTOF LOMENINGOCOELES.
and
C.
Froman.
(May 24),
R.
Lipschitz,
S. Afr.
Med.
J.
ENCEPHA-
M.
Beck,
J. 43:609-610
1969.
This presentation reviews findings, management, and results obtained in 34 cases of encephalomeningocoele seen over 2% years. Of these cases, 17 were in the occipital area, 2 in the parietal, and 15 were classified as sincipital. Of the sincipital group, 2 were high cerbital, 10 protruded anteriorly between the nasal bones, 2 were intranasal, and one was in the area of the anterior fontanelle. Diagnosis by physical findings and x-ray is usually fairly straightforward. The timing of operative repair is based upon the size of the defect and the integrity of its skin covering. The decision to operate depends upon the general condition of the child and the magnitude of the neurologic deficit. Repair of occipital and parietal lesions consists of excision with a repair in layers of dura, galea, and skin. In wide defects, the dura may be left open without ill effects. The sincipital defects often require a craniotomy with repair of the dural defect with removal of the external mass at a second stage. Results are good with sincipital defects. Of the 12 cases subjected to repair 11 are well and of normal intelligence whereas one child has epilepsy. With occipital defects the outlook is poorer. Of 17 cases operated, 4 died, 5 suffered mental retardation, and 8 are living normally. In both cases of parietal lesions the results were excellent.-H. V. Firor. SYMPOSIUM
ON MYELOMENINGOCELES
COMPLICATIONS. uary),
Ann.
Chir.
Inf.:
AND THEIR 5-135
(Jan-
1969.
The whole January number of Annales de Chiris devoted to the huge problem of the severe forms of spina bifida. After a short introduction by Bettex (Berne), the ethical, philosophical, religious, and social implications of the intensive and total care of myelomeningocele are magnificiently discussed by Zachary (Sheffield). In a second article, Bettex (Berne) gives the rationale of early surgical treatment of myelomeningocele. Every open myelomeningocele should be operated upon as an emergency in the first 6 hours of life. The operative technic and the results in 52 patients are discussed. In the next article, Rickham (Liverpool) reports his great experience in the treatment of hydrocephalus (more than 600 cases) complicating urgie Infantile
ABSTRACTS
OF PEDIATRIC
SURGERY
cystic myelomeningocele. Ventriculo-atria1 drainage with interposition of a valve is the best treatment to date. Rickham utilizes the Holter valve exclusively, with very good results (no operative mortality; only 12 per cent late mortality; 40 per cent nonlethal complications). It is very important to reoperate as soon as the drainage system is no longer functioning. With the interposition of a “Rickham reservoir,” however, most of the blockage accidents may be corrected without new operations. Haegel (Paris) describes a simplified technic of early operation for myelomeningocele. Nicole (Basel) discusses some complications of drainage for hydrocephalus, especially secondary craniosynostosis. Kuffer and Weber (Berne) propose anticoagulation as a long-term prophylaxis against thrombosis of the superior vena cava after ventriculo-atria1 drainage. Myelomeningocele is not only a neurosurgical problem. Its urologic complications may have a most severe prognosis. Genton and co-workers (Lausanne) discuss here all types of neurogenic bladder encountered in myelomeningocele: the atonic bladder, the autonomic bladder, the automatic bladder, and the noninhibited neurogenic bladder. Early urologic investigation is very important. Conservative treatment of neurogenic bladder in spina bifida cystica is very discouraging, but should be tried if there is no risk of renal insufficiency. In case of vesico-ureteric reflux, megaureter, hydronephrosis, and renal failure, bladder training can be dangerous. In such cases, urinary diversion is mandatory (cystostomy, cutaneous ureterostomy, ileal conduit, etc.). One of the most important investigations in neurogenic bladder is micturating cystourethrography. Nussle (Lausanne) reports his very interesting experience with the 70 mm. fluoroscopic camera. The experience of the Children’s Hospital of Munich is reported by Pompino and co-workers in a short but well-documented paper dealing with the urologic findings in 30 children after operation for myelomeningocele. Twenty-five of them have total urinary incontinence one to 5 years after surgery. Most of the children with myelomeningocele have problems in defecation. Cuendet (Geneva) devotes his contribution to the problem of anorectal function in spina bifida. The importance of the examination of a bulbocavernous reflex is stressed. Although most of the children with myelomeningocele have a total sphincteric incompetence, they almost all have constipation and thus are not too much distressed by the incontinence. The orthopedic complications of myelomenin-
INTERNATIONAL
ABSTRACTS
OF PEDIATRIC
SURGERY
gocele are very complex and very difficult to treat. Taillard and co-workers (Geneva) give a very complete review of this topic. The posterior transplantation of the psoas according to Sharrard is the best operation in preventing neurogenic luxation of the hip. Two more articles are devoted to this theme, one by Beau and Guillaumot (Nancy) and one by Nicod (Lausanne). In a last paper, Marques-Gubern and co-workers Barcelona) report their experience with myelomeningocele in Spain. They lay stress on the importance of the mental development of children with spina bifida in relation to their integration in the life of the community. In brief, this number of Annales de Chirurgie Ztrfanrilr is one of the best reviews of the problem of myelomeningocele
in recent years.-M.
Bette.r.
ANTIHIOTIC SPRAY DURING AND AFTER CLOSURE OF MYELOMENINGOCELE.D. Formby and 1. Buis.sotr. Aust. Paed. J. 5:3-7 (March), 1969.
In a control series of 30 infants with myelomeningocele 14 received an antibiotic spray (Neomycin, Polymixin, and Bacitracin) during and after early closure. Two became infected. Two children were excluded and of the comparable
593
14 infants not sprayed, 10 developed their stay in the hospital notably
infections and lengthened.-
I. R. Solomon.
NEOPLASMS MALIGNANT, APPARENTLY NONSECRETORYTUMORS OF THE AUTONOMIC NERVOUS SYSTEM IN CHILDREN. H. K&r, H. P. Wagner, and F. Ku&r.
Helv. Paediat.
Acta 24:128,
1969.
In a case of neuroblastoma in a 1 2/12-year-old female infant reported here, the author found normal values for the urinary output of vanillylmandelic acid and of homovanillic acid, but the excretion of vanillyllactic acid was exceedingly increased. In a series of 142 neuroblastomas and ganglioneuroblastomas, they found 13 other cases without increased excretion of VMA and HVA: but in all these 13 cases, dopamin, 3-methoxytyramine, normetanephrine, 3-methoxytyrosine, and/or vanillyllactic acid were increased in the urine. It is thus very important in order to further improve the diagnostic reliability of quantitative determination of catecholamine catabolites in tumors of the autonomic nervous system, to investigate not only the urinary output of VMA and HVA, hut of all the catecholamine catabolites mention above.M. Bettex.
INTERNATIONAL
AN ASSESSMENTOF THE TREATMENTOF LOMENINGOCOELES.
and
C.
Froman.
(May 24),
R.
Lipschitz,
S. Afr.
Med.
J.
ENCEPHA-
M.
Beck,
J. 43:609-610
1969.
This presentation reviews findings, management, and results obtained in 34 cases of encephalomeningocoele seen over 2% years. Of these cases, 17 were in the occipital area, 2 in the parietal, and 15 were classified as sincipital. Of the sincipital group, 2 were high cerbital, 10 protruded anteriorly between the nasal bones, 2 were intranasal, and one was in the area of the anterior fontanelle. Diagnosis by physical findings and x-ray is usually fairly straightforward. The timing of operative repair is based upon the size of the defect and the integrity of its skin covering. The decision to operate depends upon the general condition of the child and the magnitude of the neurologic deficit. Repair of occipital and parietal lesions consists of excision with a repair in layers of dura, galea, and skin. In wide defects, the dura may be left open without ill effects. The sincipital defects often require a craniotomy with repair of the dural defect with removal of the external mass at a second stage. Results are good with sincipital defects. Of the 12 cases subjected to repair 11 are well and of normal intelligence whereas one child has epilepsy. With occipital defects the outlook is poorer. Of 17 cases operated, 4 died, 5 suffered mental retardation, and 8 are living normally. In both cases of parietal lesions the results were excellent.-H. V. Firor. SYMPOSIUM
ON MYELOMENINGOCELES
COMPLICATIONS. uary),
Ann.
Chir.
Inf.:
AND THEIR 5-135
(Jan-
1969.
The whole January number of Annales de Chiris devoted to the huge problem of the severe forms of spina bifida. After a short introduction by Bettex (Berne), the ethical, philosophical, religious, and social implications of the intensive and total care of myelomeningocele are magnificiently discussed by Zachary (Sheffield). In a second article, Bettex (Berne) gives the rationale of early surgical treatment of myelomeningocele. Every open myelomeningocele should be operated upon as an emergency in the first 6 hours of life. The operative technic and the results in 52 patients are discussed. In the next article, Rickham (Liverpool) reports his great experience in the treatment of hydrocephalus (more than 600 cases) complicating urgie Infantile
ABSTRACTS
OF PEDIATRIC
SURGERY
cystic myelomeningocele. Ventriculo-atria1 drainage with interposition of a valve is the best treatment to date. Rickham utilizes the Holter valve exclusively, with very good results (no operative mortality; only 12 per cent late mortality; 40 per cent nonlethal complications). It is very important to reoperate as soon as the drainage system is no longer functioning. With the interposition of a “Rickham reservoir,” however, most of the blockage accidents may be corrected without new operations. Haegel (Paris) describes a simplified technic of early operation for myelomeningocele. Nicole (Basel) discusses some complications of drainage for hydrocephalus, especially secondary craniosynostosis. Kuffer and Weber (Berne) propose anticoagulation as a long-term prophylaxis against thrombosis of the superior vena cava after ventriculo-atria1 drainage. Myelomeningocele is not only a neurosurgical problem. Its urologic complications may have a most severe prognosis. Genton and co-workers (Lausanne) discuss here all types of neurogenic bladder encountered in myelomeningocele: the atonic bladder, the autonomic bladder, the automatic bladder, and the noninhibited neurogenic bladder. Early urologic investigation is very important. Conservative treatment of neurogenic bladder in spina bifida cystica is very discouraging, but should be tried if there is no risk of renal insufficiency. In case of vesico-ureteric reflux, megaureter, hydronephrosis, and renal failure, bladder training can be dangerous. In such cases, urinary diversion is mandatory (cystostomy, cutaneous ureterostomy, ileal conduit, etc.). One of the most important investigations in neurogenic bladder is micturating cystourethrography. Nussle (Lausanne) reports his very interesting experience with the 70 mm. fluoroscopic camera. The experience of the Children’s Hospital of Munich is reported by Pompino and co-workers in a short but well-documented paper dealing with the urologic findings in 30 children after operation for myelomeningocele. Twenty-five of them have total urinary incontinence one to 5 years after surgery. Most of the children with myelomeningocele have problems in defecation. Cuendet (Geneva) devotes his contribution to the problem of anorectal function in spina bifida. The importance of the examination of a bulbocavernous reflex is stressed. Although most of the children with myelomeningocele have a total sphincteric incompetence, they almost all have constipation and thus are not too much distressed by the incontinence. The orthopedic complications of myelomenin-
INTERNATIONAL
ABSTRACTS
OF PEDIATRIC
SURGERY
gocele are very complex and very difficult to treat. Taillard and co-workers (Geneva) give a very complete review of this topic. The posterior transplantation of the psoas according to Sharrard is the best operation in preventing neurogenic luxation of the hip. Two more articles are devoted to this theme, one by Beau and Guillaumot (Nancy) and one by Nicod (Lausanne). In a last paper, Marques-Gubern and co-workers Barcelona) report their experience with myelomeningocele in Spain. They lay stress on the importance of the mental development of children with spina bifida in relation to their integration in the life of the community. In brief, this number of Annales de Chirurgie Ztrfanrilr is one of the best reviews of the problem of myelomeningocele
in recent years.-M.
Bette.r.
ANTIHIOTIC SPRAY DURING AND AFTER CLOSURE OF MYELOMENINGOCELE.D. Formby and 1. Buis.sotr. Aust. Paed. J. 5:3-7 (March), 1969.
In a control series of 30 infants with myelomeningocele 14 received an antibiotic spray (Neomycin, Polymixin, and Bacitracin) during and after early closure. Two became infected. Two children were excluded and of the comparable
593
14 infants not sprayed, 10 developed their stay in the hospital notably
infections and lengthened.-
I. R. Solomon.
NEOPLASMS MALIGNANT, APPARENTLY NONSECRETORYTUMORS OF THE AUTONOMIC NERVOUS SYSTEM IN CHILDREN. H. K&r, H. P. Wagner, and F. Ku&r.
Helv. Paediat.
Acta 24:128,
1969.
In a case of neuroblastoma in a 1 2/12-year-old female infant reported here, the author found normal values for the urinary output of vanillylmandelic acid and of homovanillic acid, but the excretion of vanillyllactic acid was exceedingly increased. In a series of 142 neuroblastomas and ganglioneuroblastomas, they found 13 other cases without increased excretion of VMA and HVA: but in all these 13 cases, dopamin, 3-methoxytyramine, normetanephrine, 3-methoxytyrosine, and/or vanillyllactic acid were increased in the urine. It is thus very important in order to further improve the diagnostic reliability of quantitative determination of catecholamine catabolites in tumors of the autonomic nervous system, to investigate not only the urinary output of VMA and HVA, hut of all the catecholamine catabolites mention above.M. Bettex.