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We have presented some of the nutritional complications encountered in two major pediatric congenital disorders. Although these conditions represent two more common major defects, it is unlikely that many health care providers will manage large numbers of these patients. Nevertheless, the nutrition principles apply to other nutritional dilemmas of chronically ill children. When an infant consumes a low volume intake, regardless of etiology, concerns such as provision of adequate nutrition, within the confines of the infant's water balance, become paramount. Methods have been discussed for increasing caloric density and for monitoring dietary safety and adequacy. When an infant has a propensity for becoming obese one needs to consider preventive measures such as providing sound nutrition information, support, and follow-up for both patient and family. Nutritional problems can become magnified unless adequate support is provided for total health and social needs of the family. The role of the dietitian must be one active participation within the the framework of an interdisciplianry team so that appropriate innovative nutrition programs can be developed and implemented.

The appropriate use of spinal orthosis is an important adjunct in the rehabilitation of spinal deformity in children with myelomeningocele. The Newington developmental programme described here employs a series of orthotic devices, used at different stages of development, with the aim of achieving a developmental sequence as close as possible to the usual age for normal development.

Treatment of newborns with myelodysplasia (meningomyelocele and related disorders) continues to be a controversial subject. We have used a consistent plan of care and have employed the same prognostic criteria over the period from 1965 to 1982 to address the needs of 212 affected newborns. A good prognosis and early surgical care were given to 42 per cent of 53 newborns during the period 1965 to 1970, to 58 per cent of 65 newborns from 1971 to 1976, and to 71 per cent of 94 newborns from 1977 to 1982. Of the newborns with an initially poor prognosis, 19 per cent of 31 received early surgery between 1965 and 1970, as compared with 33 per cent of 27 between 1971 and 1976 and 52 per cent of 27 between 1977 and 1982. Life-table analyses of survival in the three periods revealed significant improvement over time in the survival of newborns receiving early surgical care, regardless of the initial prognosis (log-rank statistic = 8.240, P = 0.016) and in comparison to recipients of supportive care alone (log-rank statistic = 5.975, P = 0.05). We conclude that early surgery permits the survival of an increasing percentage of patients with myelodysplasia.

Eight children with myelomeningocele and chronic neurogenic urinary incontinence were provided urodynamic biofeedback training. During urodynamic biofeedback, six of the eight children demonstrated improved self-regulation of detrusor and/or sphincter functioning. However, substantial improvements in clinical symptomatology (i.e., urinary incontinence) were clearly shown by only one child. Unexpectedly, chronic neurogenic fecal incontinence was reduced in four children. Several methodological modifications are discussed which may improve clinical symptomatology and which may facilitate further urodynamic biofeedback research for these children with congenital neurogenic urinary incontinence.

The results in 100 patients of intravesical transurethral electrotherapy in the treatment of bladder paresis and micturition defects due to meningomyelocele are reported. Positive results were attained in 71 patients. The method and the physiological development of controlled micturition are discussed.

The results of selective treatment in 120 infants with open spina bifida, admitted between May 1971 and December 1976, were prospectively studied. Seventy-one infants had adverse criteria at birth and were not treated. They all died, more than 90% of them within 6 months of birth. Seven had meningocele. All were treated and survived without handicap. Forty-two infants with myelomeningocele were actively treated. Thirty-six survive at follow-up after 3 to 9 years. The quality of survival is much better than when selection was not used but 8 children have moderate or severe handicaps. The parents were fully informed and consulted at every decision-making step; they fully supported the principle of selection and the action taken on behalf of their own child.

A case of myelomeningocele in a 20-year-old woman is presented. The partially healed scar continued to produce infected debris despite vigorous local therapy. She died because of eventual local and retroperitoneal invasion of squamous cell carcinoma. The possible benefits of decisive planning are discussed.

Genetic emergencies like other medical emergencies require immediate intervention to alter the outcome of the condition in the patient and its effect upon the family. The representative conditions of myelomeningocele, Down's syndrome, cleft lip and palate and adrenogenital syndrome serve to illustrate the need for rapid and comprehensive intervention. Recognition that an emergency exists when a child with a birth defect is born and responsive thoughtful action including family counseling can significantly alter the life of the patient and his family.

This article presents a definition of the child with a myelomeningocele, a description of a myelomeningocele clinic setting, an outline of the particular needs and concerns of various age groups of children with myelomeningocele, and a summary of occupational therapy's role in the clinic. This paper is an attempt to fill a gap in the literature on the occupational therapist's role in the treatment of the child with myelomeningocele.

Mentor electronic bladder stimulators were implanted in 8 children with myelomeningocele who were being managed successfully with intermittent catheterization. Satisfactory clinical performance was obtained in only 3 of these children. In the 5 failures urine flow was sporadic and unreliable. In 6 patients electrode lead wires broke where they entered the anterior abdominal fascia. None of the 8 units functioned more than 29 months. Many technical problems must be resolved before bladder stimulators will have a significant role in the management of children with myelomeningocele.

Within the last generation the application of major advances in drug therapy, intensive care, transfusion techniques, surgery, anaesthesia, and radiotherapy, together with a vast expansion of knowledge due to increased investigative facilities, have led to an unprecedented, dramatic and beneficial increase in the number of persons who would previously have died, or lived with severe handicaps, but can now be cured. Techniques have also been developed to prolong the lives of many people who are now able to enjoy several extra years of productive and meaningful existence, and to alleviate and improve the quality of life of many seriously handicapped persons, enabling them to become integrated as useful and contented members of the community. Choosing from numerous examples of paediatric experience, it is notable that many more extremely premature infants now survive without physical or intellectual damage; infection can almost always be cured, including neonatal and all other forms of pyogenic meningitis and the now rare cases of tuberculous meningitis and miliary tuberculosis. The few remaining new cases of Rh-haemolytic disease are also readily cured. There are outstanding successes in the treatment of childhood malignancy. Paediatric surgery has made great strides. The prognosis of congenital heart disease, of obstructions of the alimentary canal and many other conditions has improved beyond recognition. Unfortunately, the indiscriminate use of advanced techniques of all types has also kept alive those who would have died but now live with distressing physical or mental handicaps or both, often for many years, without hope of ever having an independent existence compatible with human dignity. There are many examples, including those who have sustained major brain or spinal cord injuries.

At Our Lady's Hospital for Sick Children, Crumlin, the policy towards neonates with Spina Bifida is one of individualised care. This means that those children who, experience has shown, will not be long term survivors are not subjected to procedures that are distressing and traumatic to the infant and parents. They are given full nursery care. A small number of children treated on this basis thrive. The question arises whether or not they have been further handicapped by this approach. Since this is a problem which causes concern to both parents and clinicians, this study reviews the present state of these survivors. In the five year period 1979-1983, there was a total of 192 infants admitted to Our Lady's Hospital with open myelomeningocoele. 66 were treated with early surgical intervention. 75 died without any surgical procedure at a mean of 46 days. 51 children underwent late intervention, despite which 29 died. A group of 22 are alive, aged 3-7 years. The mean first admission of these 22 patients was 77 days and was uneventful. However, all 22 are severely handicapped; all are paraplegic, doubly incontinent and have some degree of mental handicap. All have hydrocephalus graded moderate to gross and 17 have been shunted. Amongst these 22 surviving patients, we have been unable to identify, in the initial period, specific events or developments which have clearly caused additional damage to the infant's intellectual or physical well-being.

Since 1976 we have implanted bilateral diaphragm pacers in 34 infants and children: 26 with central hypoventilation syndrome (CHS), three with myelomeningocele, and five with quadriplegia. Compared to adults, several modifications have been necessary to achieve effective ventilation in infants and younger children. In all instances, a tracheostomy has been necessary due to impaired neuromuscular control of upper airway patency during pacing. Bilateral pacing has been necessary to achieve adequate ventilation; in the CHS children with normal awake ventilation, bilateral pacing during sleep eliminates the need for positive pressure ventilation. For the remaining children, adequate awake ventilation is achieved with bilateral pacing, thus permitting substantially greater mobility and limiting use of the ventilator to sleep time. Our longest survivor has now been paced for 10.7 years, and in no instance has phrenic nerve damage occurred secondary to electrical stimulation. Our current pacing regime is characterized by moderate respiratory rates (21 breaths/min), long interpulse intervals (95 ms), and short inspiratory times (0.6 sec), resulting in 50%-75% fewer stimuli/min compared to our previous regime. For all infants and children requiring 24-hour ventilatory assistance, our recent successes in maintaining ventilation using significantly fewer stimuli suggest that long-term continuous pacing is a realistic future goal.

We report on a boy with myelomeningocele and a ventriculo-peritoneal shunt, who presented with progressive ureteropelvic junction obstruction and an enlarging right upper quadrant mass. Exploration revealed a large pseudocyst around the distal portion of the shunt, causing obstruction of an unsuspected retrocaval ureter. To our knowledge this is the first reported case of an upper urinary tract complication due to a ventriculo-peritoneal shunt as well as the first case of a retrocaval ureter associated with myelomeningocele.

We have made landmark strides over the past fifteen years in the medical management of infants and young children with spina bifida. Educational mainstreaming is national policy, and barrier-free physical environments are increasingly more common. Now we must direct our attention to the broader health and social needs of youth with spina bifida and their families, particularly in the areas of professional support and sexual and vocational counseling.

The life expectation of babies with paralytic lumbar meningomyelocele not offered immediate surgery at birth appears to be influenced by the extent to which parents are involved in the child's early care. 8 of the 27 children offered family-centred care in one hospital in 1971-80 and not offered immediate surgery survived to school entry and none of these children has since died. All are chairbound and incontinent, but none is intellectually retarded and many are no more handicapped than the children offered immediate surgical treatment at birth. The choice before the family at birth does not have to be presented as an urgent and immediate choice between life and death.

A comparison in terms of renal preservation as evidenced by urography is made between children with meningomyelocele on intermittent catheterization and children who had undergone diversion. Followup in the former group is only 2 1/2 years but at this time those patients on catheterization are doing as well and probably better than children who had undergone diversion and the quality of life for them is better.

Since 1974, 56 children with a neuropathic bladder were selected for intermittent or indwelling catheterisation. Indications for catheterisation included incontinence, hydronephrosis, and/or difficulty with bladder expression. Good results were obtained in 70% of girls and 50% of boys. The catheter was abandoned in 7 children, mainly because of lack of cooperation from the parents. Deterioration occurred only in those children with both hydronephrosis and vesico-ureteric reflux prior to catheterisation. Of the latter group of children, 33% suffered upper tract deterioration, and we now consider vesico-ureteric reflux to be a relative contra-indication to catheterisation, except in those children with severe disabilities and deformities, in whom antireflux surgery or urinary diversion would be unacceptable or technically difficult. Indwelling penile catheters should not be recommended for boys, but catheterisation via a perineal urethrostomy may prove helpful in those unable either to catheterise themselves intermittently or to wear a penile appliance.

Improvements in technology have dramatically increased the survival of children with spinal dysraphism. Because this complex condition affects multiple organ systems as well as the psychosocial functioning of the child and family, these children require care from a host of specialists in order to achieve optimum functioning. This article reviews the pathophysiology and discusses the current management of the medical and psychosocial effects of spinal dysraphism. It also briefly discusses strategies for intervention, long-term outcomes, and some controversies regarding care and screening.

Failure of the neural tube to close in a fetus is one of the most devastating and yet common congenital malformations. Various organ systems including the skeletal, muscular, urologic, digestive, respiratory, skin and central nervous systems are involved. This involvement results in complicated and interrelated problems requiring services from the medical specialties of pediatrics, orthopedics, urology and neurosurgery; the allied health professions of physical therapy, occupational therapy, speech pathology, nutrition, social work and nursing; and the psychoeducational professions of education and psychology. This article attempts to summarize the information about the current needs and management techniques of children with meningomyelocele for the general pediatrician who is frequently called upon to play an important role in coordination and communication with the families and other professionals caring for these children.