TREATMENT Management and supervision of a child and family with a myelo- meningocele require a multidisciplinary team approach, including surgeons, physicians, and therapists, with one individual (often a pediatrician) acting as the advocate and coordinator of the treat- ment program. The news that a newborn child has a devastating condition such as myelomeningocele causes parents to feel con- siderable grief and anger. They need time to learn about the handicap and the associated complications and to refect on the various procedures and treatment plans. A knowledgeable indi- vidual in an unhurried and nonthreatening setting must give the parents the facts, along with general prognostic information and management strategies and timelines. If possible, discussions with other parents of children with NTDs are helpful in resolving important questions and issues. Surgery is often done within a day or so of birth but can be delayed for several days (except when there is a CSF leak) to allow the parents time to begin to adjust to the shock and to prepare for the multiple procedures and inevitable problems that lie ahead. Evaluation of other congenital anomalies and renal function can also be initiated before surgery. Most pediatric centers aggres- sively treat the majority of infants with myelomeningocele. After repair of a myelomeningocele, most infants require a shunting procedure for hydrocephalus. If symptoms or signs of hindbrain dysfunction appear, early surgical decompression of the posterior fossa is indicated. Clubfeet can require taping or casting, and dislocated hips can require operative procedures. Careful evaluation and reassessment of the genitourinary system are some of the most important components of the man- agement. Teaching the parents, and ultimately the patient, to regularly catheterize a neurogenic bladder is a crucial step in maintaining a low residual volume and bladder pressure that prevents urinary tract infections and refux leading to pyelone- phritis, hydronephrosis, and bladder damage. Latex-free cathe- ters and gloves must be used to prevent development of latex allergy. Periodic urine cultures and assessment of renal function, including serum electrolytes and creatinine as well as renal scans, vesiculourethrograms (VCUGs), renal ultrasonography, and cys- tometrograms (CMGs), are obtained according to the risk status and progress of the patient and the results of the physical exami- nation. This approach to urinary tract management has greatly reduced the need for urologic diversionary procedures and sig- nifcantly decreased the morbidity and mortality associated with progressive renal disease in these patients. Some children can become continent with surgical implantation of an artifcial urinary sphincter (these are used less often) or bladder augmenta- tion at a later age. Functional ambulation is the wish of each child and parent and may be possible, depending on the level of the lesion and on intact function of the iliopsoas muscles. Almost every child with a sacral or lumbosacral lesion obtains functional ambulation; approximately half the children with higher defects ambulate with the use of braces, other orthotic devices, and canes. Ambula- tion is often more diffcult as adolescence approaches and body mass increases. Deterioration of ambulatory function, particu- larly during earlier years, should prompt referral for evaluation of tethered spinal cord and other neurosurgical issues. In utero surgical closure of a spinal lesion has been successful in a few centers. Preliminary reports suggest a lower incidence of hindbrain abnormalities and hydrocephalus (fewer shunts) as well as improved motor outcomes. This suggests that the defects may be progressive in utero and that prenatal closure might prevent the development of further loss of function. In utero diagnosis is facilitated by maternal serum α-fetoprotein screening and by fetal ultrasonography
Posted on: Mon, 13 Oct 2014 22:56:09 +0000