Primary Care Needs of Children with Hydrocephalus
Children with hydrocephalus may have life-long special health needs. These needs may alter their primary care. It is important that caregivers understand hydrocephalus in order to provide primary health care to these children and their families.
Clinical Manifestations at Time of Diagnosis
Although the signs and symptoms of hydrocephalus may be somewhat varied by the specific cause of the condition, there are common clinical manifestations associated with increased intracranial pressure. If the accumulation of excessive cerebrospinal fluid (CSF) occurs slowly, the infant or young child may be asymptomatic until the hydrocephalus is quite advanced. Significant dilation of the ventricle may occur before abnormal head growth is apparent. Full or distended fontanels, frontal bossing, prominent scalp veins, vomiting, irritability, and even opisthotonic posturing may be observed before dramatic changes are noted in head circumference.
In the older child with fused cranial sutures, the development of hydrocephalus may result in the non-specific symptoms of headache, nausea, vomiting, and personality changes, including irritability, lethargy, and loss of interest in normal daily activities. Spasticity or ataxia of the lower extremities and urinary incontinence may occur. These children frequently complain of vision problems as increased intracranial pressures on the second, third, or sixth cranial nerves result in extraocular muscular paresis and papilledema. Alterations in growth, sexual development and fluid, and electrolyte imbalance may occur if increased pressure occurs at the site of the hypothalamus.
Currently, the only form of treatment for hydrocephalus requires brain surgery. The most common is the surgical implantation of a shunt system.
Intellectual function is difficult to predict early in the disease process. The cause of hydrocephalus appears to be the most important determining factor of intellectual function. Hydrocephalus that is not complex has a better cognitive prognosis than hydrocephalus associated with brain injury. In recent studies, two-thirds of children with hydrocephalus had normal or borderline normal intelligence. In children with Intelligence Quotient (IQ) scores above 70, performance IQ scores are lower than full-scale and verbal IQ’s. This discrepancy indicates a need for preschool and school counseling and testing to identify areas of learning disability.
Vision and Hydrocephalus
Some children and adults with hydrocephalus develop vision (eye) problems. In one small study, 80% of infants with hydrocephalus had problems with their vision (Persson et al., 2017). These problems can range from minor decreases in visual acuity to severe vision loss. Remember – it is critical to report any changes in vision to your doctor as a change may indicate treatment failure. There are other signs that a person could have hydrocephalus, such as headache or hearing a noise in the ears (whooshing). It would be important to mention these to the doctor if they are taking place. For more detailed information, visit Vision and Hydrocephalus.
According to one study, 25% of children diagnosed with hydrocephalus will have some trouble with motor function (Fernell et al., 1988). These can vary from severe paraplegia to mild imbalance or weakness. The severity of the motor deficit is most often diagnosis-related; children with conditions such as porencephaly, Dandy-Walker malformation, and the meningomyelocele, also commonly known as myelomeningocele, which is a type of spina bifida, have more serious motor deficits than children with simple congenital hydrocephalus.
Hydrocephalus also affects fine motor control. Kinesthetic-proprioceptive abilities of the hands are often affected negatively, and this, coupled with impaired bimanual manipulation and frequent visual deficits, may make it difficult for the child with hydrocephalus to perform well on time-limited nonverbal intelligence tests.
Epilepsy is common in people with hydrocephalus. Of individuals with shunted hydrocephalus, an estimated 20% have epilepsy (Klepper et al., 1998).
Past studies have shown:
- 4.5% of people with Normal Pressure Hydrocephalus have epilepsy (Larsson et al., 2017).
- 11% of children with spina bifida and hydrocephalus have epilepsy (Persson et al., 2006).
- Up to 35% of children with congenital hydrocephalus (diagnosed before or at birth) have epilepsy (Persson et al., 2006; Fernell et al., 1988).
- 44-70% of children with posthemorrhagic hydrocephalus of prematurity have epilepsy (Battaglia et al., 2005; Tully et al., 2016).
For more detailed information, visit Epilepsy and Hydrocephalus.
Primary Care Management
Growth & Development
Both precocious puberty and short stature have been reported in children with hydrocephalus (Hochhaus et al., 1997; Lopponen et al., 1996). Sexual development before the age of 8 in girls and 9 in boys is considered precocious and warrants further diagnostic study. Heights below the 5th percentile, if not compatible with family stature, indicate growth retardation. Treatment is available for both of these conditions, and children should be referred to an endocrinologist if symptoms occur.
In children suspected of having hydrocephalus, or who are known to have hydrocephalus, head circumference should be measured by experienced personnel. Until the cranial sutures are completely fused, which can be delayed in these children, the growth of head size is a major diagnostic tool in evaluating the child’s condition. Once a shunt has been placed, head circumference may decrease 1 to 2 cm as the pressure is relieved. After this initial decrease, the head should grow only in the proportion of the child’s body. The significance of head size measurements in the child who has a shunt cannot be overestimated, and daily measurements may be necessary when evaluating the shunt-dependent infant for possible shunt malfunction.
Standard early infant developmental screening assessment tools used in primary care practice, such as the Denver Developmental Screening Tool, may be of little help in assessing infants with hydrocephalus. It is important for the practitioner to interpret developmental findings in light of other clinical observations to assist the parents in developing reasonable expectations for their infant. The primary care provider must document carefully motor skill acquisition because a loss of skill may indicate shunt malfunction or progression of the primary cause of hydrocephalus. This applies to older school-aged children as well. Ataxia, slurred speech, lack of progression in school, or incontinence also may indicate a deterioration of neurological status and the need for further evaluation.
Often children with hydrocephalus will benefit from infant stimulation programs or physical therapy, and the practitioner must be familiar with the programs available in the family’s community to help them identify programs that would be most beneficial for their child.
Diphtheria, Tetanus, Pertussis (DPT) Pertussis can pose a special problem in infants with hydrocephalus. Because children, with a history of seizures, are at increased risk of seizures after pertussis vaccination deferral of the DPT immunization may be prudent until neurologic stability is ascertained. The risk of contracting pertussis is low but because neurologically impaired children may be at an increased risk or morbidity and mortality from illness caused by Bordetella pertussis, immunization is not absolutely contraindicated, and in some patients (such as those with well-controlled seizures, corrected hydrocephalus or cerebral palsy) will be indicated. The primary care provider will need to weigh the risk of disease versus the risk of side effects of the vaccination. In these difficult situations, consultation with the child’s neurosurgeon or neurologist may be advisable to help assess the child’s potential for having seizures.
Measles. Measles vaccine also has been implicated in post-vaccination seizures, with a higher incidence of this occurring in infants and children who have a history of convulsions. It is not believed these post-vaccine seizures produce permanent neurologic damage and the high ongoing risk of natural measles with its high morbidity rate justifies measles immunization in children with a personal of convulsions.
Haemophilus influenza type B. Conjugated polysaccharide-diphtheria Haemophilus influenza type B (HIB) vaccination is recommended at 18 months for all children. The use of HIB vaccine is variable but generally, this vaccine is given less often than other recommended vaccinations. Because of the increased risk of HIB CNS infections in children with shunts, children with hydrocephalus who have shunts should definitely receive the new conjugated vaccine as recommended. Children who have a history of documented HIB disease before 2 years of age may not produce adequate antibodies to prevent a second infection and therefore should be immunized.
Other immunizations. Vaccination for polio, mumps, and rubella should be given as routinely scheduled.
Because of the high incidence of visual defects in children with hydrocephalus, special attention should be paid to visual screening. Hirschberg, cover test, and funduscopic examinations should be performed during each office visit and the result carefully documented. At about six months of age, the child should be referred to a pediatric ophthalmologist for a thorough examination. Yearly examinations should be scheduled thereafter.
In addition to routine office screening for hearing acuity, infants with a history of CNS infection or antibiotic treatment with amino-glycosides should undergo auditory-evoked response testing. Subsequent shunt malfunctions or CNS infections require a reassessment of hearing, and periodic evaluation by an audiologist is recommended.
Routine dental care is advised. If the child is receiving phenytoin for seizure control, more frequent dental care may be needed because of the possibility of hyperplasia of the gums. If the child has a ventriculoatrial shunt, prophylaxis with penicillin is recommended for all dental work including adjustment of braces to prevent bacterial endocarditis.
Special Family Concerns
Parents of children with hydrocephalus constantly worry about continued shunt function. With every malfunction, there is a need for surgery and the perceived threat of brain damage. This constant worry and the daily responsibilities and stress of caring for a child who may have multiple medical problems is very difficult for families. The financial strain caused by numerous medical visits or surgical procedures may deplete a family’s financial reserve, and private insurance may not be obtainable unless it is offered through a large group employment policy. Concerns about the child’s ability to be self-supporting and independent in the future are also an issue for parents as the child grows into adolescence.
Depending on the child’s condition, the number of specialists and community resources used, and the family’s strengths or abilities, the parents may need assistance from the practitioner in case management. Coordination and communication between specialists, practitioners, family, and school personnel can often become complex and overwhelming to parents. The practitioner can function as a central clearinghouse to help the parents understand the advised medical treatments, to follow-up on necessary referrals, and to identify priorities for that family and child.
This article was produced by the Hydrocephalus Association, copyright © 2020. It was adapted from an article in the Journal of Pediatric Health Care written by Patricia Ludder Jackson, MS, RN, PNP, Program Coordinator of the Nursing Leadership in Pediatric Primary and Chronic Care Program at the University of California, San Francisco. It may be reproduced provided a full citation of sources is given.