Childs Nerv Syst DOI 10.1007/s00381-013-2335-x
ORIGINAL PAPER
Factors affecting quality of life in early childhood in patients with congenital hydrocephalus Nitin James Peters & J. K. Mahajan & Monika Bawa & Pardeep Kumar Sahu & Katragadda L. N. Rao
Received: 18 October 2013 / Accepted: 27 November 2013 # Springer-Verlag Berlin Heidelberg 2013
Abstract Background Predicting the outcome of congenital hydrocephalus in early infancy and childhood is difficult. Various consequences of hydrocephalus like ophthalmic, audiometric, musculoskeletal, and the developmental abnormalities play a complex role. We analyzed the quality of life of these patients in early life. Methods A prospective multispecialty assessment of the patients with congenital hydrocephalus, who had undergone ventriculoperitoneal shunt in the Pediatric Surgery unit of a tertiary care hospital, was carried out and analyzed. Results There were 24 boys and 6 girls. Eighty-three percent were operated before the age of 3 months. Mean age at follow up was 6 years (3–9 years). Shunt complications were seen in 53 % (16) of the patients, out of which 68.75 % had shunt blocks. In 70 % (21) of the patients, the ventricle to hemisphere ratio (VHR) was between 51 and 70 %, 20 % (6) had a VHR of more than 70 % and only 3 patients had a VHR between 40–50 %. Audiological and ophthalmic problems were seen in 20 and 66 % of the children, respectively. Musculoskeletal abnormalities were present in 36 % of the patients. Developmental profile showed 44 % being normal, 30 % retarded, and 26 % were borderline cases. The schooling had started in 56.6 %, out of which only one child went to a special school. Conclusions A multitude of problems associated with congenital hydrocephalus should be diligently diagnosed, aggressively followed and intervened with in the early years of life.
Preschool management well before the peer interaction should optimize the social integration and improve the quality of life in these patients. Keywords Hydrocephalus . Ventriculoperitoneal shunt . Quality of life . Congenital
Introduction Patients of congenital hydrocephalus, who have undergone shunt surgery in the neonatal period require regular monitoring and follow up [1]. The prognosis in these patients is difficult to predict and is further complicated by the presence of associated disorders as well as the complications of the shunt procedure. For socially relevant existence of these individuals, their assessment should be done early in life so that interventions, if any, should be instituted before the commencement of the school. Despite ventriculoperitoneal shunt (VPS) insertion being a commonly performed procedure for congenital hydrocephalus, there is a surprising paucity of the studies, describing the quality of life (QOL) and the intellectual outcome in these patients in the preschool years of life. This study aims to determine the outcome of these individuals after VPS in the early years of life along with the factors affecting the parameters of QOL.
Materials and methods N. J. Peters : J. K. Mahajan : M. Bawa : P. K. Sahu : K. L. N. Rao Department of Paediatric Surgery, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India J. K. Mahajan (*) Department of Pediatric Surgery, Advanced Pediatric Centre, PGIMER, Chandigarh, India e-mail: [email protected]
This is an analysis of the case records of 30 patients, who had undergone a low-pressure Chhabra's VP shunt for congenital hydrocephalus in our Pediatric Surgery unit. These patients were called for follow up and evaluated prospectively for psychomotor, neurodevelopment, ophthalmological, and audiological status over a period of 1 year.
Childs Nerv Syst
The study included consecutive patients with isolated congenital hydrocephalus, who were at least 3 years of age at the time of assessment and had completed a minimum of 1 year following the insertion of the VP shunt. The patients of hydrocephalus secondary to neonatal ventricular hemorrhage, associated with spina bifida, incomplete case records, and having associated brain parenchymal abnormalities were excluded from the study. The study was approved by the institute ethics committee. Neurodevelopmental and psychomotor assessment All the patients were examined for the development and intelligence quotient according to the Developmental Profile II (Alpern et al.) [2]. The child's functioning was evaluated in one of the five key areas in this test, with main emphasis being on the academic age. The patient himself or a caregiver, in case of a younger patient, answered the test items.
The mean follow-up period was 5.6±2.32 years (range 1–8.9 years). Cranial ultrasound was done in all the patients and 25 patients underwent non-contrast computed tomography (NCCT) scans as well. Only three patients had a communicating hydrocephalus, whereas the remaining 27 (90 %) had an obstructive type of hydrocephalus. In 21 (70 %) patients, the ventricle to hemisphere ratio (VHR) was between 51 and 70 %, 6 (20 %) had a VHR of more than 70 %. Only three patients had a VHR between 40 and 50 %. Out of 30 patients, 16 (53 %) had complications in the follow-up (Table 1). All the patients with shunt block presented with the features of raised intracranial tension and were investigated with an NCCT scan of the cranium. Two patients were managed conservatively whereas nine patients underwent revision of the VP shunt which was performed on the left side. All the patients with shunt complications had an uneventful recovery. Musculoskeletal abnormalities
Ophthalmic assessment Visual acuity was assessed by means of the C-chart and the method of “fixing and following” of gaze for the illuminated objects. Further, retinoscopy and fundoscopy were performed for the refractive errors and the state of the disk. Due to the smaller age group, color vision and the field of vision were not tested. Audiological assessment The hearing screening was done using a standard pure tone or the play audiometry with Madsen OB-922 clinical audiometer. Impedance audiometry to rule out the middle ear problems was carried out using a Siemens SD-30 impedance audiometer. Further speech assessment was done for the patients, who had a poor performance in the audiometry. Statistical analysis Statistical analysis was done by descriptive analysis of the data. Data was entered and analyzed using software SPSS (ver. 12). The significance of the differences in percentage level was assessed using Chi Square and the ANOVA tests.
Eleven out of 30 patients (36 %) had variable degrees of muscle weakness of the lower limbs; however, the sensations were intact (Table 2). Late onset of the disease was associated with a higher incidence of musculoskeletal abnormalities (MSA). The incidence of MSA was 33.8, 22.2, and 66.6 %, respectively, when the diagnosis of hydrocephalus was made within first 3, 3 to 6, and later than 6 months of age (p =0.366). Patients with shunt complications had more abnormalities (37.8 %) than those without complications (28.5 %); however, the difference was not statistically significant. Neurodevelopment and psychomotor assessment Development Profile II assessment included parental observations as well as the patient's performance in certain activities and based on the development quotient (DQ) scores, the patients were further divided into three broad categories of retarded, borderline, and normal. Forty-four percent had normal scores, 30 % children were retarded, and 26 % of the patients were in the borderline category. A slightly higher DQ score was recorded in patients, who were diagnosed at an earlier age and had an early surgery. DQ Table 1 Complications of ventriculoperitoneal shunt
Results Out of the 30 patients, 24 (80 %) were males and 6 (20 %) were females. Eleven patients (36.6 %) underwent shunt surgery within 1 month of age, 14 (46.6 %) between 1 and 3 months, and 4 patients had VPS inserted after 3 months of age (mean 3 months). At the time of follow-up assessment, the age of the patients ranged from 3 to 9 years (mean 6 years).
No. of patients (n =16)
Percentage
Shunt block CSF leak from ventricular end Seizures CSF pseudocyst Shunt breakage and displacement Infection
11 (68.75 1 (6.25 1 (6.25 1 (6.25 1 (6.25 1 (6.25
%) %) %) %) %) %)
Childs Nerv Syst Table 2 Musculoskeletal abnormalities in patients of congenital hydrocephalus
Ophthalmological examination
Musculoskeletal deformity (n =11)
No. of patients (percentage)
Weakness in LL, Not able to walk Weakness in LL, walking with support Weakness in LL, waddling gait, but could walk independently*
5 (45.5 %) 3 (27.3 %) 2 (27.3 %)
Out of 30 patients, 10 (33.3 %) had an impaired visual acuity. Seven (70 %) of them had hyperopia and three (30 %) had myopia. Seventeen patients (56.6 %) had a normal visual acuity. Strabismus was present in eight patients (26.6 %), involvement being unilateral in seven of them. Esotropia was present in one patient. On fundoscopic examination, normal fundus was present in 26 (86.6 %) patients and two patients each, had an optic atrophy and disk edema. Ophthalmic abnormalities were higher amongst the patients who had an early onset of hydrocephalus (p values=0.200). Eighty-three percent of the patients with VHR more than 71 % had one or the other type of ophthalmic abnormalities (p =0.231). The significance of all the above-mentioned variables has been shown in Table 3.
*One patient had an associated varus deformity, LL-Lower limbs
score was higher in patients, whenever, the interval between the onset of disease and the surgery was less than 1 month (p = 0.295). VHR of less than 50 % was associated with a significantly better DQ score (p =0.035). Patients who had complications following shunt surgery had lower DQ scores; though the difference was not statistically significant.
School performances Audiometric and speech analysis Four patients (13 %) had mild bilateral low frequency loss of hearing. One (3.3 %) patient, each, had a mild high frequency loss of hearing bilaterally and mild low frequency loss unilaterally. Speech was uniformly appropriate for age for all the patients except one. Hearing loss was limited to the patients with early onset of hydrocephalus (p =0.088). VHR above 50 % seemed to be a consistent factor in patients with hearing loss (p =0.489). Significantly, all patients with hearing loss had one or other type of shunt complication (p =0.035).
Nine patients (30 %) have not started going to school due to the parental concern for the safety of the children. Four patients (13 %) never went to school, despite being old enough to attend school due to mental retardation and parental anxiety. Eight patients (26 %) were attending schools with normal children but were scoring lower grades as compared to their peers and were average in studies as per the parents. Another eight patients were attending schools with normal children and were also scoring good grades. Only one patient was attending a school for the special children.
Table 3 Relationship between study and outcome variables Study variables
Age of onset (months)
1–3 3–6 >6 Age at surgery (months) 0–6 6–12 >12 Interval between onset and surgery 3 VHR % 40–50 51–70 >70 Shunt complications Yes No
Total no. of Outcome variables patients (n) MSA no. of patients (%) DQ (mean) no. of patients (%)
18 9 3 21 8 1 11 14 5 3 21 6 16 14
p value
ORIGINAL PAPER
Factors affecting quality of life in early childhood in patients with congenital hydrocephalus Nitin James Peters & J. K. Mahajan & Monika Bawa & Pardeep Kumar Sahu & Katragadda L. N. Rao
Received: 18 October 2013 / Accepted: 27 November 2013 # Springer-Verlag Berlin Heidelberg 2013
Abstract Background Predicting the outcome of congenital hydrocephalus in early infancy and childhood is difficult. Various consequences of hydrocephalus like ophthalmic, audiometric, musculoskeletal, and the developmental abnormalities play a complex role. We analyzed the quality of life of these patients in early life. Methods A prospective multispecialty assessment of the patients with congenital hydrocephalus, who had undergone ventriculoperitoneal shunt in the Pediatric Surgery unit of a tertiary care hospital, was carried out and analyzed. Results There were 24 boys and 6 girls. Eighty-three percent were operated before the age of 3 months. Mean age at follow up was 6 years (3–9 years). Shunt complications were seen in 53 % (16) of the patients, out of which 68.75 % had shunt blocks. In 70 % (21) of the patients, the ventricle to hemisphere ratio (VHR) was between 51 and 70 %, 20 % (6) had a VHR of more than 70 % and only 3 patients had a VHR between 40–50 %. Audiological and ophthalmic problems were seen in 20 and 66 % of the children, respectively. Musculoskeletal abnormalities were present in 36 % of the patients. Developmental profile showed 44 % being normal, 30 % retarded, and 26 % were borderline cases. The schooling had started in 56.6 %, out of which only one child went to a special school. Conclusions A multitude of problems associated with congenital hydrocephalus should be diligently diagnosed, aggressively followed and intervened with in the early years of life.
Preschool management well before the peer interaction should optimize the social integration and improve the quality of life in these patients. Keywords Hydrocephalus . Ventriculoperitoneal shunt . Quality of life . Congenital
Introduction Patients of congenital hydrocephalus, who have undergone shunt surgery in the neonatal period require regular monitoring and follow up [1]. The prognosis in these patients is difficult to predict and is further complicated by the presence of associated disorders as well as the complications of the shunt procedure. For socially relevant existence of these individuals, their assessment should be done early in life so that interventions, if any, should be instituted before the commencement of the school. Despite ventriculoperitoneal shunt (VPS) insertion being a commonly performed procedure for congenital hydrocephalus, there is a surprising paucity of the studies, describing the quality of life (QOL) and the intellectual outcome in these patients in the preschool years of life. This study aims to determine the outcome of these individuals after VPS in the early years of life along with the factors affecting the parameters of QOL.
Materials and methods N. J. Peters : J. K. Mahajan : M. Bawa : P. K. Sahu : K. L. N. Rao Department of Paediatric Surgery, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India J. K. Mahajan (*) Department of Pediatric Surgery, Advanced Pediatric Centre, PGIMER, Chandigarh, India e-mail: [email protected]
This is an analysis of the case records of 30 patients, who had undergone a low-pressure Chhabra's VP shunt for congenital hydrocephalus in our Pediatric Surgery unit. These patients were called for follow up and evaluated prospectively for psychomotor, neurodevelopment, ophthalmological, and audiological status over a period of 1 year.
Childs Nerv Syst
The study included consecutive patients with isolated congenital hydrocephalus, who were at least 3 years of age at the time of assessment and had completed a minimum of 1 year following the insertion of the VP shunt. The patients of hydrocephalus secondary to neonatal ventricular hemorrhage, associated with spina bifida, incomplete case records, and having associated brain parenchymal abnormalities were excluded from the study. The study was approved by the institute ethics committee. Neurodevelopmental and psychomotor assessment All the patients were examined for the development and intelligence quotient according to the Developmental Profile II (Alpern et al.) [2]. The child's functioning was evaluated in one of the five key areas in this test, with main emphasis being on the academic age. The patient himself or a caregiver, in case of a younger patient, answered the test items.
The mean follow-up period was 5.6±2.32 years (range 1–8.9 years). Cranial ultrasound was done in all the patients and 25 patients underwent non-contrast computed tomography (NCCT) scans as well. Only three patients had a communicating hydrocephalus, whereas the remaining 27 (90 %) had an obstructive type of hydrocephalus. In 21 (70 %) patients, the ventricle to hemisphere ratio (VHR) was between 51 and 70 %, 6 (20 %) had a VHR of more than 70 %. Only three patients had a VHR between 40 and 50 %. Out of 30 patients, 16 (53 %) had complications in the follow-up (Table 1). All the patients with shunt block presented with the features of raised intracranial tension and were investigated with an NCCT scan of the cranium. Two patients were managed conservatively whereas nine patients underwent revision of the VP shunt which was performed on the left side. All the patients with shunt complications had an uneventful recovery. Musculoskeletal abnormalities
Ophthalmic assessment Visual acuity was assessed by means of the C-chart and the method of “fixing and following” of gaze for the illuminated objects. Further, retinoscopy and fundoscopy were performed for the refractive errors and the state of the disk. Due to the smaller age group, color vision and the field of vision were not tested. Audiological assessment The hearing screening was done using a standard pure tone or the play audiometry with Madsen OB-922 clinical audiometer. Impedance audiometry to rule out the middle ear problems was carried out using a Siemens SD-30 impedance audiometer. Further speech assessment was done for the patients, who had a poor performance in the audiometry. Statistical analysis Statistical analysis was done by descriptive analysis of the data. Data was entered and analyzed using software SPSS (ver. 12). The significance of the differences in percentage level was assessed using Chi Square and the ANOVA tests.
Eleven out of 30 patients (36 %) had variable degrees of muscle weakness of the lower limbs; however, the sensations were intact (Table 2). Late onset of the disease was associated with a higher incidence of musculoskeletal abnormalities (MSA). The incidence of MSA was 33.8, 22.2, and 66.6 %, respectively, when the diagnosis of hydrocephalus was made within first 3, 3 to 6, and later than 6 months of age (p =0.366). Patients with shunt complications had more abnormalities (37.8 %) than those without complications (28.5 %); however, the difference was not statistically significant. Neurodevelopment and psychomotor assessment Development Profile II assessment included parental observations as well as the patient's performance in certain activities and based on the development quotient (DQ) scores, the patients were further divided into three broad categories of retarded, borderline, and normal. Forty-four percent had normal scores, 30 % children were retarded, and 26 % of the patients were in the borderline category. A slightly higher DQ score was recorded in patients, who were diagnosed at an earlier age and had an early surgery. DQ Table 1 Complications of ventriculoperitoneal shunt
Results Out of the 30 patients, 24 (80 %) were males and 6 (20 %) were females. Eleven patients (36.6 %) underwent shunt surgery within 1 month of age, 14 (46.6 %) between 1 and 3 months, and 4 patients had VPS inserted after 3 months of age (mean 3 months). At the time of follow-up assessment, the age of the patients ranged from 3 to 9 years (mean 6 years).
No. of patients (n =16)
Percentage
Shunt block CSF leak from ventricular end Seizures CSF pseudocyst Shunt breakage and displacement Infection
11 (68.75 1 (6.25 1 (6.25 1 (6.25 1 (6.25 1 (6.25
%) %) %) %) %) %)
Childs Nerv Syst Table 2 Musculoskeletal abnormalities in patients of congenital hydrocephalus
Ophthalmological examination
Musculoskeletal deformity (n =11)
No. of patients (percentage)
Weakness in LL, Not able to walk Weakness in LL, walking with support Weakness in LL, waddling gait, but could walk independently*
5 (45.5 %) 3 (27.3 %) 2 (27.3 %)
Out of 30 patients, 10 (33.3 %) had an impaired visual acuity. Seven (70 %) of them had hyperopia and three (30 %) had myopia. Seventeen patients (56.6 %) had a normal visual acuity. Strabismus was present in eight patients (26.6 %), involvement being unilateral in seven of them. Esotropia was present in one patient. On fundoscopic examination, normal fundus was present in 26 (86.6 %) patients and two patients each, had an optic atrophy and disk edema. Ophthalmic abnormalities were higher amongst the patients who had an early onset of hydrocephalus (p values=0.200). Eighty-three percent of the patients with VHR more than 71 % had one or the other type of ophthalmic abnormalities (p =0.231). The significance of all the above-mentioned variables has been shown in Table 3.
*One patient had an associated varus deformity, LL-Lower limbs
score was higher in patients, whenever, the interval between the onset of disease and the surgery was less than 1 month (p = 0.295). VHR of less than 50 % was associated with a significantly better DQ score (p =0.035). Patients who had complications following shunt surgery had lower DQ scores; though the difference was not statistically significant.
School performances Audiometric and speech analysis Four patients (13 %) had mild bilateral low frequency loss of hearing. One (3.3 %) patient, each, had a mild high frequency loss of hearing bilaterally and mild low frequency loss unilaterally. Speech was uniformly appropriate for age for all the patients except one. Hearing loss was limited to the patients with early onset of hydrocephalus (p =0.088). VHR above 50 % seemed to be a consistent factor in patients with hearing loss (p =0.489). Significantly, all patients with hearing loss had one or other type of shunt complication (p =0.035).
Nine patients (30 %) have not started going to school due to the parental concern for the safety of the children. Four patients (13 %) never went to school, despite being old enough to attend school due to mental retardation and parental anxiety. Eight patients (26 %) were attending schools with normal children but were scoring lower grades as compared to their peers and were average in studies as per the parents. Another eight patients were attending schools with normal children and were also scoring good grades. Only one patient was attending a school for the special children.
Table 3 Relationship between study and outcome variables Study variables
Age of onset (months)
1–3 3–6 >6 Age at surgery (months) 0–6 6–12 >12 Interval between onset and surgery 3 VHR % 40–50 51–70 >70 Shunt complications Yes No
Total no. of Outcome variables patients (n) MSA no. of patients (%) DQ (mean) no. of patients (%)
18 9 3 21 8 1 11 14 5 3 21 6 16 14
p value
