Lipodermoid: A case report

Lipodermoids are epibulbar, developmental growths of normal adipose in an abnormal site, ie. near the lacrimal gland and extending between the superior rectus and lateral rectus muscles posteriorly. Differentiation of benign lipodermoids from non-benign lesions is essential. When other ocular anomalies or systemic conditions are found in conjunction with lipodermoids then Goldenhar-Gorlin syndrome must be considered. This paper presents a case of unilateral lipodermoid and scoliosis in an otherwise healthy adult male.     

Frontonasal tumors: their diagnosis and management.

Fifty-three patients with frontonasal tumors were seen in a 17 -year period. Of these patients, 33 were females (62 percent) and 50 were caucasians (94 percent). The correct diagnosis was made preoperatively in 87 percent of the cases. Eighty-five percent of the patients were children, and most of the lesions (83 percent) were developmental anomalies rather than true neoplasms. The most common lesions were dermoids (17), hemangiomas (16), and encephaloceles (9), but 13 different types of lesions were encountered. The treatment of all is surgical excision, and a cooperative effort by the neurosurgeon and the plastic surgeon is required in some cases.     

Oculo-auriculo-vertebral (Goldenhar) spectrum associated with pericentric inversion 9: coincidental findings or etiologic factor?

Oculo-auriculo-vertebral (OAV) spectrum or Goldenhar syndrome is a complex and heterogeneous condition characterized by hemifacial microsomia (unilateral ear abnormalities and ipsilateral mandibular hypoplasia) as well as vertebral anomalies and epibulbar dermoids or lipodermoids. Although most cases of OAV spectrum are sporadic, both autosomal recessive and autosomal dominant inheritance have been reported. Furthermore, the association of OAV spectrum with different types of chromosomal abnormalities has been described. We present a premature newborn delivered after 36 weeks of gestation, whose birth weight was 2,100 g, birth length 43 cm and head circumference 32.5 cm. OAV spectrum with associated axial skeleton anomalies, eventration of the right hemidiaphragm, accessory spleen, unlobulated right lung, agenesis of right kidney, right ovary and right uterine horn, and partial agenesis of corpus callosum were found. She was the second child of unrelated parents, who have a healthy boy. Both parents refused chromosomal analysis of their peripheral blood. Trypsin G-banding and C-banding chromosomal analysis of the patient's peripheral blood revealed a pericentric inversion of chromosome 9 with break points at p11 and q13. This may be a coincidental or causal finding.     

A multidisciplinary approach to the problems of the multihandicapped blind child

A multidisciplinary team is described which was developed for dealing with the complex problems of the visually handicapped child in British Columbia. One hundred blind children were studied. Optic atrophy was the most frequent type of ocular pathology, followed by cataracts and retinopathy of prematurity. The most common etiological basis of blindness was genetic followed by unknown, infective and due to excessive neonatal oxygen. Seventy-seven percent of the patients had one to seven additional disabilities. The role of the physician in the handling of blind children and the need for him to be increasingly exposed to the chronically handicapped in his training are emphasized.     

The value of tear film breakup and Schirmer's tests in preoperative blepharoplasty evaluation

The results of tear film breakup (BUT) and Schirmer's I and II tests were retrospectively analyzed on 146 patients undergoing elective blepharoplasty over a 41-month period. These tests were evaluated in conjunction with ocular history, orbital and periorbital anatomy, and Bell's phenomenon in order to determine their value, if any, in identifying patients at risk of developing a post-blepharoplasty dry eye complication. One-hundred and six patients (73 percent) had test results that were within normal limits, and two of these patients (1.9 percent) complained postoperatively of a transient gritty or burning sensation. Forty patients (27 percent) had abnormal results to one, two, or all three tests, and two of these patients (5 percent) also complained postoperatively of a transient gritty or burning sensation. These four symptomatic patients all had preoperative dry eye histories and abnormal orbital and periorbital anatomy. When analyzed alone, an abnormal tear film breakup (BUT) or Schirmer's test was not a good predictor of possible postblepharoplasty dry eye complications. An abnormal preoperative ocular history or abnormal orbital and periorbital anatomy proved to be the best predictor for the possible development of a postblepharoplasty dry eye complication.     

Do Children Lose and Maintain Weight Easier Than Adults: A Comparison of Child and Parent Weight Changes From Six Months to Ten Years

Recent research has shown weight maintenance for obese preadolescent children over 60 and 120 month intervals, while studies on adults consistently show they fail to maintain weight loss. This paper is designed to examine differences in percent overweight changes at 6, 60 and 120 months in obese parents and children from 113 families who participated in randomized controlled outcome studies evaluating family-based behavioral treatment. Analyses showed children had significantly greater changes in percent overweight than their parents at each time point. Chi-Square and Logistic Regression analyses showed children were more likely than their parents at each time point to have percent overweight decreases greater than 20%, with over 20% of the children and less than 1% of the parents showing changes this large. The implications of these results for weight control are discussed. These results suggest there may be differences in the efficacy of treating obesity in children versus adults.     

Are children with clefts underweight for age at the time of primary surgery?

Children with clefts, especially those with a cleft palate, have an impaired sucking mechanism and are therefore prone to nutritional problems. This study was undertaken to determine whether children with clefts of the lip and/or palate are underweight for age at the time of primary surgery. Underweight for age was defined as being less than 80 percent of expected weight for age or below the 3rd percentile as plotted on standard percentile charts. The records of all children with clefts seen at the Red Cross Children's Hospital between 1976 and 1996 were reviewed. Of these 740 records, 100 were excluded for inadequate data (47), severe systemic syndrome (27), no operation done (22), or craniofacial cleft (4). The records of 640 children were thus included; 195 (30.5 percent) were underweight for age. By comparison, only 13.7 percent of a similar group of noncleft controls (n = 872) were underweight for age. The difference between these two groups was highly significant (p < 0.01). Factors that influenced weight at the time of primary surgery were type of cleft and age at the time of surgery. Children with cleft palate, whether associated with a cleft lip or not, were found to be more underweight for age than those with an isolated unilateral cleft lip (p = 0.008). Children who had surgery after the age of 1 year were 1.5 times more likely to be underweight for age than children who had surgery under 1 year of age (p < 0.01). Children with isolated cleft palates who were underweight for age had a tendency toward a higher fistula rate (36 percent) than those of normal weight (24 percent) (p = 0.18).     

The ophthalmic anomalies in children with Down syndrome in Split-Dalmatian County

Our aim was to present the ophthalmic anomalies in patients with Down syndrome in Split-Dalmatia County born from 1992 until 2009 year. It was a cross-sectional study. 153 children with Down syndrome aged 0-18 years from the Split-Dalmatia County were examined. One hundred twelve participants were borne in Split, 13 in Vrgorac,16 in Makarska, 12 in Sinj. All enrolled children underwent a complete ophthalmological examination (anterior segment, ocular motility, refractive status, fundus, measuring intraocular pressure (IOP). Of 89.5% percent of responders with refractive errors, 48.1% had myopia, 35.0% had hypermetropia, astygamtism in 16.7%, 28.7% strabismus, nystagmus (8.4%), cataracts (1.3%), glaucoma (1.9%), supernumerary optic disc vessels (24.1%) and keratoconus (1.3%). Conclusion: In patients with Down syndrome the prevalence of refractive errors (myopia prevalence), as well as other ophthalmological diseases was determined.     

Part I – Young Girls with Non-Refluxing Ureters

This paper is the study of 144 girls with histories of urinary tract infection followed at the Stanford Medical Center who were found to be free of ureterovesical reflux. The mean age at onset of infection for the entire group was about four years and ranged from the first few months of life to age 10. Ninety-two percent of the 505 infection episodes in these children presented with symptoms referable to the lower tract, and bacterial localization studies confirmed that 85 percent of the infections were limited to the bladder. Escherichia coli was the most common organism isolated and most infections were caused by a pure culture of a single bacteria.In only two of the 144 patients studied was there any evidence of upper tract damage related to infection. The possibility that these patients had reflux at an earlier age could not be discounted.In response to short-term antibacterial therapy in 66 of the patients followed closely for an average of 40 months each, 20 percent of the patients had no further infections and 80 percent went on to recurrence. With each succeeding treatment an additional 20 percent of the patients were “cured,” but the remainder experienced recurrent infections during the follow-up. This reinfection pattern supports the use of long-term antibacterial prophylaxis in all girls who have more than three or four recurrences of infections. Urethral dilation appeared to have no value in reducing the reinfection rate. While it appears that in the absence of ureterovesical reflux few, if any, of these children will go on to develop upper tract damage, long-term prophylactic suppressive medication can clearly be justified on the grounds of reducing patient morbidity.     

Surgical management of parotid hemangioma

Hemangiomas represent one of the most common childhood neoplasms. They are often managed conservatively, requiring numerous years for spontaneous involution. No effective medical treatment has been reported for children with large, deforming hemangiomas of the parotid gland and overlying cheek. The authors retrospectively studied 17 children who underwent surgical resection of parotid hemangiomas at Childrens Hospital Los Angeles from 1997 to 2003. All 17 patients had improvements in facial asymmetry and deformity. There were no major complications. Minor complications included hematoma (11.8 percent), transient facial nerve palsy (11.8 percent), and blood transfusion (5.9 percent). All operations were performed on an outpatient basis. Surgical resection of parotid hemangiomas provides an aesthetic benefit to young children with low associated morbidity. Early resection by an experienced surgeon should be considered as a treatment option for these disfiguring lesions.     

Pure orbital blowout fracture: new concepts and importance of medial orbital blowout fracture.

Pure orbital blowout fracture first occurs at the weakest point of the orbital wall. Although the medial orbital wall theoretically should be involved more frequently than the orbital floor, the orbital floor has been reported as the most common site of pure orbital blowout fractures. A total of 82 orbits in 76 patients with pure orbital blowout fracture were evaluated with computed tomographic scans taken on all patients with any suspicious clinical evidence, including nasal fracture. Isolated medial wall fracture was most common (55 percent), followed by medial and inferior wall fracture (27 percent). The most common facial fracture associated with medial wall fracture was nasal fracture (51 percent), not inferior wall fracture (33 percent). This finding suggests that the force causing nasal fracture is an important causative factor of pure medial wall fracture as the buckling force from the medial orbital rim. Of patients with medial wall fractures, 25 percent had diplopia and 40 percent had enophthalmos. On plain radiographs, diagnostic signs were found in 79 percent of medial wall fractures and in 95 percent of inferior wall fractures. On computed tomographic scans, late enophthalmos was expected in 76 percent of medial wall fractures. Therefore, the medial orbital blowout fracture may be an important cause of late enophthalmos, because it has a high incidence of occurrence, a low diagnostic rate, and a high severity of defect. Among the causes of limitation of ocular motility, muscle traction of the connective septa and direct muscle injury were found frequently, but true incarceration of the muscle was extremely rare in all fractures. The medial and inferior orbital walls are clearly demarcated by the bony buttress, which is an important structure supporting these orbital walls. Its buttress was closely correlated with the fracture of these orbital walls. Most orbital blowout fractures without collapse of the bony buttress had a trapdoor fracture with or without small fragments of punched-out fracture.     

Vision screening at 8 and 18 months. Steering Committee of Oxford Region Child Development Project.

OBJECTIVE--To determine the effectiveness of an existing screening programme based in the community for ocular and vision defects in infants considered at increased risk of such defects. DESIGN--Children with ocular or vision defect by the age of 2 were ascertained by searching records. Those from populations at high risk were matched with their results from screening tests. The characteristics of the cases among this population were compared with those of the cases in the remainder of the population. Patterns of referral and age at referral were studied in both groups. SETTING--The study was conducted within Oxfordshire Health District. SUBJECTS--433 Children at high risk born in 1984 to mothers living in the health district at delivery and who either weighed less than 2000 g or weighed 2000 g and over and required admission to a special care nursery for longer than 24 hours. The low risk population (6254) were infants without these characteristics who were resident in the health district at the time of referral. INTERVENTIONS--Screening tests for vision or ocular defects already routinely used were applied by health visitors at 8 and 18 months to the children at high risk. MAIN OUTCOME MEASURE--Comparison of results of screening tests with vision and ocular defects detected by the age of 2. RESULTS--Screening tests in current use for vision loss and squint in this age group were insensitive and had a low positive predictive value when applied to a high risk population. Defects that were not apparent on direct inspection were unlikely to be detected by these tests. In the high risk group the relative risk of having a defect was 2.8 (95% confidence interval 1.8 to 4.5) but 85% of all cases detected by the age of 2 were in children at low risk. Referral patterns and age of referral differed in the two groups. CONCLUSIONS--Screening by health visitors of high risk populations contributes little to the detection of vision and ocular defects. This type of evaluation needs to be applied also to low risk populations, who have different referral patterns and contribute most of the cases.     

Long-duration freewheel running and submandibular lymphocyte response to forced exercise in older mice

Submandibular lymph nodes (SLN) are crucial for immune surveillance of the anterior ocular chamber and upper respiratory tract; little is known about how training and exercise affect SLN lymphocytes. The intent of this study was to describe the impact of long term freewheel running followed by acute strenuous exercise on SLN lymphocytes in mice. Female C57BL/6 mice were assigned to running wheels or remained sedentary for 8 months, and further randomized to treadmill exercise and sacrifice immediately, treadmill exercise and sacrifice 24 h after exercise cessation, or no treadmill exposure. SLN lymphocytes were isolated and analyzed for CD3, CD4, CD8, and CD19 cell surface markers, phosphatidylserine externalization as a marker of apoptosis, and intracellular glutathione as a marker of oxidative stress. Compared with running wheel mice, older sedentary mice had a lower percent of T cells and higher percent of B cells (p < 0.05). Although intracellular glutathione did not differ between groups, running mice had a lower percent of Annexin V(+) SLN lymphocytes 24 h after treadmill exercise. Further research will be needed to determine if voluntary exercise translates into improved anterior ocular and upper respiratory tract health.     

Ocular findings in patients with chronic renal failure undergoing haemodialysis

The aim of this paper was to evaluate the ocular findings in patients with chronic renal failure (CRF) undergoing haemodialysis (HD). In 64 patients undergoing haemodialysis (30 female and 34 male), aged 24-83 years (mean 58 years) on haemodialysis 1-213 months (mean 47 months) complete ocular examination were performed: visual acuity (VA), intraocular pressure (IOP), biomicroscopic examination and fundoscopy. On right eye sixty-nine percent of patents had VA 0.6 or better, and on left eye 84% of patients had VA 0.6 or better. Mean IOP before dialysis was 15 mmHg and after dialysis was 14 mmHg. In 9 patients (14%) we found corneo-conjunctival calcium deposits. No correlation of ocular calcification and parathyroid hormone (PTH) level or calcium and phosphate product were observed. 39 (60%) patients had cataract. Hypertensive vascular changes were seen in 44 (68%) patients and in 6 (7%) patients age-related macular degeneration. Seven patients had diabetes mellitus and in 5 diabetic retinopathy was observed. Patients with CRF or who are receiving HD represent unique group of patients. Pathologic change could be found in many tissue and organs, therefore we suggest ocular examination more frequently in dialysis patients.     

Soil-Transmitted Helminth Infections and Correlated Risk Factors in Preschool and School-Aged Children in Rural Southwest China

We conducted a survey of 1707 children in 141 impoverished rural areas of Guizhou and Sichuan Provinces in Southwest China. Kato-Katz smear testing of stool samples elucidated the prevalence of ascariasis, trichuriasis and hookworm infections in pre-school and school aged children. Demographic, hygiene, household and anthropometric data were collected to better understand risks for infection in this population. 21.2 percent of pre-school children and 22.9 percent of school aged children were infected with at least one of the three types of STH. In Guizhou, 33.9 percent of pre-school children were infected, as were 40.1 percent of school aged children. In Sichuan, these numbers were 9.7 percent and 6.6 percent, respectively. Number of siblings, maternal education, consumption of uncooked meat, consumption of unboiled water, and livestock ownership all correlated significantly with STH infection. Through decomposition analysis, we determined that these correlates made up 26.7 percent of the difference in STH infection between the two provinces. Multivariate analysis showed that STH infection is associated with significantly lower weight-for-age and height-for-age z-scores; moreover, older children infected with STHs lag further behind on the international growth scales than younger children.     

Antibody responses following rubella immunization analyzed by HLA and ABO types

HLA typing was performed on 232 rubella seronegative (HAI antibody titer <8) children immunized with RA 27/3 vaccine. High convalescentphase geometric mean antibody titers were noted in children one to five years of age, in girls one to 12 years of age, in subjects with AB blood type and in vaccinees with HLA types B14 and BW22. Of nine children with rubella titers512, 44 percent had HLA type A28; in contrast, only eight percent of the total group had A28 HLA type. HLA-A2 was present in four, and HLA types B12 and BW17 were present in three of the six children in whom seroconversion did not occur. Fifteen of 37 sibling pairs had identical antibody responses; in contrast, only 18 percent of randomly paired children had titers identical to each other. Vaccine-related symptomatology could not be associated with any HLA type. The results of this study suggest that infection with and antibody response to RA 27/3 rubella vaccine is influenced by genetic factors, which include cellular membrane determinants.     

A Predictive Study of Congenital Heart Disease and Need for Care

For long-term planning in the delivery of health care, prevalence data are essential for budget estimates in terms both of distribution and training of manpower and fiscal responsibility. From incidence figures, from the knowledge of the natural history of congenital heart disease and from predicted population estimates it is possible to construct a model that reflects the prevalence of congenital heart disease. This has been done for the state of California; the methods used and the data gathered should prove useful nationally.It is estimated that there were in 1975 in California 17,531 children under 21 years of age with congenital heart disease; 24 percent of these had ventricular septal defects and 23 percent had pulmonary stenosis, 11 percent had atrial septal defects and 9 percent had aortic stenosis; the other forms of congenital heart disease constituted the remaining 33 percent. Based on these estimates it is then possible to plan the medical resources necessary for optimal care.     

31 cases with oculoauriculovertebral dysplasia (Goldenhar syndrome): clinical, neuroradiologic, audiologic and cytogenetic findings

Goldenhar syndrome (GS) or oculoauriculovertebral dysplasia (OAVD) is characterized by pre-auricular skin tags, microtia, facial asymmetry, ocular abnormalities and vertebral anomalies of different size and shape. The phenotypical findings of this syndrome are variable due to heterogenous aetiology. For that reason, the physician sometimes faces difficulty when making a definite diagnosis of OAVD. We reviewed the clinical and laboratory findings of 31 patients (15 boys and 16 girls) aged from 1 day to 16 years with the clinical diagnosis of GS. The characteristic features were pre-auricular skin tags (90%), microtia (52%), hemifacial microsomia (77%) and epibulbar dermoids (39%). Vertebral anomalies were noted in 70% of the patients. Cardiac malformations were found in 39% while a genitourinary anomaly was noted in 23% and various central nervous system malformations in 47%. There were 3 pregnancies following an intracytoplasmic sperm injection (ICSI) technique among the 31 patients. Two patients with GS came from the same family. Their relatives had hydrocephaly, myelomeningocele and neural tube defects. It is known that some chromosomal aberrations are seen in GS. We performed chromosome analysis of 29 patients. Among these cases, only one patient with severe mental and motor retardation had a 47,XX,+der(22)t(11,22)(q23; q11 karyotype due to a maternal balanced translocation t(11;22)(q23;q11). This translocation was demonstrated in her sister, brother and maternal uncle. Additionally CATCH 22 analysis in 13 cases with OAVD with a CATCH 22 phenotype revealed no deletion. OAVD patients present with different morphologic features and systemic manifestations. A multidisciplinary approach should be undertaken by departments such as pediatric cardiology, audiology, ophthalmology and plastic surgery when evaluating patients with OAVD. Chromosome analysis should be performed in every patient with Goldenhar syndrome.     

Epidemiological and clinical features of 1,149 persons with Lyme disease identified by laboratory-based surveillance in Connecticut

Laboratory-based surveillance of Lyme disease in Connecticut during 1984 and 1985 identified 3,098 persons with suspected Lyme disease; 1,149 were defined as cases. Lyme disease incidence in Connecticut towns ranged from none to 1,407 cases per 100,000 population in 1985. A comparison of 1985 data with data from 1977 epidemiologic studies indicated that incidence increased by 129 percent to 453 percent in towns previously known to be endemic for Lyme disease and that Lyme disease had spread northward into towns thought to be free of Lyme disease in 1977. Children aged five to 14 years had the highest incidence. Of persons with Lyme disease, 83 percent had erythema migrans, 24 percent had arthritis, 8 percent had neurologic sequelae, and 2 percent had cardiac sequelae. The distribution of symptoms was age-dependent: case-persons less than 20 years old were almost twice as likely to have arthritis than older case-persons (35 percent versus 18 percent). Of persons with arthritis, 92 percent of those less than 20 years of age, compared to 68 percent of older persons, did not have antecedent erythema migrans. We conclude that Lyme disease is increasing in incidence and geographic distribution in Connecticut. Of those with Lyme disease, children may be more likely than adults to develop arthritis and have it as their first major disease manifestation.     

Role of the School Eye Clinic in Modern Ophthalmology

Routine sight tests for children at intervals throughout their school career are clearly important; three-quarters of those referred to the school eye clinics in this area had some ocular defect. It is probably no longer necessary for myopic schoolchildren to be treated by a consultant ophthalmologist after their initial examination.Forty-five per cent. of children referred to the school eye clinics in this area were found to have squint and/or hypermetropic/anisometropic/astigmatic refractive errors. Priority should be given to this group because of the association of amblyopia with these conditions. Their treatment requires closer association with the hospital ophthalmic department, perhaps even complete unity. Transfer of children at present seen in the school eye clinic to a hospital-based “children''s eye clinic” would also fit in with a unified health service administration structure and be better placed to indicate, evaluate, and control future developments towards the prevention of amblyopia.