Salivary microbiome and asthma risk in children with orofacial defects.

BACKGROUND: Patients with congenital orofacial defects, cleft lip (CL), cleft palate (CP), and cleft lip and palate (CLP) have continuous exposure of the respiratory system to the microbiome from the oral environment, offering opportunities to develop mucosal immunity in the airway. This two-part study aims to analyze data on asthma occurrence in CL, CP, and CLP infants and the composition of the salivary microbiome, and to evaluate the oral microbiota and its association with the risk of developing childhood asthma. METHODS: Patient data from the research database of Chang Gung Memorial Hospital from 2004 to 2015 were retrospectively analyzed by multivariable regression. Diseases diagnoses were defined by ICD codes. Asthma must also meet the criteria for receiving selective ß2 agonistic or/and inhaled corticosteroid treatments twice within 1 year. Analysis of the saliva microbiome was performed prospectively from 2016 to 2020 in 10 healthy term infants and 10 CLP infants on postnatal 7th day, 1 month, and 6 months by next-generation sequencing. RESULTS: Asthma and nonasthma groups included 988 and 3952 patients, respectively. The incidence of asthma development was higher in patients with CP than in CL and CLP groups (aOR: 5.644, CI: 1.423-22.376). The species composition of the microbiome at 1 and 6 months was significantly different between infants with CLP and healthy infants. CONCLUSION: Children with orofacial defects have a higher risk of developing asthma with a possible contribution from oral microbiota in the early months of life.

The Multiple-U Technique: A Novel Microvascular Anastomosis Technique That Guarantees Everted Anastomosis Sites with Solid Intima-to-Intima Contact.

SUMMARY: The key to successful microvascular anastomosis is achieving intima-to-intima contact, which is not always easy. In this article, the authors propose the multiple-U technique, which is a novel microvascular anastomosis technique that characterizes easy and reliable intima-to-intima contact. The technique was performed on patients who underwent free flap reconstruction for head and neck defects at the Kaohsiung Chang Gung Memorial Hospital from September 1, 2020, to November 30, 2020. The immediate patency test results for all vessel anastomoses were positive, and the postoperative recovery courses of the patients were without any vascular complications. In conclusion, the multiple-U technique is a widely available technique that guarantees everted anastomosis sites and solid intima-to-intima contact. This technique can be performed on both arterial and venous anastomoses regardless of vessel size and wall thickness.

Mutation survey in Taiwanese patients with Stickler syndrome.

PURPOSE: The purpose of this study was to identify gene mutation and phenotype correlations in a cohort of Taiwanese patients with Stickler syndrome. MATERIALS AND METHODS: Patients clinically diagnosed with Stickler syndrome or suspected Stickler syndrome were enrolled. DNA was extracted from venous blood samples. For the targeted next-generation sequencing (NGS) approach, specific primers were designed for all COL2A1, COL11A1, COL11A2, COL9A1, and COL9A2 exons and flanking intron sequences. RESULTS: Twenty-three patients from 12 families were enrolled in this study. The myopia power in these 23 cases (35 eyes) ranged from -4.625 to -25.625 D, with a median of -10.00 D. Four patients had retinal detachment. Fourteen patients had a cleft palate. These 23 patients and 13 healthy controls were enrolled in the NGS study. Three families had significant single nucleotide variants (SNVs) in COL2A1. The mutation rates in this survey were 25% (3/12 families) and 35% (8/23 cases). The SNV of family #1, located at exon 27, c.1753G >T, p. Gly585Val, was novel and has not yet been reported in the ClinVar database. Families #10 and #11 had the same SNV, located in exon 33, c.2101C >T, p. Arg701X. Both variants were classified as likely pathogenic according to the American College of Medical Genetics and Genomics guidelines. CONCLUSION: Genetic mutations in COL2A1 were found in 25% of Taiwanese families with Stickler syndrome. One novel variant was identified using NGS, which expanded the COL2A1 mutation spectrum. Molecular genetic analysis is helpful to confirm the clinical diagnosis of patients with suspected Stickler syndrome.

Ultrasound-Guided Moving Shot Radiofrequency Ablation of Benign Soft Tissue Neoplasm.

Background and Objective: To evaluate the effectiveness of radiofrequency ablation (RFA) using the moving-shot technique for benign soft tissue neoplasm. Materials and Methods: This retrospective study reviewed eight patients with benign soft tissue neoplasm presenting with cosmetic concerns and/or symptomatic issues who refused surgery. Six patients had vascular malformation, including four with venous malformation and two with congenital hemangioma. The other two patients had neurofibroma. All patients underwent RFA using the moving-shot technique. Imaging and clinical follow-up were performed in all patients. Follow-up image modalities included ultrasound (US), computed tomography (CT), and magnetic resonance (MR) imaging. The volume reduction ratio (VRR), cosmetic scale (CS), and complications were evaluated. Results: Among the seven patients having received single-stage RFA, there were significant volume reductions between baseline (33.3 ± 21.2 cm3), midterm follow-up (5.1 ± 3.8 cm3, p = 0.020), and final follow-up (3.6 ± 1.4 cm3, p = 0.022) volumes. The VRR was 84.5 ± 9.2% at final follow-up. There were also significant improvements in the CS (from 3.71 to 1.57, p = 0.017). The remaining patient, in the process of a scheduled two-stage RFA, had a 33.8% VRR after the first RFA. The overall VRR among the eight patients was 77.5%. No complications or re-growth of the targeted lesions were noted during the follow-up period. Of the eight patients, two received RFA under local anesthesia, while the other six patients were under general anesthesia. Conclusions: RFA using the moving-shot technique is an effective, safe, and minimally invasive treatment for benign soft tissue neoplasms, achieving mass volume reduction within 6 months and significant esthetic improvement, either with local anesthesia or with general anesthesia under certain conditions.

Investigate the improvement of facial skin texture with the VISIA system after total thyroidectomy.

BACKGROUND: During clinical practice we have noticed that some patients with hyperthyroidism have finer skin with less wrinkles, pores, and spots after thyroidectomy, and the improvement can be observed within a few weeks after the operation. However, there is no evidence or study in the literature to proof this finding. AIM AND OBJECTIVE: This study was designed to evaluate and quantify the skin characters of patients with hyperthyroidism before and after thyroidectomy. MATERIAL AND METHODS: This is a prospective study to include patients with hyperthyroidism who received total thyroidectomy between March 1st, 2018 and February 28th, 2019. The patients received blood test for T4 and TSH analysis and VISIA measurements for skin texture quantification, at the preoperative stage, three, and six months postoperatively. A total of 8 patients were included. Repeated measurement was used to determine the lab data and VISIA measurement changes before and after the operation. Mauchly's sphericity test was performed to determine whether the violation of sphericity occurs, and the Greenhouse-Geisser correction was used when the violation of sphericity occurs. RESULTS: All the patients were female and generally healthy without systemic medical disease except the hyperthyroidism. The T4 and TSH levels were not significantly different before and after the thyroidectomy. In terms of the skin character measurements, the wrinkles, texture, pores, UV spots, and brown spots were not improved after thyroidectomy. A trend of improvement in spots, red area, and porphyrin was noted, although not statistically significant. CONCLUSIONS: Surgical removal of the thyroid gland in patients with hyperthyroidism does not improve the skin quality and texture in examinations via the VISIA system.

Velar Closing Ratio As a Predictor for the Verlopharyngeal Function After Double Opposing Z-Plasty for Postpalatoplasty Velopharyngeal Insufficiency in Patients With Cleft Palate.

BACKGROUND: This study was designed to identify the potential predictors of postoperative velopharyngeal function after double opposing Z-plasty (DOZP) for the treatment of velopharyngeal insufficiency (VPI) in patients who had prior palatoplasty for cleft palate. METHODS: This retrospective study reviewed the medical records of consecutive patients who received DOZP for VPI after receiving a prior palatoplasty treating cleft palate between 2004 and 2017. The speech outcome of patient was measured using the Pittsburgh Weighted Speech Scale (PWSS) at 6 months following surgery and determined the outcome suggests velopharyngeal competence (PWSS ≤2) or incompetence (PWSS >2). Stepwise logistic regression was used to identify the variables for the prediction of competent surgical outcome. The specific receiver operating characteristic curves with an area under the curve (AUC) was used to evaluate the predictor related to the surgical outcome as competence. RESULTS: The study included 93 patients. Age, relative velar length, velar lengthening, and closure pattern were not significantly associated with postoperative competence status of the patient. The only variable that predicted a successful surgical outcome was preoperative velar closing ratio. However, the accuracy of velar closing ratio in predicting a competent surgical outcome is only moderate (AUC = 70.37). CONCLUSION: The results of this study showed that preoperative velar closing ratio may predict, with moderate accuracy, a successful surgical outcome in patients with postpalatoplasty VPI who undergo DOZP. Therefore, in patients with a low preoperative velar closing ratio, some alternative surgical methods other than DOZP may be considered to avoid unsatisfactory surgical outcome.

Use of Nasal Conformer After Birth Effectively Improves Nostril Symmetry in Patients With Unilateral Incomplete Cleft Lip.

PURPOSE: To investigate the clinical effects of preoperative nasoalveolar molding (NAM) and nasal conformer use in patients with unilateral incomplete cleft lip on the basis of their medical records and images. PATIENTS AND METHODS: Data and images of 16 patients born with unilateral incomplete cleft lip who were hospitalized between January 2015 and August 2017 were retrieved from the medical records. The primary outcome was the extent of improvement in columella height (CH) before cheiloplasty. Other outcome measurements included the CH, nostril width, and nostril height, which were measured by ImageJ image processing software (version 1.4; National Institutes of Health, Bethesda, MD) and presented as ratios. Mann-Whitney U tests were used to compare the non-normally distributed data. RESULTS: Patients in the NAM group and those in the nasal conformer group showed significantly improved (P < .05) preoperative cleft-side CH-to-normal-side CH ratios compared with the corresponding ratios at birth. There was no significant difference in terms of the extent of improvement in CH between the groups. CONCLUSIONS: Preoperative use of nasal conformers in patients with unilateral incomplete cleft lip not only corrects the deformed nasal cartilage but also increases the CH and improves the overall preoperative nasal symmetry. In addition, compared with NAM, this method costs less, is more straightforward, and requires fewer outpatient clinic visits.

Muscle relaxation for individuals having tattoos removed through laser treatment: possible effects regarding anxiety and pain.

Effectively managing pain is vital for the well-being and satisfaction of patients undergoing dermatologic treatments involving lasers. This study investigates the potential outcome of using muscle relaxation techniques to reduce pain among people having their tattoos removed with laser treatment. This study consists of 56 participants (mean age 18.1 ± 2.1 years) that had tattoos removed using the principle of selective photothermolysis. These participants underwent muscle relaxation before receiving the laser treatment. Their peripheral skin temperatures (PST) were measured both at the beginning and the end of the muscle relaxation period. Then, the Beck Anxiety Inventory was applied to evaluate anxiety levels. Once the laser treatment was completed, pain levels were measured using a visual analogue scale. A total of 125 person-sessions of laser treatment and psychometric assessments were performed in this study. The muscle relaxation method significantly increased the PST of the participants while reducing the levels of anxiety and pain throughout the course of the laser treatment procedure. The PST, anxiety scores, and pain scores all showed significant correlations with one another. According to the results obtained, this study proposes that muscle relaxation techniques be considered possibly auxiliary treatment options for individuals having tattoos removed through laser treatment. Additional studies with a comparison group and a larger sample size are required in the future to confirm the effectiveness of such intervention.

The upregulation of zinc finger protein 670 and prostaglandin D2 synthase in proliferative vitreoretinopathy.

PURPOSE: Proteins in the vitreous play an important role on the induction of proliferative vitreoretinopathy (PVR) after retinal detachment. The aim of this study was to investigate the variation of protein patterns in the vitreous of PVR eyes and examine whether differentially expressed protein levels were expressed in experimental PVR retina. METHODS: Vitreous samples from PVR and macular hole patients were selected for proteomic analysis. The vitreous protein samples were separated by two-dimensional electrophoresis (2-DE). The differentially expressed protein spots in the two groups were excised and subjected to in-gel digestion and identification by electrospray ionization mass spectrometry (ESI-MS) analysis. Two differentially expressed proteins, zinc finger protein 670 (ZFP 670) and prostaglandin D2 synthase (PGD2S), were further validated by immunohistochemical staining and western blotting analysis in the retina of the experimental rabbit PVR model. RESULTS: In proteome analysis of human vitreous samples, five proteins had increased expression in PVR, including zinc finger protein 670 (ZFP 670), prostaglandin D2 synthase (PGD2S), IgG (Immunoglobulin G) light chain, transthyretin precursor, and haptoglobin precursor. ZFP 670 and PGD2S levels were expressed significantly higher in the experimental PVR retinas than in the control group. CONCLUSIONS: Levels of ZFP 670 and PGD2S were elevated in the vitreous fluid of patients with PVR. In addition, there were higher expressions of ZFP 670 and PGD2S in the experimental PVR retina. This result will expand our knowledge of pathophysiologic characteristics of PVR, and might be helpful for further developing possible treatment on this disorder.

Management of malocclusion and facial asymmetry secondary to fractures of the mandibular condyle process.

INTRODUCTION: Most condylar process fractures can be managed conservatively with satisfactory outcome. However, unsuccessful treatment can result in malocclusion and facial asymmetry. We report our experience in surgical management of malunited condylar process fractures. METHODS: This is a retrospective review of clinical records, photographs, imaging, and dental models of 12 consecutive patients who presented with malocclusion and facial asymmetry after nonoperative or failed treatment of condylar process fractures. Eight patients who presented relatively early (<6 months) after the initial trauma were treated with subcondylar osteotomy (SCO), whereas 4 patients who presented relatively late (>18 months) were treated with sagittal split osteotomy (SSO). These 2 groups were compared in terms of 3 parameters, namely, maximum mouth opening, aesthetic improvement, and patient satisfaction. RESULTS: The 2 groups were statistically similar in all 3 parameters, with the SCO group trending toward higher scores in all 3 parameters. The mean increase in maximal mouth opening in the SCO group was 21 versus 2.5 mm in the SSO group. CONCLUSIONS: Subcondylar osteotomy, performed at a relatively early time point, is at least as effective, if not more effective, than traditional SSO in the treatment of subcondylar malunions. Given this finding, a lower threshold should be adopted for the primary treatment of acute subcondylar fractures with open reduction and internal fixation, especially those with moderate displacement that may be at high risk for malunion.

Attenuated glial reaction in experimental proliferative vitreoretinopathy treated with liposomal doxorubicin.

PURPOSE: We investigated the therapeutic effect of liposomal doxorubicin (Lipo-dox) on experimental proliferative vitreoretinopathy (PVR). METHODS: The toxicity of Lipo-dox was determined in vitro in cultured rabbit retinal pigment epithelium (RPE) cells by tetrazolium-based (MTT) assay for cell viability performed 48 and 96 hours after treatment, and in vivo by electroretinography and histopathology. The therapeutic effect of intravitreous injection of Lipo-dox was evaluated in a rabbit model of PVR induced by injection of rabbit RPE cells after gas compression of the vitreous. The presence of PVR was determined by indirect ophthalmoscopy on days 1, 7, 14, 21, and 28 after injection. Western blot and immunofluorescence studies were performed to evaluate the expression of the glial markers vimentin and glial fibrillary acidic protein (GFAP). A pharmacokinetic study also was performed and analyzed by liquid chromatography coupled with tandem mass spectrometry (LC-MS). RESULTS: The 50% inhibitory concentrations (IC50) of doxorubicin (Doxo) and Lipo-dox in RPE cells were 0.01-0.1 and 0.1-1.0 µg/mL, respectively. Lipo-dox (10 µg/mL) did not reduce the amplitude reduction in the ERG study or produce obvious retinal toxicity. Lipo-dox still could be detected in the vitreous 7 days after injection. The Lipo-dox (10 µg/mL)-treated eyes showed lower grade PVR than did the untreated eyes. Lipo-dox also decreased the retinal expression levels of vimentin and GFAP. CONCLUSIONS: Lipo-dox can attenuate the severity of experimental PVR, and reduces the glial cell expression of intermediate filaments in PVR retinas. Lipo-dox has a wider safe dosage range and a longer half-life in the vitreous than does primary Doxo.

Risk factors for abnormal anal cytology over time in HIV-infected women.

OBJECTIVE: The objective of the study was to assess the incidence of, and risk factors for, abnormal anal cytology and anal intraepithelial neoplasia (AIN) 2-3 in human immunodeficiency virus (HIV)-infected women. STUDY DESIGN: This prospective study assessed 100 HIV-infected women with anal and cervical specimens for cytology and high-risk human papillomavirus (HPV) testing over 3 semiannual visits. RESULTS: Thirty-three women were diagnosed with an anal cytologic abnormality at least once. Anal cytology abnormality was associated with current CD4 count less than 200 cells/mm(3), anal HPV infection, and a history of other sexually transmitted infections (STIs). Twelve subjects were diagnosed with AIN2-3: 4 after AIN1 diagnosis and 4 after 1 or more negative anal cytology. AIN2-3 trended toward an association with history of cervical cytologic abnormality and history of STI. CONCLUSION: Repeated annual anal cytology screening for HIV-infected women, particularly for those with increased immunosuppression, anal and/or cervical HPV, a history of other STIs, or abnormal cervical cytology, will increase the likelihood of detecting AIN2-3.

Simvastatin reduces VCAM-1 expression in human umbilical vein endothelial cells exposed to lipopolysaccharide.

OBJECTIVE: Reducing the expression of endothelial cell adhesion molecules (ECAMs) is known to decrease inflammation-induced vascular complications. In this paper we looked at whether statins can reduce inflammation-induced ECAM expression after lipopolysaccharide (LPS) treatment in endothelial cells. METHODS: Human umbilical vein endothelial cells (HUVECs) were pretreated with different concentrations of simvastatin, atorvastatin, and rosuvastatin and subsequently exposed to 5 µg/ml LPS. Semi-quantitative RT-PCR analysis was used to measure the mRNA expression of ECAMs, including VCAM-1, ICAM-1, and E-selectin. RESULTS: VCAM-1 mRNA appeared to be the only target that was affected by the statins, with its expression being partially and almost completely reduced by simvastatin at 50 and 125 µM concentrations, respectively, and only partially reduced by atorvastatin, but not reduced by rosuvastatin. VCAM-1 protein production was inhibited by simvastatin at concentrations from 5 to 125 µM. Leukocyte-endothelial cell adhesion assay revealed that simvastatin could inhibit the adhesion of labelled U937 cells to the HUVEC monolayer. CONCLUSIONS: This study showed that simvastatin reduces VCAM-1 expression in HUVECs exposed to LPS and decreases leukocyte-endothelial cell adhesion.

Effect of extracorporeal shockwave treatment on the melanogenic activity of cultured melanocytes.

In addition to the traditional lithotripsy treatment, extracorporeal shockwaves (ESWs) have been shown to be effective in the treatment of certain musculoskeletal disorders and in enhancing skin flap neovascularization. However, relatively little is known about its effect on melanocytes. To investigate its effect on the melanogenic activity of cultured melanocytes, mouse B16F10 melanocytes were treated with defocused ESWs of different energies (15, 21, and 27 kV) and at different doses (300 and 600 impulses). Cell viability was measured 1 and 24 h after treatment. Melanin content was measured and compared against a standard curve generated with fungal melanin. Cellular tyrosinase activity was calculated with the 3,4-dihydroxyphenylalanine (DOPA) oxidase assay. The results demonstrated that ESW treatment reduced cell viability. Our results also indicated that the overall decrease in cell viability lasted for 6 days. After ESW treatment with 300 or 600 impulses at 21 kV, no significant change in melanin content or tyrosinase activity of the B16F10 melanocytes was noted as compared to those of the control. The present study suggests that ESW treatment does not alter the melanogenic activity of the cultured melanocytes.

Traumatic superior orbital fissure syndrome: assessment of cranial nerve recovery in 33 cases.

BACKGROUND: Superior orbital fissure syndrome is a rare complication that occurs in association with craniofacial trauma. The characteristics of superior orbital fissure syndrome are attributable to a constellation of cranial nerve III, IV, and VI palsies. This is the largest series describing traumatic superior orbital fissure syndrome that assesses the recovery of individual cranial nerve function after treatment. METHODS: In a review from 1988 to 2002, 33 patients with superior orbital fissure syndrome were identified from 11,284 patients (0.3 percent) with skull and facial fractures. Severity of cranial nerve injury and functional recovery were evaluated by extraocular muscle movement. Patients were evaluated on average 6 days after initial injury, and average follow-up was 11.8 months. RESULTS: There were 23 male patients. The average age was 31 years. The major mechanism of injury was motorcycle accident (67 percent). Twenty-two received conservative treatment, five were treated with steroids, and six patients underwent surgical decompression of the superior orbital fissure. After initial injury, cranial nerve VI suffered the most damage, whereas cranial nerve IV sustained the least. In the first 3 months, recovery was greatest in cranial nerve VI. At 9 months, function was lowest in cranial nerve VI and highest in cranial nerve IV. Eight patients (24 percent) had complete recovery of all cranial nerves. Functional recovery of all cranial nerves reached a plateau at 6 months after trauma. CONCLUSIONS: Cranial nerve IV suffered the least injury, whereas cranial nerve VI experienced the most neurologic deficits. Cranial nerve palsies improved to their final recovery endpoints by 6 months. Surgical decompression is considered when there is evidence of bony compression of the superior orbital fissure.

Abnormal anal cytology in HIV-infected women.

OBJECTIVE: The purpose of this study was to assess the prevalence of and risk factors for abnormal anal cytology and human papillomavirus (HPV) infections in women who are human immunodeficiency virus (HIV) positive. STUDY DESIGN: We conducted an observational single center study of 100 HIV-infected women with cervical and anal specimens that were obtained for cytologic and high-risk HPV testing with Hybrid Capture 2. RESULTS: Seventeen women had abnormal anal cytology; 16 women had anal HPV; 21 women had abnormal cervical cytology, and 24 women had cervical HPV. Abnormal anal cytology was associated with cervical HPV infection, abnormal cervical cytology, and anal HPV infection in univariate analysis. In multivariate analysis, abnormal anal cytology was associated with a CD4 count <200 cells/mm(3), a history of sexually transmitted disease, and concurrent cervical cytologic abnormality. CONCLUSION: HIV-infected women are at high risk for abnormal cytology and HPV infections of both the anus and cervix. Risk factors for abnormal anal cytology include abnormal cervical cytology, cervical and anal HPV infections, and low CD4 count.

Retrobulbar hematoma as a rare complication after secondary correction of enophthalmos.

Retrobulbar hematoma is a rare complication after orbital surgery, with the potentially disastrous consequence of visual impairment and blindness. We report a female patient who was admitted for enophthalmos correction because of the unsatisfactory result of the primary repair of an orbital blowout fracture and who subsequently experienced the complication of retrobulbar hematoma after operation. The initial presentation of the patient was eye pain with vomiting, followed by proptosis and visual impairment. Retrobulbar hematoma was confirmed by computed tomography, and immediate medical and surgical treatments were instituted. The vision of the patient recovered gradually during outpatient follow-up.

Multiple digital gangrene after a radial artery penetrating injury: a case report.

We describe the case of a young female patient who sustained a radial artery penetration injury from broken glass after falling and was managed only by external compression without arterial repair. Although the patient did not have any reported predisposing factors and the radial artery was normal as well as patent in the angiographic examination, she developed progressive multiple digital ischemia and required subsequent amputation of the gangrenous fingers. The possible mechanism might be an occlusion of the digital arteries originating from the injured radial artery by dispersion of thrombembolic particles distally during the injury as there was sparing of involvement of the ring finger, which wore a ring that restricted the thromboembolic particles from passing distally during the accident. This case revealed that particular attention should be paid to those patients with a radial artery penetration injury to facilitate early detection and implementation of therapy for possible finger ischemia and subsequent gangrene.

Endoscopic repair of anterior table: frontal sinus fractures with a Medpor implant.

OBJECTIVES: Isolated anterior table frontal sinus fractures are commonly repaired through a coronal incision. Endoscopic repair of these injuries has recently been described. This study evaluates the endoscopic repair of isolated anterior table frontal sinus fractures with a Medpor implant. METHODS: Preinjury photographs of 10 cadaveric heads were obtained. Anterior table frontal sinus fractures were generated in all cadavers. The fractures were documented with postinjury computerized tomography (CT) scans. The fractures were then endoscopically repaired with a Medpor implant. Five cadavers received prefabricated implants generated from the post injury CT data. Five cadavers received a standard implant (0.85 mm sheet) contoured intraoperatively. The success of each repair was documented with post repair CT scans, photographs, and direct transcutaneous visualization. RESULTS: All 10 defects were successfully repaired within 1 to 2 mm. All implants were palpable, but no objective asymmetry could be appreciated photographically or on CT scan. CONCLUSION: Anterior table frontal sinus fractures can be endoscopically repaired with either a standard 0.85 mm sheet or a prefabricated implant. Clinical application of this technique would be expected to reduce operating time, surgical morbidity, and cost.

Visual complications of Stickler syndrome in paediatric patients with Robin sequence.

BACKGROUND: The most frequent diagnosis in patients with syndromic Pierre Robin sequence is Stickler syndrome, which may be complicated by congenital high myopia and substantial risk of retinal detachment. However, cases of Stickler syndrome with probable visual complications are rarely identified among this group of patients by members of the cleft team. This study was designed to determine the prevalence of Stickler syndrome among the author's group of patients with Robin sequence, and to investigate the visual outcome among paediatric patients with Robin sequence and Stickler syndrome. MATERIAL AND METHODS: Eight children (six male and two female) with Stickler syndrome and Robin sequence were referred to be followed up every 6 months in the Ophthalmologic Department because of high myopia at less than 10 years of age. Three patients came from the author's study group and five were referred by other cleft surgeons. They were examined with repeat ophthalmic and indirect fundus examinations including cycloplegic refraction, and slit lamp biomicroscope examinations. Laser photocoagulation (2 cases) treatment for retinal degeneration or operation (3 cases) for retinal detachment was performed once evidence of significant ophthalmologic finding was noted. RESULTS: Of the 91 cases of newborns with isolated cleft palate treated by the first author, eight patients had Robin sequence, and among these, three had Stickler syndrome. The prevalence of Stickler syndrome among this subgroup of patients was 37.5% (3/8). Among these three patients and the additional five referred by other cleft surgeons, the average spherical equivalents of the first cycloplegic refraction for the 16 eyes was -12.39+/-2.72 diopter (D) (range -8.75 to -18.5D). Of the eight patients, five did not need any therapy, two children had retinal degeneration in the left eye and retinal detachment in the right eye while one child had retinal detachment in the right eye only. Laser photocoagulation was performed in the two left eyes with retinal degeneration and was successful. Surgery was performed on the three eyes with retinal detachment, one was successful while two failed and the patients developed total blindness at ages four and six respectively. Out of these three children with retinal complications, only one child remained free of visual deterioration in both eyes during the follow-up period. CONCLUSION: Early identification of Stickler syndrome in children with Robin sequence by cleft surgeons is necessary to insure early referral to an ophthalmologist for detection of myopia, monitoring for retinal detachment, and prevention of visual complications.