Surgical Correction of Hallermann-Streiff Syndrome: A Case Report of Esotropia, Entropion, and Blepharoptosis

We report a case of surgical treatment for Hallermann-Streiff syndrome in a patient with ocular manifestations of esotropia, entropion, and blepharoptosis. A 54-year-old man visited Yeouido St. Mary's Hospital complaining of ocular discomfort due to cilia touching the corneas of both eyes for several years. He had a bird-like face, pinched nose, hypotrichosis of the scalp, mandibular hypoplasia with forward displacement of the temporomandibular joints, a small mouth, and proportional short stature. His ophthalmic features included sparse eyelashes and eyebrows, microphthalmia, nystagmus, lower lid entropion in the right eye, and upper lid entropion with blepharoptosis in both eyes. There was esodeviation of the eyeball of more than 100 prism diopters at near and distance, and there were limitations in ocular movement on lateral gaze. The capsulopalpebral fascia was repaired to treat the right lower lid entropion, but an additional Quickert suture was required to prevent recurrence. Blepharoplasty and levator palpebrae repair were performed for blepharoptosis and dermatochalasis. Three months after lid surgery, the right medial rectus muscle was recessed 7.5 mm, the left medial rectus was recessed 7.25 mm, and the left lateral rectus muscle was resected 8.0 mm.

The Risk Factor Analysis of Infectious Corneal Ulcers Leading to Eyeball Removal

PURPOSE: To determine the risk factors of corneal ulcers that require eyeball removal even when properly managed. METHODS: We performed a retrospective review of 30 patients (30 eyes) who were diagnosed with corneal ulcers and underwent eyeball removal, even though their cases were properly managed, between 1990 and July, 2005. The Patients included of 14 males and 16 females. The mean age of the patients was 61.6+/-12.2. The ages of 20 patients were between 60 and 79. RESULTS: Unknown (13/30 [43.4%]) was the most commonly identified predisposing factor. The other predisposing gactors were ocular trauma (10/30 [33.3%]); bullous keratopathy (3/30 [10%]); keratoplasty (1/30 [3.3%]); pterygium excision (1/30 [3.3%]); history of Mooren's ulcer (1/30 [3.3%]); and bee sting (1/30 [3.3%]). The visual acuity at the time of evisceration was LP(-) (14/30 [46.7%]); LP(+)(4/30 [13.3%]); and hand motion (12/30 [40%]). Organisms were cultured in 7 patient (23.3%). Among these patients, funguses were cultured in 4 patients, and bacteria were cultured in 3 patients. The most common causes of evisceration were perforation (13/30 [43.3%]); pain (7/30 [23.3%]); impending perforation (7/30 [23.3%]); phthisis bulbi (1/30 [3.3%]); and endophthalmitis (1/30 [3.3%]). CONCLUSIONS: According to this study, old age, ocular surface and other ocular disease and the corneal ulcer caused by fungi are the risk factor that can lead to a condition that requires removal of the eyeball. Consequently, we should pay special attention to patients who have these risk factors.

Orbital Implant Infection after Drilling Procedure

PURPOSE: To report the severity of orbital implant infection related to a drilling procedure and to provide a foundation for reevaluating this procedure. METHODS: We investigated retrospectively 17 patients who were suspected of orbital implant infection and whose infected implants were ultimately removed. In these patients, increased mucopurulent eye discharge and implant exposure after the drilling procedure were observed. We attempted to treat them with topical and systemic antibiotics, but the discharge and implant exposure did not improve. RESULTS: Of the 17 removed implants, 13 were hydroxyapatite and 4 were Medpor(R). The pegs used were plastic pegs in 12 cases and titanium pegs in 5 cases. The average duration from the first orbital implant insertion to drilling was 10.2+/-5.3 months. The average duration from drilling to severe infection symptoms was 37.0+/-30.0 months. The average duration from the onset of symptoms to implant removal was 12.9+/-12.6 months. The mean follow-up period after final surgery was 15.4 months. In most cases, the deep portions of the removed implants were fragile, malodorous, and filled with pus. Acute and chronic inflammatory cell infiltrations were observed in the pathological examinations, and H. influenzae, S. aureus, S. viridans were identified. They did not receive any drilling procedure thereafter and no infection or inflammatory sign was subsequently observed. CONCLUSIONS: Drilling and pegging can cause unexpected, severe complications such as orbital implant infection, in which the infected implant has to be removed to be cured. Therefore, before performing a drilling procedure doctors should carefully consider the patient's preferences and urgent needs.

Touch Imprint Cytology Contributed to the Frozen Section Diagnosis of Merkel Cell Carcinoma : A Case Report / 대한세포병리학회지

Merkel cell carcinoma (MCC), a rare primary cutaneous small cell neuroendocrine carcinoma, is a tumor with distinct cytological features. In many cases, immunohistochemical staining (IHC) is required for the differentiation from other small round cell malignancies. Here we describe the cytological findings of Merkel cell carcinoma; these findings contributed to the diagnosis prior to performing IHC. A lower eyelid mass was excised and submitted for frozen section diagnosis. The frozen section diagnosis was consistent with a malignancy, but the more specific diagnosis was limited by the lack of specific histological features. Touch imprint cytology revealed a high cellularity with loosely cohesive small to large sized cells. The tumor cells showed hyperchromatic nuclei with fine chromatin and inconspicuous nucleoli, and thin-rimmed-cytoplasm including the characteristic eosinophilic button-like paranuclear inclusion, previously described as a pathognomonic cytological finding of MCC; this was not found in the H&E frozen section. In conclusion, we suggest that the touch imprint cytology may help in the differential diagnosis of small round cell neoplasms prior to performing IHC especially in frozen section diagnosis.

A Case of Granulocytic Sarcoma Involving the Forniceal Conjunctiva

PURPOSE: We report a case of the granulocytic sarcoma located at the conjunctival fornix in a 10-year-old girl with acute myeloid leukemia, who was in complete remission after bone marrow transplantation. METHODS: A 10-year-old girl had a 1-week history of firm, dark brown conjunctival mass at lower fornix and mild lid swelling of the right eye. Several months previously she had had some chalazions at lower lid and a history of acute myeloid leukemia that was currently in complete remission after bone marrow transplantation. Other ocular manifestations were not specific. Excision of the conjunctival mass and a biopsy for diagnostic and therapeutic purposes were performed. RESULTS: The 12 x 6 x 5 mm-sized mass was totally excised. The pathologic examination was consistent with granulocytic sarcoma. Three weeks after the biopsy, the patient had a relapse of acute myeloid leukemia. She died 2 months later while undergoing intensive therapy. CONCLUSIONS: This condition should be considered in the diagnosis of granulocytic sarcoma in patients with a history of acute myeloid leukemia.

A Korean Case of Cornelia de Lange Syndrome

PURPOSE: Cornelia de Lange syndrome is a rare disease showing characteristic facial appearance, developmental delay, growth retardation, low birth weight, skeletal formation anomaly, hirsutism and various ophthalmologic problems. METHODS: We experienced a case of an 18-year-old female with Cornelia de Lange syndrome showing superficial keratitis with entropion, ptosis, high myopia, lacrimal cutaneous fistula and characteristic facial appearance. She was born with low birth weight, operated for cleft palate and diagnosed with ventricular septal defect. In addition, she showed psychological lag and developmental impairment. RESULTS: We performed entropion correction surgery, administered medical therapy for superficial keratitis and prescribed glasses for her myopia. CONCLUSIONS: This is the first case report on the successful correction of entropion with Cornelia de Lange syndrome in Korea.

Treatment of Exposed Hydroxyapatite Orbital Implant Using Acellular Dermal Allograft

PURPOSE: To investigate the results of acellular dermal allograft (SureDerm(R), Hans Biomed Co., Korea) as a new covering material in the exposure of hydroxyapatite orbital implant. METHODS: SureDerm graft was performed in 5 patients with exposed hydroxyapatite orbital implant. Under local anesthesia, the anterior surface of the exposed implant was trimmed and the designed SureDerm was sutured at the margin of the sclera with 6-0 Vicryl. Finally, conjunctival suture was done. The patients were followed up regularly until postoperative 10 months. RESULTS: The average diameter of exposed implant was 9.6 X 9.0 mm. The grafted SureDerm had survived in all patients at postoperative 10 months and exposure of implant was treated successfully in all cases. CONCLUSIONS: We consider that acellular dermal allograft will be a good replacing material when preserved sclera is not available. It also features the advantage of avoiding the intricate harvesting procedure and the scarring of the donor site in dermo-fat graft.

Result of Partial Nasolacrimal Duct Obstruction After Silicone Tube Intubation

PURPOSE: In a preliminary series of 36 eyes with partial nasolacrimal duct obstruction, the types of dacryoscintigraphy result were classified and the results of silicone tube intubation were analysed. METHODS: The results of dacryoscintigraphy, performed in 36 eyes of 29 patients with partial nasolacrimal duct obstruction, were classified into the following 3 types: pre-lacrimal sac type, proximal nasolacrimal duct type and distal nasolacrimal duct type. Punctoplasty accompanied by silicone tube intubation was conducted selectively. RESULTS: In dacryoscintigraphy, symptoms improved in 6 eyes among 6 in distal nasolacrimal duct type (100%), 14 eyes among 18 in proximal nasolacrimal duct type (77.8%) and 8 eyes among 12 in pre-lacrimal sac type (66.7%). CONCLUSIONS: We conclude that dacryoscintigraphy predicts the results of silicone tube intubation. Furthermore, silicone tube intubation was effective in proximal nasolacrimal duct type and distal nasolacrimal duct type, but less effective in pre-lacrimal sac type of partial nasolacrimal duct obstruction.

Analysis of Cause and Outcome of Posterior Capsular Rupture during Phacoemulsification in Cataract Surgery

PURPOSE: This study is to evaluate the time, cause, management and consequences of the posterior capsule rupture during cataract extraction using phacoemulsification. METHODS: We retrospectively reviewed the medical records of 30 eyes of 30 patients who had undergone posterior capsule rupture during cataract operation with phacoemulsification from February 1997 to February 2000. RESULTS: The rupture occurred most frequently during the phacoemulsification (46.7%) and irrigation and aspiration procedure (36.7%). The capsule-ruptured patients' preoperative conditions were small pupil (30.0%), lack of cooperation (10.0%) and high vitreous pressure (6.7%). But no specific reason was found in half of the cases; probably surgeon's incomplete technique was the cause. We performed anterior vitrectomy in 66.7% of all cases. All cases were started as phacoemulsification, with 4 cases (13.3%) requiring conversion to extracapsular cataract extraction. Intraocular lenses were implanted in the sulcus (53.3%) and in the bag (33.3%). Final corrected visual acuity of 0.5 or better was achieved in 83.3% and 0.8 or better was in 63.3%. Final visual acuity of 0.1 or worse was occurred in 2 cases (6.7%) and the reasons were corneal edema due to endothelial cell damage and postoperative intraocular pressure elevation, respectively. CONCLUSIONS: When properly managed, a torn posterior capsule can result in an excellent visual outcome. Preoperative and intraoperative management such as more frequent administration of mydriatics, use of iris retractors and retrobulbar anesthesia are most important to prevent the posterior capsule rupture.

An Epidemiological Study of the Risk Factors Associated with Anterior Polar Cataract

PURPOSE: To investigate the prevalence rate and risk factors for anterior polar cataract. METHODS: We examined the frequency of anterior polar cataract in 603 patients who had undergone cataract operation in the Department of Ophthalmology from July 1999 to July 2000. Moreover, we evaluated retrospectively the risk factor of 628 anterior polar cataracts and compared with that of 302 non-anterior polar cataract as well as with that of 552 non-cataract persons as a control from March 1995 to December 2000. This study defined risk factors related to the cataract formation as birth place, residence, occupation, sun-light exposure, drinking, smoking, systemic disease, long-term used drugs, family history, ocular trauma, glasses, and feminine parity. RESULTS: The prevalence rate of anterior polar cataract was 16.1% in total cataract patients, it had male of 81.4% and female of 18.6%. Average age of anterior polar cataract patients was 51.9 years; below fifty years was 44 patients (45.4%), 50~59 years 28 patients (28.9%), 60~69 years 16 patients (16.5%), over 70 years 9 patients (9.3%). Our findings for multivariate analysis that ocular trauma, intraocular disease in male and diabetes in female were significantly associated with anterior polar cataract. CONCLUSIONS: In conclusion, 16.1% of total cataract patients had anterior polar cataract; 81.4% was male and 45.5% was below fifty years of age.

Phototherapeutic Keratectomy for Granular Corneal Dystrophy

PURPOSE: In this study, we evaluated the results of PTK(phototherapeutic keratectomy) for granular corneal dystrophy. METHODS: 5 eyes (4 patients) with granular corneal dystrophy were treated by PTK using Summit ExiMed UV200 and VSIX Star excimer laser systems. The epithelium was removed mechanically with a surgical blade. We used methylcellulose 1.0% as a surface modulator before laser ablation. Focal ablations of the central cornea with an ablation zone of 6.0 mm were performed. Mean ablation depth was 105 +/- 15.81 micrometer (range; 80~120 micrometer) Additional hyperopic PRK was performed on 4 eyes out of 5 eyes at the corneal mid-periphery up to maximum +5.00 D to prevent post-PTK hyperopic shift. Mean follow up period was 54.4 +/- 23.43months (range; 24~84months). RESULTS: Removal of corneal opacities allowed for improvement in both uncorrected visual acuity and best corrected visual acuity in all patients. There was an improvement in best-corrected visual acuity of a mean of 2.6 lines on the Snellen chart. Mean corneal thickness in the area of pathology decreased from 490.5 micrometer before surgery to 391.5 micrometer after surgery. There was a hyperopic shift in all the eyes in spite of additional hyperopic PRK by a mean of +2.44 +/- 0.99 D. There was mild recurrent dystrophic change in an eye but not significant enough to affect visual acuity during the follow up period. No major complications developed in any patients. CONCLUSIONS: Our results suggest that PTK is a safe and effective alternative to penetrating and lamellar keratoplasty in patients with granular corneal dystrophy. Mild post-PTK hyperopic shift occurred in all patients in spite of combined hyperopic PRK up to maximum +5.00 D correction at the corneal mid- peripheral zone according to the refractive powers.

Distal Femoral Fracture / 대한정형외과학회잡지

Fractures involving joints and about the joints provide complex problems with small comminuted fractures, surrounding soft tissue injuries and articular cartilage damage. Various problems are also encountered on the weight bearing surface, especially on the convex side of the distal Femur. Even though many auther had recommended kinds of management upto now, operative methods are more suggested in order to get good results regarding with the accurate anatomical reduction, rigid internal fixation and early joint motion. The clinical analysis of rnanagements of 20 cases of distai femoral fracture demonstrate that seperate dual incision-bilateral or right angle plane-to originate fracture site, and anatomical reduction & rigid internal fixation, early non-weight bearing ROM exercise of joint were sometimes mandatory to treat the displaced, comminuted fracture.

Fracture and Dislocation of Cervical Spine / 대한정형외과학회잡지

There was increasing tendency to stabilize unstable cervical spine injuries surgically with the benefit of good stability of the spine, easy nursing care, early mobilization and therefore early rehabilitation. A clinical study was performed on 47 patients with fractures and dislocations of the cervical spine treated at the department of orthopedic surgery, Inje Medical College, Paik Hospital from Jan. 1975 to Dec. 1981. Following is the summery of the our findings. 1. The prevalent age distribution was between 3rd and 6th decade and the ratio between males and females was 10:1. The most common cause of injuries was automobile accident (70%). 2. The most common site of the injuries was C5-6 (34%) and the most frequent mechanism of injury was flexion-rotation type (47%). 3. In overall patients, neurologic damage was found at first examination in 73% and among these, complete paralysis below the injured level in 26%, incomplete paralysis in 11% and nerve root injury in 35%. 4. Among 47 patients, conervative treatment was performed on 9 patients, anterior spinal fusion on 8 patients, anterior spinal fusion with Halo application on 4 patients, posterior wiring with posterior spinal fusion on 16 patients and posterior wiring with anterior spinal fusion on 8 patients. 5. In the several methods of treatment, the posterior wiring with anterior spinal fusion revealed the best results, the correction rate of displacement was 92%, the correction rate of angular deformity 98% and neural recovery rate 72%. 6. The posterior wiring with posterior spinal fusion revealed good results in correction of displacement and angular deformity but required rigid external support for a long time. The anterior spinal fusion revealed poor results in correction of displacement(67%) and angular deformity(38%) and required rigid external support for a long time and had increasing tendency of kyphotic angle after operation. 7. In the treatment of unstable cervical spine injury, we thoughy that early posterior reduction with posterior wiring and followed anterior spinal fusion was ideal for accurate reduction, rigid stability and early mobilization with simple external support.

A Clinical Study of the Surgical Treatment of the Thoraco-Lumbar Spinal Injuries / 대한정형외과학회잡지

Among 334 thoracolumbar spinal injury patients who were admitted to this hospital from June 1972 to June, 1982, 66 patients with fracture and fracture dislocation of thoraco-lumbar spine which were defined as unstable clinically and radiologically were treated with surgical measures. The ratio between male and female was 7.3:1, the majority was found in third and fourth decade (46 cases, 69%), and the most common cause of injury was falling from a height (38 cases, 58%). The most common site of the injury was lumbar spine (29 cases, 44%) and the most common mechanism of injury was flexion-rotation (29 cases, 44%). Our surgical measures were Harrington rod instrumentation with either anterior or posterior fusion (25 cases, 38%), posterior wiring and fusion (14 cases, 21%), anterior decompression and anterior fusion (14 cases, 21%), posterior fusion (4 cases, 6%), anterior fusion and posterior fusion (3 cases, 5%) and etc. The average correction of displacement was 65% and the average correction of kyphotic deformity was 50%. The most remarkable correction was found at the cases of Harrington rod instrumentation (71%, 74%). Neurological deficit had already developed in 43 cases(65%) prior to operation, and the recovery was observed in 18 cases(42%). Most excellent recovery of neural deficit was found also at the cases of Harrington rod instrumentation (11 cases, 52%). We have analysed the results of these treatment and obtained following conclusions. 1. For the unstable fracture and fracture-dislocation of thoraco-lumbar spine with or without neural involvement, immediate surgical treatments were valuable to expect restoration of anatomical reduction and promotion of every possible recovery of neural function with spinal stability and fewest complication. 2. Fixation with Harrington rod instrumentation appears to provide better reduction and stability with neural improvement than other methods, and therefore early undertaking of rehabilitation activities is possible. 3. For the patients who are seriously compromised or require anterior decompression, immediate posterior reduction and fixation with Harrington rod instrumentation followed anterior decompression and anterior fusion of the involved segments at the eariest feasible time, we feel, is the treatment of choice.

A Giant Cell Tumor of the Lumbar Vertebra: One Case Report / 대한정형외과학회잡지

Giant cell tumor of the spine is a rare and potentially malignant condition which presents rather difficult clinical problems in treatment because of their localization and unpredictable clinical course. Authors experienced a case of giant cell tumor involving third lumbar vertebra which was successfully treated by ea bloc resection and anterior interbody fusion of the adjoining vertebrae with iliac bone graft. At eighteen months follow up, there is no evidence of recurrence and solid union of the bone graft was noted.