BACKGROUND: Bilateral Tessier number 3 clefts are extremely rare, and their surgical treatments have not been well established. CASE PRESENTATION: The authors describe the case of a patient with a right Tessier number 3, 11 facial cleft with microphthalmia, a left Tessier number 3 facial cleft with anophthalmia, and cleft palate. We repaired simultaneously the bilateral soft tissue clefts by premaxillary repositioning, cleft lip repair, facial cleft repair by nasal lengthening, midfacial advancement, and an upper eyelid transposition flap with repositioning both the medial canthi. Postoperatively, the patient showed an esthetically acceptable face without unnatural scars. CONCLUSIONS: We achieved good results functionally and esthetically by midfacial advancement with facial muscle reposition instead of traditional interdigitating Z-plasties. The surgical modality of our anatomical repair and 3 months follow-up results are presented.
Anophthalmos ; Cicatrix ; Cleft Lip ; Cleft Palate ; Eyelids ; Facial Muscles ; Follow-Up Studies ; Humans ; Microphthalmos

PURPOSE: We describe our experience with the Permacol graft in anophthalmic socket reconstruction, and compare it to the autologous buccal mucosal graft, emphasizing the postoperative vascularization and contraction of each graft. METHODS: This was a retrospective comparative study. We measured the time necessary for the graft surface to be completely vascularized, as well as the fornix depth of the conjunctival sac in anophthalmic patients. RESULTS: Ten patients underwent Permacol graft reconstruction, with 44 undergoing buccal mucosal graft reconstruction. Seven eyelids (70%) in the Permacol group had a good outcome, with improvement in lower eyelid position and prosthesis retention. Nine out of 10 eyelids (90%) in this group showed complete vascularization of the graft at 2.6 ± 1.9 months postoperatively, while the grafted buccal mucosa was fully vascularized at 1.1 ± 0.3 months postoperatively (p < 0.01). Postoperative fornix depth in the Permacol group was 9.1 ± 2.2 mm, compared to 14.9 ± 4.5 mm in the buccal mucosal graft group (p < 0.01). Mean increases in fornix depth were 33.1% and 67.9% of the mean vertical length of the implanted graft. CONCLUSIONS: The Permacol graft can be useful as spacer graft material in anophthalmic socket patients. It takes longer to vascularize, and undergoes greater graft shrinkage with time, compared to the buccal mucosal graft.
Acellular Dermis* ; Anophthalmos ; Eyelids ; Humans ; Lacrimal Apparatus ; Mouth Mucosa* ; Prosthesis Retention ; Retrospective Studies ; Transplants*

Encephalocraniocutaneous lipomatosis (ECCL) is a rare neurocutaneous syndrome that affects ectomesodermal tissues (skin, eyes, adipose tissue, and brain). The neurologic manifestations associated with ECCL are various including seizures. However, ECCL patients very rarely develop brain tumors that originate from the neuroepithelium. This is the first described case of ECCL in combination with dysembryoplastic neuroepithelial tumor (DNET) that presented with intractable seizures. A 7-year-old girl was admitted to our center because of ECCL and associated uncontrolled seizures. She was born with right anophthalmia and lipomatosis in the right temporal area and endured right temporal lipoma excision at 3 years of age. Seizures began when she was 3 years old, but did not respond to multiple antiepileptic drugs. Brain magnetic resonance (MR) imaging performed at 8 and 10 years of age revealed an interval increase of multifocal hyperintense lesions in the basal ganglia, thalamus, cerebellum, periventricular white matter, and, especially, the right temporal area. A nodular mass near the right hippocampus demonstrated the absence of N-acetylaspartate decrease on brain MR spectroscopy and mildly increased methionine uptake on brain positron emission tomography, suggesting low-grade tumor. Twenty-four-hour video electroencephalographic monitoring also indicated seizures originating from the right temporal area. Right temporal lobectomy was performed without complications, and the nodular lesion was pathologically identified as DNET. The patient has been seizure-free for 14 months since surgery. Although ECCL-associated brain tumors are very rare, careful follow-up imaging and surgical resection is recommended for patients with intractable seizures.
Adipose Tissue ; Anophthalmos ; Anticonvulsants ; Basal Ganglia ; Brain ; Brain Neoplasms ; Cerebellum ; Child ; Drug Resistant Epilepsy ; Female ; Follow-Up Studies ; Hippocampus ; Humans ; Lipoma ; Lipomatosis* ; Magnetic Resonance Spectroscopy ; Methionine ; Neoplasms, Neuroepithelial* ; Neurocutaneous Syndromes ; Neurologic Manifestations ; Positron-Emission Tomography ; Seizures* ; Thalamus ; White Matter

PURPOSE: To investigate the clinical results of patients who have undergone simultaneous dermo-fat graft and insertion of orbital implants in patients who are unable to put on an ocular prosthesis due to severe conjunctival sac contracture or large orbital implant exposure. METHODS: A retrospective analysis was performed of patients who underwent dermo-fat graft simultaneously with orbital implant insertion for replacement of the conjunctival sac from 2007 to 2012. Eight eyes were enrolled in this study and all patients were followed up for phthisis bulbi or implant exposure. RESULTS: Among the eight eyes, five eyes (62.5%) that were diagnosed with orbital implant exposure underwent orbital implant exchange and dermo-fat graft, and two eyes (25%) were anophthalmic enophthalmic patients and underwent secondary orbital implant insertion and dermo-fat graft. One patient (12.5%) underwent orbital implant insertion and dermo-fat graft simultaneously during the evisceration operation. We followed the progress for 46.3 months. For seven out of eight eyes, the results of the wound healing process were successful. One patient underwent removal and reinsertion of the orbital implant with dermo-fat graft, and the wound in this case healed well. However, after five months, dermo-fat re-graft was performed for orbital implant re-exposure and it was not exposed thereafter. Overall cosmetic appearance was satisfactory in each patient, and all patients were able to comfortably retain a prosthesis. CONCLUSIONS: We found that undergoing dermo-fat graft simultaneously when performing orbital implant insertion is effective for replacement of the conjunctival sac and orbital volume.
Anophthalmos* ; Contracture ; Eye, Artificial ; Humans ; Orbit ; Orbital Implants* ; Prostheses and Implants ; Retrospective Studies ; Transplants* ; Wound Healing ; Wounds and Injuries

PURPOSE: In this study we compared the surface wettability of ocular prosthesis and depositions depending on different types of artificial tear eye drops. METHODS: The artificial tear eye drops contain sodium hyaluronate (HA) 0.1%, 0.18%, 0.3%, carboxylmethylcellulose sodium (CMC), hydroxymethylcelluose + dextran (HMC), propylene glycol + polyethylene glycol (PG), polysorbate 80 (PS) povidone (Pov) were evaluated. Flat rectangular parallelepiped blocks consisting of polymethylmethacrylate (PMMA) or silicone materials were made. One artificial tear eye drop was applied on the surface of two different blocks of artificial eyes using a 23-gauge needle. Then, the static method contact angle was measured by using a contact angle goniometer. To measure the deposits, a petri dish was covered with 3 mL of artificial tear eye drops and dried for 48 hours at room temperature. Then, the light transmittance at the center of the petri dish was measured to investigate the amount of the residue. RESULTS: The contact angles of HA 0.1%, 0.18%, 0.3%, CMC, HMC, PG, PS and Pov on PMMA were 78.69degrees, 84.29degrees, 75.46degrees, 80.93degrees, 66.29degrees, 71.26degrees, 58.40degrees and 70.24degrees, respectively. The contact angles on silicone were 53.68degrees, 60.87degrees, 64.46degrees, 62.78degrees, 38.89degrees, 63.58degrees, 30.68degrees and 51.41degrees, respectively. The largest decrease in transparency was observed in the artificial tear eye drops containing HMC. CONCLUSIONS: The wettability and deposits on the surface of ocular prosthesis can vary based on the components and concentration of artificial tear eye drops. The results from this study should be considered when choosing the right artificial tear eye drops for improving dry eye symptoms in patients wearing ocular prostheses.
Anophthalmos ; Dextrans ; Dry Eye Syndromes ; Eye, Artificial* ; Humans ; Hyaluronic Acid ; Needles ; Ophthalmic Solutions* ; Polyethylene Glycols ; Polymethyl Methacrylate ; Polysorbates ; Povidone ; Propylene Glycol ; Silicones ; Sodium ; Tears* ; Wettability*

PURPOSE: In this study we compared the surface wettability of ocular prosthesis and depositions depending on different types of artificial tear eye drops. METHODS: The artificial tear eye drops contain sodium hyaluronate (HA) 0.1%, 0.18%, 0.3%, carboxylmethylcellulose sodium (CMC), hydroxymethylcelluose + dextran (HMC), propylene glycol + polyethylene glycol (PG), polysorbate 80 (PS) povidone (Pov) were evaluated. Flat rectangular parallelepiped blocks consisting of polymethylmethacrylate (PMMA) or silicone materials were made. One artificial tear eye drop was applied on the surface of two different blocks of artificial eyes using a 23-gauge needle. Then, the static method contact angle was measured by using a contact angle goniometer. To measure the deposits, a petri dish was covered with 3 mL of artificial tear eye drops and dried for 48 hours at room temperature. Then, the light transmittance at the center of the petri dish was measured to investigate the amount of the residue. RESULTS: The contact angles of HA 0.1%, 0.18%, 0.3%, CMC, HMC, PG, PS and Pov on PMMA were 78.69degrees, 84.29degrees, 75.46degrees, 80.93degrees, 66.29degrees, 71.26degrees, 58.40degrees and 70.24degrees, respectively. The contact angles on silicone were 53.68degrees, 60.87degrees, 64.46degrees, 62.78degrees, 38.89degrees, 63.58degrees, 30.68degrees and 51.41degrees, respectively. The largest decrease in transparency was observed in the artificial tear eye drops containing HMC. CONCLUSIONS: The wettability and deposits on the surface of ocular prosthesis can vary based on the components and concentration of artificial tear eye drops. The results from this study should be considered when choosing the right artificial tear eye drops for improving dry eye symptoms in patients wearing ocular prostheses.
Anophthalmos ; Dextrans ; Dry Eye Syndromes ; Eye, Artificial* ; Humans ; Hyaluronic Acid ; Needles ; Ophthalmic Solutions* ; Polyethylene Glycols ; Polymethyl Methacrylate ; Polysorbates ; Povidone ; Propylene Glycol ; Silicones ; Sodium ; Tears* ; Wettability*

PURPOSE: To evaluate the distribution of conjunctival bacterial flora in anophthalmic socket patients with a prosthetic eye, and compare the bacterial positive culture rates between patients with subjective symptoms such as eye wax or irritation and patients without symptoms. METHODS: Twenty-six anophthalmic socket patients with a prosthetic eye who visited our clinic between December 2009 and May 2011 were retrospectively analyzed. The patients were asked about their symptoms, followed by a conjunctiva examination. Specimens were obtained from the inferior conjunctival cul- de- sac with a sterile cotton-tipped applicator. The collected specimens were cultured. RESULTS: The results indicated that the overall positive culture rate in the anophthalmic conjunctival socket was 69.2%, and the predominant organism was S. epidermidis (38.5%). Potential pathogenic bacteria were found in 4 eyes with a 15% positive culture rate. The incidence of bacteria was significantly higher (85.4%) in patient samples with subjective symptoms compared to patients without symptoms (50%). The bacterial positive culture rate of the potential pathogen bacteria in the group with symptoms was higher at 21%, but was not statistically significant. CONCLUSIONS: Performing a pathogen culture test is necessary for prosthetic eye patients who complain of their symptoms. Additionally, the proper antibacterial treatment should be performed according to the antibiotics sensitivity of cultured bacteria.
Anophthalmos ; Anti-Bacterial Agents ; Bacteria ; Conjunctiva ; Eye ; Humans ; Incidence ; Retrospective Studies

PURPOSE: To evaluate the surgical results of lower eyelid retraction using autologous ear cartilage graft. METHODS: Fifty patients (54 eyes) who received surgical correction of lower eyelid retraction by lower eyelid retractors and conjunctiva recession from the tarsal plate with autologous ear cartilage grafts from March 2002 to July 2010 were evaluated. Medical records were reviewed and clinical characteristics, surgical outcomes, and postoperative complications were analyzed retrospectively. RESULTS: The use of prosthesis due to anophthalmos or microphthalmos (22 eyes) was the most common cause of lower eyelid retraction. The mean postoperative follow-up period was 16.6 months (1-98 months). Lower eyelid retraction was successfully corrected in 52 of 54 eyes. Postoperatively, 2 cases of corneal erosions, 1 case of conjunctival erosion, and 2 pyogenic granulomas developed. Corneal and conjunctival erosions resolved with conservative management and granulation tissues were removed by excision. CONCLUSIONS: Correction of lower eyelid retraction using autologous ear cartilage graft is an excellent surgical procedure with low complication rates for eyelid retraction of various etiologies.
Anophthalmos ; Conjunctiva ; Ear ; Ear Cartilage ; Eye ; Eyelids ; Follow-Up Studies ; Granulation Tissue ; Granuloma, Pyogenic ; Humans ; Medical Records ; Microphthalmos ; Postoperative Complications ; Prostheses and Implants ; Transplants

PURPOSE: To evaluate the usefulness of a new technique for the subciliary approach to the surgical management of anophthalmic, shallow inferior fornices. METHODS: Six patients with difficulty retaining an ocular prosthesis, due to anophthalmic, shallow inferior fornices with sufficient conjunctiva, underwent this operation from October 2003 to April 2005. Surgery consisted of the fixation of the conjunctival fornix to the periosteum immediately posterior to the inferior orbital rim through the subciliary approach. This study was prospective. RESULTS: The mean age of the patients was 40.1+/-14.7 years (range, 21~62 years). Three of six patients were male, and three were female. The mean duration of surgical anophthalmos was 16.8+/-13.2 years (range, 0.6~40 years), and the mean follow-up period was 9.3+/-8.4 months (range, 3~25 months). All 6 patients who could not wear prostheses because of shallow fornices could wear ocular prostheses successfully after the operation. An ocular prosthesis could be fitted at a mean duration of 1.83+/-0.75 weeks (range, 1~3 weeks), postoperatively. Only one patient had mild entropion, but there were no conjunctival contractures or loss of fornices postoperatively. CONCLUSIONS: The authors believe that this new technique through the subciliary approach is simple and easy to perform and enables the patient to wear an ocular prosthesis earlier than does forniceal reconstruction through the transconjunctival approach. This technique is very useful in patients with anophthalmic, shallow inferior fornices who have sufficient conjunctiva but loose attachments of the fornix to the underlying tissue, causing shallow fornices.
Anophthalmos ; Conjunctiva ; Contracture ; Entropion ; Eye, Artificial ; Female ; Follow-Up Studies ; Humans ; Male ; Orbit ; Periosteum ; Prospective Studies ; Prostheses and Implants

PURPOSE: To evaluate the clinical effects of conjunctiva-Muller muscle resection through conjunctival incision in anophthalmic patients with mild ptosis. METHODS: Conjunctiva-Muller muscle resection was performed by one surgeon in 8 patients (8 eyes) who had received evisceration or enucleation and responded to 10% phenylephrine solution to correct ptosis. The average age of the patients was 35.87+/-13.4 years. Ptosis was seen from 1 to 34 months after evisceration or enucleation. The preoperative MRD 1 was -2 to 0.5 mm (average: -0.25+/-1.10 mm) and the difference of MRD 1 between before and after 10% phenylephrine use was 2.56+/-0.98 mm. The Muller muscle was resected 7.5 to 9 mm through conjunctival incision during surgery to match the MRD 1 of sound eye. Mean follow-up period after the operation was 2 to 16 months (average: 8.1 months). RESULTS: Postoperatively, the MRD 1 increased by 1.81+/-0.88 mm on the average, corresponding to the improvement in lid elevation after the use of 10% phenylephrine performed before resection. Surgery was successful in most patients, and postoperative difference in MRD 1 was less than 1 mm from the sound eye. No special postoperative complication was observed. CONCLUSIONS: Conjunctiva-Muller muscle resection is one of the effective methods of correcting mild ptosis in anophthalmic patients.
Adult ; Anophthalmos/*complications ; Blepharoptosis/etiology/*surgery ; Conjunctiva/*surgery ; Eyelids/physiopathology ; Facial Muscles/*surgery ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Muscle Contraction ; Ophthalmologic Surgical Procedures/*methods ; Time Factors ; Treatment Outcome