The surgical management of orbital neurofibromatosis

Orbitofacial neurofibromatosis may cause severe soft-tissue deformity along with pulsating exophthalmos. The orbit is enlarged, and the greater wing of the sphenoid is absent. The eye in some cases may be functionally useless, immobile, displaced, and blind. In these, orbital exenteration with soft-tissue excision and orbital reconstruction is recommended. External cover is provided by the thin eyelid skin, and a prosthesis ultimately allows a very satisfactory end result. Four such cases with a minimum of 2 years of follow-up are presented.     

Treatment of exophthalmos

Current procedures for Graves' exophthalmos fail to achieve complete correction. The standard orbital decompressions were therefore modified to maximize the degree of volumetric increase behind the axis of the globe. In 15 orbits, the preoperative exophthalmos averaged 9.5 mm, whereas the postoperative exophthalmos was 4.1 mm. Postoperative CT study demonstrated that the remaining posterior orbital wall, combined with the persistently increased intraocular muscle volume, blocked retrodisplacement of the globe, despite adequate total volumetric increase. The increased muscle volume varied from 2 to 5 cc. Despite this residual exophthalmos, the modified four-wall expansion provides excellent aesthetic results with visual improvement and resolution of chemosis and exposure keratitis.     

Orbital floor and infraorbital rim reconstruction after total maxillectomy using a vascularized calvarial bone flap.

A number of techniques have been introduced to support the orbital floor after maxillectomy without orbital exenteration. These methods include skin graft or muscular sling, but they have resulted in severe complications, such as enophthalmos, global ptosis, diplopia, and facial deformity. Currently, advanced microvascular reconstruction using bone and soft tissue is performed by many surgeons. This usually results in the filling of the postmaxillectomy defect, but the lack of support for the orbital rim and floor by the bone flap may still cause the complications mentioned above. Vascularized calvarial bone flap was chosen in this study for reconstruction of the orbital floor and infraorbital rim to function as a buttress, to reconstruct recipient sites of poor vascular bed after radiation therapy, and to withstand further postoperative radiation. By providing a solid floor and rim, these complications can be prevented with satisfactory function and aesthetically acceptable results. From September of 1995 to July of 1998, we performed vascularized bone flap for the reconstruction of the orbital floor and infraorbital rim in four cases after total maxillectomy involving the orbital floor. With a follow-up period from 19 to 35 months (mean, 27 months), we obtained significant improvement of functional and aesthetically acceptable results without global ptosis, enophthalmos, diplopia, or severe facial contour deformity.     

Surgical reconstruction of the contracted orbit

Anophthalmic patients and patients afflicted with retinoblastoma incur severe deformity of the orbit. Treatment of the severely contracted orbit is very difficult, and patient satisfaction is often poor. Since 1988, we have performed temporalis muscle transfer and surgical expansion of the contracted bony orbit in 26 patients. Satisfactory results were obtained. Gradual expansion of the orbit was performed in case of congenital anophthalmic patients. The treatment should be established in multiplicity, among many methods available for contracted eye sockets, according to the degree of orbital deformity and the amount of residual conjunctiva. In case of severe deformity, volume expansion surgery and temporalis muscle transfer are necessary. If augmentation is required in the periorbital region, rib bone onlay graft must be performed. We were able to shorten the operative time by modifying the three-wall orbital expansion technique of Tessier and Wolfe to a more simplified method. Our observations show that our procedures achieved symmetry in both eyes in all patients, and there have been no remarkable complications.     

Surgical treatment of thyrotoxic exophthalmos

Conservative treatment of thyrotoxic exophthalmos has not given satisfactory results. Our observations, modifications of the standard surgical technique, and the results of orbital decompression for this condition are presented. Through a transverse incision close to the lower eyelid margin, the floor and the lateral orbital wall are explored. The posterior part of the orbital floor and the zygomatic part of the lateral orbital wall, as well as the periorbital fat, are removed. Through an incision made over the medial margin of the orbit, the medial orbital wall is explored and its ethmoidal part is removed. By the same approach, further retrobulbar fat is removed. Through an upper eyelid incision, fat is removed from the eyelid region and the levator aponeurosis is divided. This produces satisfactory symmetrical decompression of the orbit with good correction of exophthalmos and a significant decrease in the signs and symptoms of this condition.     

The D Trisomy Syndrome: A Case Report with a Description of Ocular Pathology

A male infant with trisomy of one of the 13-15, or D, group of autosomes is described. The infant survived for only a few hours and had the following congenital abnormalities: defect of the scalp and underlying vertex of the skull, hemangioma, microphthalmia, cleft palate, ulnar deviation of forearms and wrists with flexion deformity of the fingers, polydactyly of the feet, interauricular septal defect, cryptorchidism and polycystic kidneys. Retinal dysplasia was the major abnormality in the microphthalmic eye.The congenital anomalies caused by D trisomy are sufficiently characteristic for the chromosomal error to be suspected in a newborn infant. A chromosome analysis is advisable for confirmation of the diagnosis.     

Correction of severe facial deformity.

A craniofacial team has been developed to corrdinate the treatment of patients with severe facial deformity and to minimize the surgical risks. Two hundred patients have been evaluated in the last 4 years and more than 100 treated by this team to correct orbital hypertelorism, oxycephaly and plagiocephaly with exophthalmos, features of Crouzon''s disease and Apert''s and Treacher Collins syndromes, hemifacial microsomia, and severe lower facial deformity and malocclusion. Surgical principles include extensive subperiosteal stripping of bone, osteotomy as necessary, and repositioning maintained by bone grafts. Postoperative evaluation is being maintained for 5 years or to maturity in younger children. Although many of the results are less than perfect, there has been sufficient improvement, especially psychologic and functional, to warrant continuation of the surgical program under closely controlled conditions.     

生物相容性MEDPOR仪眼治疗眼窝塌陷畸形

目的：探讨眼窝塌陷畸形的重建手术方法和临床效果,方法：对42例无眼球或眼球萎缩伴眼窝塌陷形的患者,行高密度多孔聚乙烯（MEDPOR）义眼座植入联合穹隆成形术,结果：所有患者眼窝畸形均得以矫正,随访6个月－3年,义眼座在眼眶内无脱出,移位或合并感染,装入仿真义眼片后,双眼对称,义眼活动度可达10－20度,结论：MEDPOR义眼座植入联合穹隆成形术矫治复杂性眼窝畸形在总体上取得了良好的效果,MEDPOR义眼具有良好的组织相容性,是矫正眼窝塌陷畸形的理想材料。     

Modified three-wall orbital expansion to correct persistent exophthalmos or exorbitism.

It is believed that a variation of the 3-wall orbital expansion of Tessier can be used to correct persistent exophthalmos, as well as exorbitism. The operation is described, and the results in 3 patients are shown.     

EXOPHTHALMOS—From the Standpoint of the Otolaryngologist

Exophthalmos may be due to an inflammatory process or to tumor formation. Inflammatory processes are most likely to occur in children and young adults. Tumors are the most common cause of exophthalmos in adults.Since the advent of chemotherapy and the antibiotics, rarely does orbital cellulitis develop from sinal infection.Tumors causing exophthalmos are likely to be benign if they arise from the frontal sinus and malignant if they arise from the maxillary and ethmoid sinuses.     

Exophthalmos from the standpoint of the otolaryngologist

Exophthalmos may be due to an inflammatory process or to tumor formation. Inflammatory processes are most likely to occur in children and young adults. Tumors are the most common cause of exophthalmos in adults.Since the advent of chemotherapy and the antibiotics, rarely does orbital cellulitis develop from sinal infection. Tumors causing exophthalmos are likely to be benign if they arise from the frontal sinus and malignant if they arise from the maxillary and ethmoid sinuses.     

3D-Assisted Quantitative Assessment of Orbital Volume Using an Open-Source Software Platform in a Taiwanese Population

Orbital volume evaluation is an important part of pre-operative assessments in orbital trauma and congenital deformity patients. The availability of the affordable, open-source software, OsiriX, as a tool for preoperative planning increased the popularity of radiological assessments by the surgeon. A volume calculation method based on 3D volume rendering-assisted region-of-interest computation was used to determine the normal orbital volume in Taiwanese patients after reorientation to the Frankfurt plane. Method one utilized 3D points for intuitive orbital rim outlining. The mean normal orbital volume for left and right orbits was 24.3±1.51 ml and 24.7±1.17 ml in male and 21.0±1.21 ml and 21.1±1.30 ml in female subjects. Another method (method two) based on the bilateral orbital lateral rim was also used to calculate orbital volume and compared with method one. The mean normal orbital volume for left and right orbits was 19.0±1.68 ml and 19.1±1.45 ml in male and 16.0±1.01 ml and 16.1±0.92 ml in female subjects. The inter-rater reliability and intra-rater measurement accuracy between users for both methods was found to be acceptable for orbital volume calculations. 3D-assisted quantification of orbital volume is a feasible technique for orbital volume assessment. The normal orbital volume can be used as controls in cases of unilateral orbital reconstruction with a mean size discrepancy of less than 3.1±2.03% in females and 2.7±1.32% in males. The OsiriX software can be used reliably by the individual surgeon as a comprehensive preoperative planning and imaging tool for orbital volume measurement and computed tomography reorientation.     

Congenital isolated absence of the nasal columella: reconstruction with an internal nasal vestibular skin flap and bilateral labial mucosa flaps

Congenital aplasia of the nasal columella is a very rare anomaly. The deformity is characterized by the isolated absence of the columella from the nasal tip to the root of the philtrum, including the medial crura of the alar cartilages; surrounding structures such as the septum, nose, and upper lip are normal. To the best of our knowledge, only four such cases have been described to date. The embryopathogenesis for this uncommon disease is presently unknown. Our report describes a 14-year-old girl with congenital agenesis of the columella as an isolated anomaly. Her family history was positive for the presence of the same congenital deformity, which also affected her older brother; there was, however, no consanguinity between the parents. The columella defect was reconstructed with an internal nasal vestibular skin flap and bilateral upper labial mucosa flaps. There are many techniques available to repair columella defects, including free grafts from the ear, local flaps from the forehead, face, upper lip, and nose, distant flaps such as tube pedicle flaps, and free flaps from the ear. Each of these techniques has advantages and disadvantages. Because of this, the treatment of columella defects should be individualized.     

Pseudo-iatrogenic hypospadias: the megameatus intact-prepuce hypospadias variant

This article presents the authors' experience with 21 patients with the megameatus variant of hypospadias who were treated during an 8-year period. In nine of the cases, the parents were convinced that the defect was a complication of circumcision, and the patients were examined in consultations in preparation for litigation. Seven of those nine patients had been previously examined by either a plastic surgeon or a urologist, who failed to recognize this variant. The typical appearance of the defect and how to differentiate this congenital deformity from true iatrogenic hypospadias are described. The features of the megameatus intact-prepuce variant of hypospadias include a wide spatulated glans, a deep groove, a large wide patulous meatus at the subglanular groove, an intact prepuce before circumcision, no evidence of glanular scarring, and no history of bleeding at the time of circumcision.     

Monosomy 20p due to a de novo del(20)(p12.2). Clinical and radiological delineation of the syndrome

A 16 year-old boy with monosomy 20p was studied. The clinical and radiological data compared with those from the three previously reported cases, permit the delineation of a distinct syndrome of low birthweight, flat face, low nasal bridge, long philtrum, short neck, small overfolded ears, chest deformity, kyphoscoliosis, congenital heart defect, hypoplastic or absent ribs and rachischisis (butterfly-shaped vertebral bodies). The critical chromosome segment causing this syndrome is tentatively defined as 20p13.     

Split-thickness skin grafting of the myelomeningocele defect: a subset at risk for late ulceration

The appropriate method and timing of the management of the myelomeningocele defect have prompted considerable discussion. Use of split-thickness skin grafts acutely has accomplished wound closure with low morbidity and mortality. This study was designed to address the question of long-term suitability of the technique of split-thickness skin grafting of the myelomeningocele patient. The incidence of late and/or severe skin ulceration and the presence of gibbus deformity were correlated with the method of skin closure. Long-term follow-up revealed a higher incidence of chronic skin ulceration in the split-thickness skin graft group as compared with the primary closure group. All skin breakdowns appeared in the presence of a gibbus deformity, and gibbus deformity was more prevalent in the split-thickness skin graft group. The incidence of skin ulceration and gibbus deformity was site-dependent. A thoracic or thoracolumbar myelomeningocele repair with split-thickness skin graft was significantly more likely to be complicated by skin problems than the defect in the lumbar, lumbosacral, or sacral region. This relationship was secondary to the frequency of gibbus deformity in the more cephalad defects than defects caudad. A treatment plan is outlined that is based on the primary variable of the location of the myelomeningocele and secondarily by defect size.     

Assessment of intraorbital structure volume using a numerical segmentation image technique (NSI): the fatty tissue and the eyeball

INTRODUCTION: Measurement of the degree of exophthalmos is one of the main methods used in the assessment of pathological processes that occur in the orbital space and is widely used. However, this only provides initial information about the volume relations between the intraorbital structures. The aims of our work were as follows: to draw up a new computer application, namely the numerical segmentation image (NSI) technique, for the automatic calculation of the volume of the intraorbital structures on the basis of magnetic resonance imaging (MRI) images, to determine its usefulness in the segmentation of fatty tissue and the eyeball and to estimate their volume in relation to the degree of exophthalmos. MATERIAL AND METHODS: A total of 45 patients (90 orbits) were included in the study. All the patients underwent MRI examination of the orbits by a 1.5 T scanner using a head coil. The degree of exophthalmos was determined clinically and radiologically in relation to the interzygomatic line. Quantitative assessment of the eyeball and fatty tissue was made using an NSI application. RESULTS: The influence of fatty tissue volume on the degree of exophthalmos was determined as being statistically significant (r = 0.367, p = 0.000374) but was smaller in comparison with the relationship between total eye muscle volume and degree of exophthalmos; eyeball volume was found to have the least influence (r = 0.344, p = 0.000374). Two eyeballs of significantly smaller volume were found in the group of 90 orbits analysed. CONCLUSIONS: The NSI technique is a clinically useful application, providing objective data calculated individually for each orbit. A credible protocol for estimating the degree of exophthalmos on the basis of the NSI technique should include the eye muscle volume, fatty tissue volume and, in cases where eyeball pathologies coexist, the eyeball volume as well.     

Surgical correction of Tessier number 8 cleft

The number 8 Tessier cleft can be a discrete horizontal shadow at the level of the lateral canthus of the palpebral fissure or a true coloboma with absence of the commissure between the upper and lower eyelids. A surgical technique, which has been used in eight patients, is described to correct this congenital defect. Four flaps are created and transposed as two Z-plasties. The lateral canthal ligament is fixed to the lateral orbital rim, and the orbicularis muscle is interdigitated to restore its continuity. This procedure reconstructs the depth of the conjunctival fornix, provides proper form and length to the palpebral fissure, and restores continuity and an anatomic angle to the malformed canthus.     

A stereotactic system for guiding complex craniofacial reconstruction.

A stereotactic system has been designed to address the problem of achieving symmetry in complex and extensive craniofacial defects. Preliminary testing suggests that such a system, which allows for the intraoperative application of preoperative CT planning, will be useful in guiding the reconstruction of congenital or acquired bony time, is being used to investigate the correlation of intraoperative globe position following enophthalmos correction with long-term outcome, particularly as it relates to the size and location of the orbital defect, and the timing of the procedure.     

An unusual presentation of constriction band syndrome

Congenital constriction band syndrome is a rare entity with a wide spectrum of associated congenital anomalies. Review of the pathogenesis and an unusual case of constriction band syndrome in a newborn are presented. Surgical excision of the deformity and the band was performed within the first week of life. There were no vascular or neural structures within the excised tissue, and there were no other associated anomalies other than dextrocardia and an equinovarus deformity of the foot. The wound was closed primarily without the need for Z-plasties. This alternative method of treatment can be considered in such unusual locations of constriction band syndromes.