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Analysis of limb reduction defects in babies exposed to chorionic villus sampling.

In 1991 we reported a cluster of babies with limb abnormalities and suggested that chorionic villus sampling (CVS) was aetiologically associated with these defects. To address the issue more objectively, we have assessed reported limb reduction defects in 75 babies exposed to CVS in utero. 13 babies had an absent limb or a defect through the humerus or femur; 9 had defects through the radius or tibia; 22 defects of the carpus, tarsus, metacarpus, or metatarsus; 25 defects of the digits; and 6 defects of the terminal phalanx or nail only. There was a strong correlation between the severity of the defects and the duration of gestation when CVS was done. The median gestational age at CVS ranged from 56 (range 49-65) postmenstrual days for the most severe category to 72 (51-98) days for the least severe. The relation was seen for both isolated limb defects and for cases with oromandibular-limb hypogenesis syndromes. This relation is further evidence that CVS has an aetiological role in some limb reduction anomalies.

Abnormalities, Multiple↗

Solitary spindle-cell xanthogranuloma of the eyelid.

PURPOSE: To describe a rare presentation of a solitary spindle-cell xanthogranuloma in a 3-year-old boy with a 6-month history of a right upper lid soft tissue lesion. DESIGN: Interventional case report. METHODS: An excisional full-thickness biopsy of the right upper lid was performed. Histologic examination and immunohistochemistry of the specimen were performed. RESULTS: Histopathologic evaluation revealed a well-demarcated nodule arising from the tarsus that contained a storiform array of spindle cells admixed with numerous multinucleated giant cells (some Touton type) and lymphocytes. The spindle cells labeled with antibodies to Factor XIIIa, CD68, lysozyme, Mac 387, and vimentin. The multinucleate giant cells labeled with CD68, lysozyme, and vimentin. Findings were consistent with the diagnosis of solitary spindle-cell xanthogranuloma. CONCLUSIONS: Spindle-cell xanthogranuloma resembles juvenile xanthogranuloma and fibrous histiocytoma (dermatofibroma) and should be considered in the differential diagnosis of spindle-cell tumors of the eyelid and periocular region. If similar lesions are noted elsewhere, the diagnosis of progressive nodular histiocytoma should be considered.

Biomarkers↗

Topical mitomycin-C for pagetoid invasion of the conjunctiva by eyelid sebaceous gland carcinoma.

PURPOSE: To evaluate the efficacy of topical mitomycin-C for pagetoid invasion of the conjunctiva by sebaceous gland carcinoma. DESIGN: Prospective noncomparative consecutive interventional case series. INTERVENTION: All patients received topical 0.04% mitomycin-C four times daily for 1 week followed by 1 week off medication. The treatment cycles were repeated until resolution of the conjunctival malignancy was clinically evident. PARTICIPANTS: Four patients with histopathologically proven intraepithelial (pagetoid) invasion of the conjunctiva by sebaceous gland carcinoma were managed with this regimen. RESULTS: The mean patient age was 78 years. Before treatment, the main tumor site included the upper eyelid in two cases and the lower eyelid in two cases. Previous resection of the tumor had been performed elsewhere in three cases over the prior 6 years. At the time of our examination, map biopsies confirmed pagetoid invasion involving 25% to 90% of the conjunctival surface, with bulbar, forniceal, and tarsal conjunctival involvement in all four cases and corneal extension in one case. There was no evidence of deep tumor within the conjunctival stroma or tarsus in any case. After treatment, medication intolerance and early discontinuation occurred in one patient, and continued tumor progression was documented. Of the remaining three patients, chemotherapy was used for a mean of four cycles with complete resolution of the pagetoid invasion, confirmed histopathologically in two cases, and without recurrence in all three cases over 12 months (mean) follow-up. The medication caused moderate temporary local irritation but no serious intraocular or extraocular complications. CONCLUSIONS: Preliminary evidence suggests that topical mitomycin-C is effective treatment for pagetoid invasion of the conjunctiva by sebaceous gland carcinoma. Longer follow-up is necessary to assess the duration of tumor control.

Adenocarcinoma, Sebaceous↗

Slow-release artificial tears and the treatment of keratitis sicca.

The slow-release artificial tear (SRAT) is a biodegradable, cellulosic polymer without preservative which is inserted beneath the tarsus of the lower lid, and which provides continuous lubrication and tear film stability to the eye. The SR-AT has been shown to reduce both subjective symptoms and objective signs in patients with keratitis sicca, and is a valuable adjunct to therapy in patients with this disease.

Cellulose↗

Practical ptosis surgery. The Wendell L. Hughes Lecture.

The author has emphasized the concept that ptosis surgery should be done in a way that may be modified in the event that the immediate result is not satisfactory. The value of local injection plus fractional anesthesia in the adult allows the surgeon to solicit the aid of the patient to evaluate the result on the table more accurately. A regional block is unsatisfactory because the nerve supply to the levator muscle may be knocked out and titration of the result would not be possible at the time of surgery. The subaponeurotic tunnel without incising the conjunctiva makes a better anatomic dissection of the aponeurosis. The attachment to the tarsus of the advanced aponeurosis 2 to 3 mm from the ciliary border gives the surgeon a leeway of approximately 7 to 8 mm to recess in the event of an overcorrection. In the event of an undercorrection, the aponeurosis is further advanced and reattached by the three vertical mattress sutures.

Adult↗

Partial and total reconstruction of the lower eyelid.

Our techniques for reconstruction of the lower lid are based on the maintenance of tarsal support in the reconstructed lid. The surgical approach is dictated by the position and extent of the defect produced by tumor excision. Lysis of the extension of the lateral canthal ligament to the lower lid allows closure of small, full-thickness defects. Mobilization of tarsal remnants by temporal advancement flaps provides for closure of larger defects. An advancement flap of split-thickness upper lid tarsus is combined with a pedicle flap of skin from the upper lid for total lower lid reconstruction.

Eyelid Neoplasms↗

The histopathology of involutional ectropion.

Twenty eyelid specimens from patients with involutional ectropion, obtained by full-thickness horizontal shortening procedures, were examined histopathologically by light and electron microscopy and compared with six normal eyelids from exenteration specimens. Paralytic, cicatricial, and congenital ectropions were excluded from the study. The main histopathologic features included: (1) collagen degeneration and elastosis of the tarsal plate; (2) increased amounts of adipose tissue in the distal tarsus and capsulopalpebral fascia; (3) subacute inflammation and epidermidalization of the tarsal conjunctiva; (4) focal degeneration, fibrosis and elastosis of pretarsal orbicularis, and occasionally minimal change in the muscle of Riolan; and (5) arteriosclerosis of the marginal artery. The combination of these histopathologic changes characterize and may contribute to the development of ectropion of the eyelid associated with aging.

Aged↗

Modified corncrib (inverted T) procedure with Quickert suture for repair of involutional entropion.

PURPOSE: The purpose of the study was to determine long-term results of involutional entropion repair by a modified corncrib (inverted T) procedure with a Quickert suture. METHODS: A retrospective review of consecutive patients with involutional entropion who underwent repair by a modified corncrib procedure from January 1986 to July 1994 was performed. A full-thickness basedown triangle (corncrib) excised at the lateral limbus corrects horizontal laxity and everts the eyelid margin. The stem of the T is represented by the vertical closure of the eyelid margin and the tarsus. The top of the T is represented by the horizontal wound at the lower tarsal border after excision of an ellipse of skin and preseptal orbicularis muscle medial and lateral. The resultant horizontal scar prevents overriding of preseptal over pretarsal orbicularis oculi muscle. A translid double-armed Quickert suture preplaced 2 to 3 mm lateral to the punctum plicates the dehisced lower eyelid retractors and also prevents overriding of preseptal over the pretarsal orbicularis muscle. This suture pierces the conjunctival cul-de-sac and exits the skin just anterior to the lashes. RESULTS: In 63 eyelids of 59 patients (4 bilateral cases), there were no failures with a median follow-up of 49.3 months. Complications were minimal. CONCLUSIONS: The modified corncrib (inverted T) procedure is performed through a single surgical wound, requires minimal surgical dissection, and provides excellent long-term results.

Aged↗

Correlation of goblet cell density and mucosal epithelial membrane mucin expression with rose bengal staining in patients with ocular irritation.

PURPOSE: This study was designed to compare goblet cell densities and mucosal epithelial membrane mucin (MEM) expression in impression cytology specimens obtained from control subjects and patients with one of the following clinically defined diseases: aqueous tear deficiency (ATD) associated with Sjögren syndrome, ATD not associated with Sjögren syndrome, inflammatory Meibomian gland disease associated with rosacea, and Meibomian gland atrophy. These data were correlated with ocular surface rose Bengal staining scores, Schirmer scores, and HLA-DR antigen staining of conjunctival epithelial cells. METHODS: Goblet cell density and MEM expression were studied by impression imprints with immunohistochemical staining using an anti-mucosal epithelial membrane mucin antibody in the temporal and inferior bulbar and inferior tarsal conjunctiva of study subjects. RESULTS: Goblet cell density adjacent to the temporal limbus was significantly reduced at 3 mm posterior to the temporal limbus in both aqueous tear deficiency groups compared with the other groups and in patients with Sjögren syndrome compared with all other groups. In the inferior tarsus, goblet cell density was significantly reduced in patients with non-Sjögren syndrome ATD as compared with all other groups, except those with inflammatory Meibomian gland disease. Mucosal epithelial membrane mucin expression in the bulbar and tarsal conjunctiva was absent in a greater percentage of patients with Sjögren syndrome compared with all other groups. Total ocular surface rose Bengal staining scores were significantly higher in patients with Sjögren syndrome as compared with all other groups and in patients with non-Sjögren syndrome ATD as compared with control groups. Rose Bengal staining scores and Schirmer I test results (without anesthesia) were inversely correlated with bulbar, but not tarsal, conjunctival goblet cell densities, and with the absence of bulbar conjunctival MEM expression. CONCLUSIONS: These results suggest that reduced goblet cell density and mucosal epithelial cell mucin expression could explain increased rose Bengal staining in patients with aqueous tear deficiency. In addition, MEM may be regarded as a marker for normal differentiation of ocular surface epithelia, with its absence signifying the development of squamous metaplasia.

Adult↗

Meibomian gland dysfunction in patients with Sjögren syndrome.

OBJECTIVE: Changes in the ocular surface of patients with Sjögren syndrome (SS) often are more severe than those in patients with dry eye without SS. This study was conducted to investigate the possible involvement of meibomian gland dysfunction in SS-related ocular surface abnormalities. DESIGN: A nonrandomized, prospective, clinical study. PARTICIPANTS: Twenty-seven eyes of 27 consecutive patients with SS (SS group) were studied. Twenty-nine eyes of age- and gender-matched non-SS patients with aqueous tear deficiency (non-SS group) were examined as control subjects. INTERVENTION: Changes in the ocular surface, tear function, and meibomian gland were examined. MAIN OUTCOME MEASURES: Tear evaporation rate, meibomian gland expression, and meibography were measured. RESULTS: Fluorescein and rose bengal staining scores were significantly higher in the SS group than in the non-SS group (P = 0.0001). Evaporation of tears was increased significantly in the SS group compared with the non-SS group. There were no significant differences in the rate of tear production between the SS and non-SS groups. Meibography showed that 11 (57.9%) of 19 eyes in the SS group had gland dropout (i.e., histologic destruction of meibomian glands) in more than half of the tarsus. The incidence was significantly higher than that in the non-SS group (5 [18.5%] of 27 eyes; P = 0.005). CONCLUSIONS: The results of this study indicate that destruction of meibomian glands and an increase in tear evaporation often are associated with changes in the ocular surface in patients with SS. Severe ocular surface changes in patients with SS may be attributed, in part, to the meibomian gland dysfunction.

Eyelid Diseases↗

[Ptosis treated by resection of the Muller muscle: analysis of a series of 51 patients].

OBJECTIVE: Patients with blepharoptosis caused by disinsertion of the levator aponeurosis or Horner syndrome require surgical repair. Often, the motivation is mainly cosmetic. It is therefore important to obtain a harmonious result with overall symmetry of the palpebral aperture, the level of the lid crease, and the lid contour. The Muller muscle conjunctival resection, as described by Putterman, is very effective for raising the lid. However, its use is tricky when a Hering dependence exists or when the phenylephrine test does not provide a symmetrical opening. Fifty-one consecutive patients who underwent a Muller muscle-conjunctival resection by a single surgeon between October 2003 and January 2005 (64 eyelids) were retrospectively analyzed in order to refine the technique and indications by answering four questions: Is the phenylephrine test 100% reliable? In case of under- or overcorrection with the phenylephrine test, can surgery be graduated in its effects? When a Hering dependence occurs, is it always detected by the phenylephrine test? Is this technique more predictable than alternative techniques? PATIENTS AND METHOD: Objective criteria (measurement of the palpebral aperture and the margin reflex distance [MRD]) and subjective criteria, much more accurate because they take into account the lid contour and the position of the lid crease, were used with photographs. The technique was used as described by Putterman. RESULTS: A reduction of the ptosis with an asymmetry inferior or equal to 1mm was obtained in 90% of the cases. According to the subjective criteria, 56% were classified as excellent with a perfect symmetry, 32% were classified as good with a slight asymmetry but without any cosmetic impact, 10% were classified as average, with a visible imperfection, and 2% (one patient) were classified as poor, resulting from an obvious cosmetic imperfection with an irregular contour requiring reoperation. In 55% of the cases, the palpebral aperture was at less than 0.5 mm of that obtained with the phenylephrine test. In 21% of the cases, the position was quite unexpected, with a gap of more than 1mm. In 14% of the results, the level was inferior to the test because of a voluntary limited resection. In 9% of the cases, a bilateral and symmetrical drop occurred because of the relaxation of the frontalis muscle after the operation. A Hering dependence was found in 46% of unilateral cases with a drop of the contralateral eyelid inferior or equal to 1mm (2mm in one case only). Among these cases, 62% were predictable with the phenylephrine test; in one case, a drop foreseen by the test did not occur. When the test produced an overcorrection, the resection was lowered by 1 or 2mm, which worked well in 10 cases out of 12. When the test produced an undercorrection, the resection was at its maximum (10 mm), but was efficient in only one out of six cases. CONCLUSION: In 21% of the cases, the phenylephrine test was not efficient in predicting the surgical result. A resection of a part of the tarsus is necessary in case of undercorrection with the phenylephrine test. Almost half of the of the cases with a Hering dependence remained undetected after the test. The Muller's muscle resection give a better result than the classical aponeurosis shortening with an anterior cutaneous approach. However, it is absolutely essential, in order to select patients and to measure surgical process, to put into effect the phenylephrine test with strict identical requirements to detect the bilateral and asymmetrical forms and a possible Hering dependence, and to standardize the surgical technique, developing one's own graphs through the analysis of postoperative results.

Adolescent↗

Transarticular application of external skeletal fixation.

External skeletal fixators provide a useful alternative to external coaptation techniques for immobilization of joints in selected patients. This is especially true when the orthopedic injury involves an open wound requiring daily treatment. Devices such as the K-E splint, the Rudy external fixator boot, and acrylic frame fixators are economical and effective for transarticular fixation in small animal patients. Clinical indications for these techniques have included fixation of tibial or radial fractures with a short distal fragment; arthrodesis of the elbow, carpus, stifle, or tarsus; protection of Achilles tendon repairs; protection of collateral ligament repairs of the tarsocrural joint; and protection when multiple ligaments of the stifle joint have been reconstructed. Use of contoured rods has facilitated transarticular application of type II Kirschner-Ehmer splints. Use of acrylic frames and the Rudy boot technique have extended safe use of transarticular external fixation to extremely small patients not accommodated by the Kirschner system alone. An understanding of the advantages and disadvantages inherent in each of these techniques is critical to proper selection of the best method for a given patient. Attention to the basic principles of pin selection, pin insertion, and frame design are needed for successful execution of the chosen technique.

Animals↗

Idiopathic multicentric osteolysis principally affecting the phalanges of the hands and feet.

The case of a 64-year-old man with idiopathic symmetrical osteolysis affecting both hands and feet is presented. The phalanges were principally affected, with relative sparing of the carpus and tarsus. The relevant literature has been reviewed and this reveals that the distribution of osteolysis in this case is unique. We suggest that this case represents a different disease entity, which has not been described previously.

Fingers↗

Effects of heavy metal exposure on the condition and health of nestlings of the great tit (Parus major), a small songbird species.

In this study we examined the possible effects of heavy metal exposure on the condition and health of great tit nestlings (Parus major) at four study sites along a pollution gradient near a large non-ferrous smelter in Belgium during three consecutive breeding seasons. Our results showed that nestlings were indeed exposed to large amounts of heavy metals. Excrements contained significantly higher concentrations of several heavy metals (silver, arsenic, cadmium, mercury, lead) near the pollution source than at study sites farther away. When taking into account the number of young in the nest at the time of sampling, nestling body mass and condition were significantly reduced at the most polluted site. Nestlings at the two most polluted sites fledged significantly later than at the least polluted site. We also observed growth abnormalities of the legs near the pollution source. Tarsus length, wing length and haematocrit values did not differ significantly among study sites.

Air Pollutants↗

Use of fetal biometry to determine fetal age in late pregnancy in llamas.

Sixty-three pregnant llamas of known breeding date were used in this study. Forty-six of them were submitted to surgery between 186 and 320 days of gestation (52-91% of average gestation period, respectively). Under general anesthesia their fetuses were exteriorized and fetal weight (W), biparietal diameter (BPD) and femoral (F), tarsus-hoof (T-H), tibial (T)) and fronto-occipital (F-O) length were determined. Additionally, the same variables were determined on 16 newborn llamas. The weight was measured in kg and the length in cm. All the collected data was entered into a spreadsheet and different regression analyses as a function of gestational age (GA) were assessed. The best fit equations and their correlation for linear regression were the following: GA=169.448+16.66(*)W, r=0.99; GA=-51.713+44.77(*)BPD, r=0.88; GA=-72.139+39.48(*)F-O, r=0.71; GA=39.304+8.35(*)T-H, r=0.97; GA=91.276+8.23(*)T, r=0.86; GA=102.029+9.94(*)F, r=0.91. For multiple regression, the dependent variable GA can be predicted by the following equation: GA=67.462+11.163(*)W+20.297(*)BPD. Results of the present study indicated measured variables to be highly correlated with GA. This could be useful on daily basis in clinical examination of the neonates, in assessment of fetal growth and well being with cesarean sections, in the determination of GA in late gestation abortions, and in perinatal and reproductive research in the llama.

Animals↗

Osteochondrosis in cattle.

Osteochondrosis is a poorly understood condition that affects many species, including cattle. Its incidence is not fully appreciated in the cattle industry for many reasons. Most of the reports show a male, purebred predilection, but this may simply be a result of the typical population for which medical attention is sought. With the increasing value of female breeding stock, a less skewed distribution of the disease will most likely result. Distribution of lesions is similar to that in the horse, with the stifle and tarsus most commonly affected. Treatment is divided into medical and surgical options, but neither seem to be strikingly effective as of yet. Surgical techniques include arthroscopy and arthrotomy, and both have their complicating factors. How osteochondrosis as a disease will affect the cattle industry is still to be decided. If the incidence rate does increase with more animals being valued for their economic potential, then treatment options will surely be reevaluated and refined.

Animals↗

Treatment of proximal hind-limb lameness in cattle.

Lameness of the hind limb (tarsus and proximal) in cattle is considered from the viewpoint of diagnosis and prognosis. Indications and techniques for radiography are discussed, as well as when indicated.

Animals↗

Expression patterns of aristaless in developing appendages of Gryllus bimaculatus (cricket).

We report the isolation and expression patterns of aristaless (al), a paired-type homeobox gene, of Gryllus bimaculatus (Gb), a hemimetabola model insect. Gryllus al (Gbal) is expressed in the most distal region of developing labrum, antenna, mandible, maxilla, labium, leg, cercus, and hindgut. Gbal is also expressed in the proximal region, corresponding to the presumptive coxopodite, of the developing antenna, mandible, maxilla, labium, and leg, but not in the developing labrum, cercus, and hindgut. During development of the leg, expression of Gbal changes dynamically with the progress in leg segmentation: Gbal is expressed in order in the presumptive pretarsus, coxa, femur, tibia and tarsus before appearance of morphological segmentation.

Amino Acid Sequence↗