Extending the reach of the heterodigital arterialized flap by vein division and repair

The heterodigital arterialized flap is ideal for nonsensory reconstruction of sizable soft-tissue defects in the proximal fingers, web spaces, and the hand. The inclusion of a dorsal vein augments the venous drainage of this digital island flap and avoids the problem of postoperative venous congestion, which is a common problem in digital island flaps. However, the presence of a dorsal vein pedicle inhibits flap mobility somewhat, and the reach of the flap is mainly limited to adjacent fingers. In situations that demand a transfer from a nonadjacent donor finger or when the reach from the adjacent donor finger is inadequate, the dorsal vein pedicle can be temporarily divided and then anastomosed microsurgically after flap transfer is performed. This enables the reach of the flap to be extended up to two fingers from the donor finger. The authors performed this "partially free" heterodigital arterialized flap in 11 consecutive patients between 1991 and 2001. The average size of the defects was 4.4 x 2.3 cm. All of the flaps survived completely, without any evidence of postoperative flap congestion. Healing of all of the flaps was primary and did not result in any scarring. All of the donor fingers had "normal" two-point discrimination of 3 to 5 mm. All of the donor fingers retained excellent or good total active motion, as graded by the criteria of Strickland and Glogovac.     

Effect of lacrimal plugs combined with deproteinized calf blood extract eye gel for filamentary keratitis

This study aims to investigate the efficacy of lacrimal plugs combined with deproteinized calf blood extract eye gel on the treatment of dry-eye-associated filamentary keratitis. Lacrimal plugs were inserted into both the upper and lower puncta of 15 patients (28 eyes). Deproteinized calf blood cxtract eye gel was applied in two patients who were not cured after this operation. All patients were asked to complete three questionnaires 1 day before the surgery and at 1 week and 1 month after the surgery. Ophthalmologic examinations were carried out and repeated at 1 day and at 1 week and 1 month after plug insertion, which include fluorescein staining, tear break-up time (BUT), Schirmer test I (STI), corneal confocal microscope (HRT-III), and impression cytology (IC). Symptoms were relieved in all patients 1 month after the application of lacrimal plugs. Deproteinized calf blood extract eye gel was applied to two patients whose symptoms remained after lacrimal plug implantation for 1 week. At 3 weeks later, the symptoms disappeared and cornea fluorescein stain was hardly identified. The lacrimal river in all patients became broader after the surgery, 11 of whom reached 0.3 mm. Cornea filamentary disappeared in all patients. The average of BUT and ST were increased to 7.50 ± 1.897 s (p < 0.001) and 8.12 ± 1.996 mm at 1 week after the operation and 7.94 s and 9.00 ± 1.897 mm at 1 month later, respectively. Photography from HRT-III suggested that the state of corneal epithelium was markedly improved, including the squamous metaplasia of the epithelial layer of the cornea and the bend of nerve fibers under the corneal epithelium. IC also suggested that the squamous metaplasia of epithelial layer of conjunctiva in these patients was improved. The symptoms of patients suffering from severe filamentary keratitis were remarkably relieved by using lacrimal plugs, which could increase the tear volume of the ocular surface, improve the condition of tear film, and promote the recovery of corneal diseases. For patients with more severe symptoms, additional usage of deproteinized calf blood extract eye gel could assist the treatment. Therefore, lacrimal plugs combined with deproteinized calf blood extract eye gel are suggested to be an effective method to treat severe filamentary keratitis.     

Development of a new lacrimal drainage conduit using POSS nanocomposite

Lacrimal surgery in cases of severely obstructed or missing canalicular ducts is highly challenging. In these cases, the placement of a bypass tube is currently the only option to restore the drainage of tears into the nose and reduce the symptomatic watery eye. Different approaches to achieve functional drainage have been tried using blood vessels or artificial implants. The implantation of the rigid Lester Jones tube is, since its introduction in the late 1960s, the gold standard. The functional success is satisfactory. However, complication rates are high and remain, even with many modifications of the original design, a major problem. These complications include mainly the displacement and blockage of the tube, requiring regular checkups, as well as irritation of the surrounding tissue including the nose and the eye. The objective of this study was to develop a new lacrimal duct conduit (LDC) to restore structural and functional integrity of the lacrimal drainage system. The conduit is constructed with a novel polymer, polyhedral oligomeric silsesquioxane-poly(carbonate-urea)urethane (POSS-PCU), that offers biocompatibility. We exploit nanotopography to evade the problems associated with current applications. A number of extrusion techniques were investigated for this purpose: ultrasonic atomization spraying, electrohydrodynamic atomization spraying/spinning, extrusion-coagulation, and high-pressure coagulation by autoclave and casting. Finally, the coagulation and cast technique were selected to construct an LDC superior to its predecessors, and its advantages highlighted.     

Oral Administration of Royal Jelly Restores Tear Secretion Capacity in Rat Blink-Suppressed Dry Eye Model by Modulating Lacrimal Gland Function

Tears are secreted from the lacrimal gland (LG), a dysfunction in which induces dry eye, resulting in ocular discomfort and visual impairment. Honey bee products are used as a nutritional source in daily life and medicine; however, little is known about their effects on dry eye. The aim of the present study was to investigate the effects of honey bee products on tear secretion capacity in dry eye. We selected raw honey, propolis, royal jelly (RJ), pollen, or larva from commercially available honey bee products. Tear secretion capacity was evaluated following the oral administration of each honey bee product in a rat blink-suppressed dry eye model. Changes in tear secretion, LG ATP content, and LG mitochondrial levels were measured. RJ restored the tear secretion capacity and decrease in LG ATP content and mitochondrial levels to the largest extent. Royal jelly can be used as a preventative intervention for dry eye by managing tear secretion capacity in the LG.     

Blind canalicular marsupialization in complete punctal absence as part of a systematic approach for classification and treatment of lacrimal system obstructions

The procedure of choice for epiphora caused by bipunctal and canalicular obstruction is conjunctivodacryocystorhinostomy. Despite its high success rate, it may result in multiple complications, such as extrusion, migration, and secondary obstruction. The author describes a simple alternative procedure to conjunctivodacryocystorhinostomy for patients with epiphora caused by bipunctal and proximal canaliculus complete occlusion and a systematic approach to treat lacrimal system obstructions. Ten instances of bipunctal and proximal canaliculus absence in five consecutive patients, caused in four patients by ocular surface disorders (topical drug toxicity, herpetic keratoconjunctivitis, and trachoma), were treated by blunt dissection of the presumed lower punctal site under a surgical microscope. The punctal site was determined by several landmarks, the peaked medial lid margin, a dimple at that site, or an area of relative avascularity. The canaliculus was exposed and expanded to create a pocket. After the procedure, the lacrimal drainage system was found patent in nine of the 10 procedures. After one additional procedure, irrigation of the lacrimal drainage system revealed a nasolacrimal duct obstruction that was treated with dacryocystorhinostomy and silicone tube insertion. After these procedures, an objective resolution of the epiphora was noted in all patients. Epiphora resulting from lack of punctal and proximal canaliculus caused by ocular surface diseases may be treated with blind exposure and marsupialization of the proximal canaliculus instead of conjunctivodacryocystorhinostomy. If, in addition, the nasolacrimal duct is obstructed, a dacryocystorhinostomy may be performed. If this proposed procedure fails, the patient can still undergo conjunctivodacryocystorhinostomy or other procedures. The procedure may be part of a systematic approach to treat lacrimal drainage obstructions that is based on an association between the location and the cause of the obstruction.     

NF-kB 和TGF-beta1在超声乳化术治疗的白内障患者结膜上皮细胞中的表 达及其意义

目的：探讨NF-kB 和TGF-beta1 在超声乳化术治疗的白内障患者结膜上皮细胞中的表达及其意义。方法：选取我院眼科收治 的白内障患者24 例,共24 眼,且均接受超声乳化白内障摘除术和后房型人工晶状体植入术治疗。在治疗前和治疗后15天、30 天,采用免疫组织化学法检测患者结膜上皮细胞中NF-kB 和TGF-beta1 的表达,并进行泪液分泌试验、荧光素染色、泪膜破裂时间 和泪河高度测量四项检查。结果：NF-资B 和TGF-茁1 呈现先增强后减弱的趋势,与术前相比,术后15 天患者NF-kB 和TGF-beta1 染 色程度显著增强,差异具有统计学意义,(P<0.05);与术后15 天比较,术后30 天NF-kB 和TGF-beta1 染色程度减弱,差异具有统计 学意义,(P<0.05);与术前相比,术后15 天泪膜破裂时间较术前明显缩短,荧光素染色明显增强;与术前相比,术后30 天四项检查 结果无差异,P＞0.05。结论：超声乳化手术可提高白内障患者结膜上皮细胞中NF-kB 和TGF-beta1的表达,与术后眼膜的炎症反应 有关。     

Bevacizumab Eye Drop Treatment Stimulates Tear Secretion in Rats Through Changes in VEGF and NGF Lacrimal Gland Levels

VEGF and NGF are known to modulate corneal healing, neovascularisation and tear secretion. While a VEGF-NGF cross talk has been recently shown to modulate corneal healing in rats, it is not known whether it also plays a role in the regulation of lacrimal function. In this study we aim to investigate the effects of anti-VEGF eye drop treatment on lacrimal gland function and on the local expression of VEGF and NGF in rats. Tear function was measured in 3 months old rats by modified Schirmer test at baseline and after 3 weeks of topical anti-VEGF eye drop treatment. Whole lacrimal glands from rats were removed after treatment and analysed by ELISA for VEGF and NGF levels. To investigate if the effects of anti-VEGF were mediated by changes in the NGF-pathway, we repeated the experiments in RCS rats, a strain with NGF-pathway impairment associated with decreased tear flow. After topical treatment with anti-VEGF eye drops, an increase in tear secretion was observed in both wild-type and RCS rats. A significant decrease of VEGF levels was also observed in lacrimal glands of both RCS and SD rats, accompanied by a significant increase in NGF levels. Inhibition of VEGF at the ocular surface in rats results in changes of tear function and lacrimal gland levels of VEGF and NGF. Further studies on the VEGF/NGF cross-talk at the ocular surface may expand our knowledge on the pathogenesis of several diseases characterized by tear dysfunction.     

不同泪道硅胶引流管留置时间对慢性泪囊炎患者视力、生活质量及复发率的影响

目的:探讨不同泪道硅胶引流管留置时间对慢性泪囊炎患者的生活质量、视力及复发率的影响。方法:回顾性选取2018年1月2019年12月期间于我院就诊的慢性泪囊炎患者91例(128眼),根据术后泪道硅胶引流管留置时间分为A、B两组,其中A组泪道硅胶引流管留置时间6周,44例(61眼),B组泪道硅胶引流管留置时间12周,47例(67眼)。对比两组疗效、视力、并发症发生率、主诉溢泪发生率、生活质量及复发率。结果:A组拔管当天的总有效率高于B组(P<0.05),两组拔管后3个月总有效率对比无明显差异(P>0.05)。拔管后6个月两组社会功能、躯体疼痛、精神健康、生理功能、精力、情感职能、生理职能、总体健康维度评分均较术前升高(P<0.05),但两组组间对比未见统计学差异(P>0.05)。两组患者术前、拔管后3个月视力组间及组内比较均未见统计学差异(P>0.05)。两组患者主诉溢泪发生率比较未见统计学差异(P>0.05)。A组并发症发生率、复发率低于B组(P<0.05)。结论:泪道硅胶引流管留置时间的长短对慢性泪囊炎患者疗效、视力、生活质量、主诉溢泪发生率无明显影响,但留置6周者并发症发生率、复发率低于留置12周者,提示临床应视患者具体情况尽量缩短泪道硅胶引流管留置时间。     

MUC16 in the lacrimal apparatus

The aim of the present study was to determine the possible expression of the mucin MUC16 in the lacrimal apparatus. Expression and distribution of MUC16 in lacrimal gland, accessory lacrimal glands, and nasolacrimal ducts was monitored by RT-PCR and immunohistochemistry. MUC16 was expressed and detected in all tissues investigated. Comparable to conjunctiva and cornea it was membrane-anchored in accessory lacrimal glands whereas in lacrimal gland acinar cells and columnar cells of the nasolacrimal ducts it was stored in intracytoplasmic vesicles without membrane-association. Subepithelial serous glands of the nasolacrimal ducts revealed staining of the secretion product. Intracelluar production of MUC16 is present in lacrimal gland and epithelial cells of the nasolacrimal ducts but it is not clear whether this MUC16 is secreted. MUC16 seems to be shedded or secreted from the epithelial surface of subepithelial serous glands of the nasolacrimal ducts. Our results show that MUC16 is present in the whole lacrimal apparatus. Its distribution pattern suggests different physiological functions with regard to tear film physiology and tear outflow. Moreover, the results demonstrate the existence of so far not recognized qualitative differences in the secretion product of main lacrimal gland and accessory lacrimal glands (glands of Krause).     

Lacrimal Hypofunction as a New Mechanism of Dry Eye in Visual Display Terminal Users

Background

Dry eye has shown a marked increase due to visual display terminal (VDT) use. It remains unclear whether reduced blinking while focusing can have a direct deleterious impact on the lacrimal gland function. To address this issue that potentially affects the life quality, we conducted a large-scale epidemiological study of VDT users and an animal study.

Methodology/Principal Findings

Cross sectional survey carried out in Japan. A total of 1025 office workers who use VDT were enrolled. The association between VDT work duration and changes in tear film status, precorneal tear stability, lipid layer status and tear secretion were analyzed. For the animal model study, the rat VDT user model, placing rats onto a balance swing in combination with exposure to an evaporative environment was used to analyze lacrimal gland function. There was no positive relationship between VDT working duration and change in tear film stability and lipid layer status. The odds ratio for decrease in Schirmer score, index of tear secretion, were significantly increased with VDT working year (P = 0.012) and time (P = 0.005). The rat VDT user model, showed chronic reduction of tear secretion and was accompanied by an impairment of the lacrimal gland function and morphology. This dysfunction was recovered when rats were moved to resting conditions without the swing.

Conclusions/Significance

These data suggest that lacrimal gland hypofunction is associated with VDT use and may be a critical mechanism for VDT-associated dry eye. We believe this to be the first mechanistic link to the pathogenesis of dry eye in office workers.     

Hormonal influence on the secretory immune system of the eye: endocrine impact on the lacrimal gland accumulation and secretion of IgA and IgG

The objective of the current investigation was to explore the processes underlying the androgen control of tear IgA and to determine whether hormone exposure also modifies tear IgG content. In addition, studies evaluated the impact of diabetes on the androgen regulation of secretory immunity in the eye. Tears and lacrimal glands were collected from age-matched, adult male rats, which had undergone hypophysectomy, selective ablation of the anterior pituitary, streptozotocin-induced diabetes, sham-surgery and/or orchiectomy and had been exposed to vehicle or physiological amounts of testosterone for varying periods of time. Our findings demonstrated that testosterone administration selectively increased the accumulation of IgA, but not IgG, in tears and lacrimal glands of orchiectomized rats. This hormone effect was associated with a 2-fold enhancement of the IgA transfer from lacrimal tissue to tears; IgA movement was against a gradient. In contrast, androgen exposure had no significant influence on the lacrimal gland/tear transfer of IgG, which was down a 90-fold gradient. Testosterone action on the lacrimal gland appeared to involve an increase in IgA production, but not a consistent alteration in the total number of IgA-containing cells. Similarly, androgen exposure had no impact on the population of IgG-containing lymphocytes in lacrimal tissue. Of interest, ablation of the anterior or entire pituitary in orchiectomized rats, which procedure inhibits testosterone-induced stimulation of tear IgA levels, significantly reduced the total number of IgA-containing cells in the lacrimal gland. Induction of diabetes by streptozotocin injection to orchiectomized rats resulted in diminished tear IgA content and decreased numbers of lacrimal IgA-positive lymphocytes, but did not prevent the testosterone-associated rise in IgA antibody content. In summary, our findings demonstrate that androgens increase the lacrimal gland production and secretion of IgA, but not IgG.     

Calorie restriction: A new therapeutic intervention for age-related dry eye disease in rats

A decrease in lacrimal gland secretory function is closely related to aging and leads to an increased prevalence of dry eye syndrome. Since calorie restriction (CR) is considered to prevent functional decline of various organs due to aging, we hypothesized that CR could prevent age-related lacrimal dysfunction. Six-month-old male Fischer 344 rats were randomly divided into ad libitum (AL) and CR (−35%) groups. After 6 months of CR, tear function was examined under conscious state. After euthanasia, lacrimal glands were subjected to histological examination, tear protein secretion stimulation test with Carbachol, and assessment of oxidative stress with 8-hydroxy-2 deoxyguanosine (8-OHdG) and 4-hydroxynonenal (HNE) antibodies. CR significantly improved tear volume and tended to increase tear protein secretion volume after stimulation with Carbachol compared to AL. The acinar unit density was significantly higher in the CR rats compared to AL rats. Lacrimal glands in the CR rats showed a lesser degree of interstitial fibrosis. CR reduced the concentration of 8-OHdG and the extent of staining with HNE in the lacrimal gland, compared to AL. Furthermore, our electron microscopic observations showed that mitochondrial structure of the lacrimal gland obtained from the middle-aged CR rats was preserved in comparison to the AL rats. Collectively, these results demonstrate for the first time that CR may attenuate oxidative stress related damage in the lacrimal gland with preservation of lacrimal gland functions. Although molecular mechanism(s) by which CR maintains lacrimal gland function remains to be resolved, CR might provide a novel therapeutic strategy for treating dry eye syndrome.     

负压封闭引流技术在腹部带蒂皮瓣供瓣区的应用

目的:探讨负压封闭引流(vaccumsealingdrainage,VSD)技术在腹部带蒂皮瓣供瓣区中的治疗作用。方法:10例上肢深度创面大小为9×6 cm-14×9 cm,创面清创后行腹部带蒂皮瓣移植,皮瓣面积10×7 cm-20×15 cm,供瓣区应用VSD覆盖,医用半透性贴膜封闭整个术区,连接中心负压,持续低负压吸引,每日外用1000 mL生理盐水持续冲洗VSD。约3周后行皮瓣断蒂术,供瓣区植皮。结果:10例皮瓣全部存活,1例患者术后当天引流区域出现疼痛,经止痛治疗后未再出现。1例患者术后2周出现VSD堵塞,经处理后恢复正常引流。无VSD装置更换病例。供瓣区周边皮肤无浸渍及感染现象,VSD撤除后见基底肉芽组织生长新鲜,创面均无感染,移植皮片存活。结论:VSD技术可以有效保护供瓣区,刺激肉芽生长,为后期皮片移植闭合创面提供了良好条件。VSD技术可安全有效的应用于腹部带蒂皮瓣供瓣区。     

A randomized prospective study of polyglycolic acid conduits for digital nerve reconstruction in humans

This article reports the first randomized prospective multicenter evaluation of a bioabsorbable conduit for nerve repair. The study enrolled 98 subjects with 136 nerve transections in the hand and prospectively randomized the repair to two groups: standard repair, either end-to-end or with a nerve graft, or repair using a polyglycolic acid conduit. Two-point discrimination was measured by a blinded observer at 3, 6, 9, and 12 months after repair. There were 56 nerves repaired in the control group and 46 nerves repaired with a conduit available for follow-up. Three patients had a partial conduit extrusion as a result of loss of the initially crushed skin flap. The overall results showed no significant difference between the two groups as a whole. In the control group, excellent results were obtained in 43 percent of repairs, good results in 43 percent, and poor results in 14 percent. In those nerves repaired with a conduit, excellent results were obtained in 44 percent, good results in 30 percent, and poor results in 26 percent (p = 0.46). When the sensory recovery was examined with regard to length of nerve gap, however, nerves with gaps of 4 mm or less had better sensation when repaired with a conduit; the mean moving two-point discrimination was 3.7 +/- 1.4 mm for polyglycolic acid tube repair and 6.1 +/- 3.3 mm for end-to-end repairs (p = 0.03). All injured nerves with deficits of 8 mm or greater were reconstructed with either a nerve graft or a conduit. This subgroup also demonstrated a significant difference in favor of the polyglycolic acid tube. The mean moving two-point discrimination for the conduit was 6.8 +/- 3.8 mm, with excellent results obtained in 7 of 17 nerves, whereas the mean moving two-point discrimination for the graft repair was 12.9 +/- 2.4 mm, with excellent results obtained in none of the eight nerves (p < 0.001 and p = 0.06, respectively). This investigation demonstrates improved sensation when a conduit repair is used for nerve gaps of 4 mm or less, compared with end-to-end repair of digital nerves. Polyglycolic acid conduit repair also produces results superior to those of a nerve graft for larger nerve gaps and eliminates the donor-site morbidity associated with nerve-graft harvesting.     

Deltopectoral skin flap as a free skin flap revisited: further refinement in flap design, fabrication, and clinical usage

The deltopectoral skin flap is an axial flap; therefore, it can be fashioned as a free skin flap. Although color and texture of the skin are well suited for facial resurfacing, the structural features of inconsistent thickness of the skin, a short vascular pedicle, a minute caliber of the nutrient vessel, and donor site morbidity often preclude the use of this flap for this purpose. The deltopectoral skin flap fabricated as a free skin flap transferred by means of a microsurgical technique was used in 27 patients between 1985 and 1998 at our hospital. The anterior perforating branches of the internal mammary vessels were the primary nutrient vessels of the flap in seven instances. The external caliber of this artery varied between 0.6 mm and 1.2 mm, with an average size of 0.9 mm. The size of the accompanying vein varied between 1.5 mm and 3.2 mm, with an average of 2.3 mm. Coaptation of these vessels with those in the recipient site was technically difficult. Thrombosis occurred at the anastomotic site in three patients, requiring reoperation. Two flaps were saved. The flap failure was drastically reduced in the remaining 20 patients by including a segment of the internal mammary vessel when fabricating the vascular pedicle. The size of the internal mammary arterial segment averaged 2.1 mm, and the average size of the accompanying vein was 2.9 mm. The problem of a bulky flap was managed by surgical defatting/thinning of the flap at the time of flap fabrication and transfer. A V-to-Y skin flap advancement technique of wound closure was used in eight individuals. The flap donor-site morbidities were minimized with this method of wound closure.     

A Novel Capsulorhexis Technique Using Shearing Forces with Cystotome

Purpose:To demonstrate a capsulorhexis technique using predominantly shearing forces with a cystotome on a virtual reality simulator and on a human eye.Method:Our technique involves creating the initial anterior capsular tear with a cystotome to raise a flap. The flap left unfolded on the lens surface. The cystotome tip is tilted horizontally and is engaged on the flap near the leading edge of the tear. The cystotome is moved in a circular fashion to direct the vector forces. The loose flap is constantly swept towards the centre so that it does not obscure the view on the tearing edge.Results:Our technique has the advantage of reducing corneal wound distortion and subsequent anterior chamber collapse. The capsulorhexis flap is moved away from the tear leading edge allowing better visualisation of the direction of tear. This technique offers superior control of the capsulorhexis by allowing the surgeon to change the direction of the tear to achieve the desired capsulorhexis size.Conclusions:The EYESI Surgical Simulator is a realistic training platform for surgeons to practice complex capsulorhexis techniques. The shearing forces technique is a suitable alternative and in some cases a far better technique in achieving the desired capsulorhexis.     

The crocodile tear syndrome.

Gusto-lacrimation, or "crocodile tear syndrome," is a rare complication, with 95 cases reported in the literature. Two patients are presented here, one after a facial fracture which apparently extended into the temporal bone proximal to the optic ganglion, and one after a Bell's palsy. The mechanism appears to be a misdirection of regenerating gustatory fibers destined for the salivary glands, so that they become secretory fibers to the lacrimal gland and cause homolateral tearing while the patient is eating. A simple procedure, involving subtotal resection of the palpebral lobe of the involved lacrimal gland, proved to be an effective corrective measure in these cases. Although it was not done in these cases, it would perhaps be advisable to do a Schirmer's test to assist in determining the amount of gland to be removed.     

A method for repositioning the external ear

The malposition of an otherwise normal-appearing external ear is not uncommon in certain craniofacial syndromes. This paper presents a 10-year experience of 14 patients who underwent external ear repositioning. In this technique, a posterior incision is used to mobilize surrounding soft tissues circumferentially around the external auditory canal, which then serves as an axis for anterior rotation and elevation. Elevation of up to 8 mm and rotation up to 30 degrees can be achieved. Further elevation is limited by the ensuing constriction and resistance of the external auditory canal. More rotation can be achieved with a Z-plasty transposition of an inferiorly based postauricular skin flap.     

Clinical assessment of bloody tears

Bloody tears are a rare clinical occurrence that can cause great anxiety for the patient and doctor alike. They can be associated with lesions of the lids, conjunctiva, cornea, lacrimal drainage system or lacrimal gland. Consideration should also be given to possible underlying systemic involvement. This paper includes a case presentation of a reported case of bloody tears and will review the differential diagnosis and management considerations in this condition.