By X. Karlen. University of Arizona.

Sturge-W eber syndrome: age of onset of sei­ products ham artin and tubcrin are m ultifunctional proteins with a zures and glaucom a and the prognosis for alfected children 5 mg proscar. J N curopathol Exp Neurol lited chrom osom e in a family ascertained through a patient with 2004;63:1236-42 proscar 5mg. Ih e natural history of patients with the Sturge-W cbcr ependym al giant cell astrocytom as and angiom yolipom as supports syndrom e 5mg proscar. Sturge-W eber-Dimitri cohort of 224 tuberous sclerosis patients indicates increased sever disease: role o f hem ispherectom y in prognosis proscar 5 mg. Sturge-W eber-Klippel-Trcnaunay syndrom e: what’s in a Ophthalm ology 1978;85:276-86 . Cutis m arm orata tclangicctatica congenita ulbar hem odynam ics in Sturge-W eber syndrom e-associated glau­ (congenital generalized phlebectasia) . Cavernous hem angiom a of surgical and medical m anagem ent o f glaucom a in Sturge-Wcbcr the retina . Sturge-W cbcr syn­ angiomas: natural history and genetic study over a 5-year period . Rccurrcnt intraopcrativc choroidal effu­ hem angiom a o f the retina , cutaneous angiom as , and intracranial sion in Sturgc-W cber syndrom e. J Pediatr O phthalm ol Strabismus vascular lesion by com puted tom ography and nuclcar m agnetic 1983;20:250-2. Int O phthalm ol 2007;27: findings and results of laser treatm ent in circum scribed choroidal 345-50. N ine cases of cavernous therapy for sym ptom atic choroidal hem angiom a: visual and ana­ hem angiom a of the retina. Eur J Canccr 1994;30A: site on chrom osom c 10 with the Sturgc-W eber syndrom e and con­ 1987-90. Singh Л number of eyelid tumors are associated with inherited neurofibromas, nodular plexiform neurofibromas that systemic diseases. Early recognition of the nature of these involve major nerve plexuses, and diffuse plexiform neuro­ tumors can result not only in prompt appropriate therapy, fibromas that encompass all the other types and insinuate but also, when indicated, a genetic workup that may unveil into the tissue planes. The following chapter bromas have greater vascularization and an increase in provides an overview of eyelid tumors and their systemic extracellular matrix; otherwise, they arc similar to their associations. Benign peripheral signs of malignant transformation are pain, increasing nerve sheath tumors composed predominantly of Schwann tumor size, and focal neurologic deficits. The lesions are soil and fleshy, commonly occurring on the face, head, and trunk (Fig. Often Nevus flammeus, or port-wine stain, of the eyelid is a con­ visualized by the end of the first decade, lesions may also genital malformation of dermal capillaries. Ihey are initially sessile, but often become peduncu­ usually appear at birth and continue to grow and evolve as lated. The subtypes vary from pink/red flat, to purple flat, and thicker, more nodular lesions. Bilateral port-wine stains of the eyelids have a higher likelihood of being associated with Sturge-Weber syndrome than unilateral lesions (Fig. The success of pulsed dye laser has marked an advance in the treatment of nevus llammeus. Oxyhemoglobin absorption of laser energy is thought to result in coagulation of dermal capillaries, and resultant treatment of the lesion. Not originally described by Gardner in 1951, the diagnosis on dilated fundus examination was critical in an era before genetic testing was available. Note nodular gene has been mapped to chromosome 5q21 and encodes hem angiom as over a oacksrour. By 35 years of age, around 95% of 1465, the number of lesions dropped sign ificantly. The description has since been modified and molecular testing is available and can detect mutations in termed Gardner syndrome to describe extracolonic up to 95% of cases. Although basal the cysts frequently arise prior to puberty and occur pri­ cell nevi may occur early in childhood, it is the risk for marily on the face, scalp, and extremities. Believed to arise from the dental lamina, keratocysts patients are extremely tall. Nearly 35% of individuals with Gorlin syn­ percent of patients develop jaw cysts by 10 years of age, drome have radiographic evidence of small cysts in the and 51% by 20 years. The most common locations include the small, transient, keratin-filled cysts (milia) found on the face, back, and chest. Basal cell carcinom a of the eyelid associated with basal cell carcinoma syndrome: results or a population-based study. In 1968, facial and eyelid lentigines were observed in 70%, conjunc­ Torre reported a patient who had many sebaceous tumors tival and caruncular pigmentation in 27%, and eyelid myx­ and primary carcinoma of the ampulla of Vater resected at omas in 16% of patients. The average age at presentation of the first malignancy is 53 years (range 23-89 years). Glands of Zeiss and meibomian glands of the eyelids are modified sebaceous glands that can be the site of origin for sebaceous ade­ noma. Extraocular sebaceous carcinoma can metastasize; therefore, thorough clinical examination is fwnira S e taceo u s adenoma ot the upper eyelid in a patient with needed to determine the extent of disease. Sebaceous adenom a of the eyelid in Muir-Torre Cystic changes within a sebaceous adenoma are indica­ syndrome. Textbook o f Dcrm ato- Scbaccous gland carcinom a of the eyelid and palpebral conjunctiva pathology. M uir-Torrc phenotype has a tum ors with scbaccous differentiation in the Muir-Torre syndrom e. The M uir-Torrc syndrom e: a 25-year retro­ palpebral conjunctiva in a patient w ith M uir-Torre-svndrom e: a spect. It is plausi­ Oculo(dermal) melanocytosis (nevus of Ota) is a rare ble, therefore, that conditions predisposing for cutaneous hamartoma of the melanocytes within the distribution area melanoma might also be a risk factor for uveal melanoma. Multiple of uveal melanoma in affected persons, usually in the chor dysplastic cutaneous nevi in a patient and a first-degree oid and ipsilateral to the side of hyperpigmentation. Although The genetic factors for familial cutaneous melanoma uveal melanoma has repeatedly been reported in patients are partly understood. Using standard techniques like G-banding it of the mechanism behind development of metastatic dis­ is possible to identify loss of entire chromosomes or entire ease in uveal melanoma is incomplete. To detect subtle altera­ the possibility that the nonrandom changes of chromosome tions and structural rearrangements, molecular cytoge­ 3 are not causally involved in the mechanism of metastatic netic techniques such as fluorescence in situ hybridization disease but simply accompany tumor progression. Abnormalities of chromosome 8 have also been strongly Since the first description of chromosomal changes in associated with reduced survival in uveal mclnoma. Chromosomal aberrations were found to correlate much more strongly with melano­ ma-related mortality. Single red signal represents monosomy ot rarely metastasize, and regular systemic liver and blood chromosome 3. Targeted therapies are better tolerated years since its discovery, our insight into ccll cycle control than conventional systemic chemotherapy, and these need and our understanding of the role of the Rb protein in to be evaluated as adjuvant treatment for uveal melanoma those mechanisms have continuously improved. Transcriptom ic profiling seems transcription factors of the E2F family, particularly promising but is still far from routine clinical application. O phthalm ol Clin N orth Am 2005;18:85-97, which arc themselves activated by cyclins and inactivated 2. Genetic predisposition to ocular m ela­ uncontrolled cel) proliferation, even though most tumors nom a. Uveal m elanom a in children In uveal melanoma, Rb seems to be functionally and adolescents. Ann Plast Surg seems to be affected by functional inactivation and reduced 1993;31:265-70. Prepuhcral and puberal m elanom as in ophthalm ol­ settings in ophthalmic oncology centers. Iridociliary m elanom a associated with ocular m elanocytosis in a 6-year-old hoy. Iris m elanom a arising in iris high-risk cytogenetic changes, which relieves patients nevus in oculo(derm al) melanocytosis. Bilateral prim ary uveal m ela­ Mathematical models suggest that metastasis occurs nom a. Bilateral ciliary body m el­ metastasis becomes clinically evident, treatment does not anomas. J Cell of uveal m elanom a using finc-ncedlc aspiration biopsy at the tim e Biochem 2004;93:708-20. O phthalm ic Cienet 1996;17: m etastatic m alignant m elanom a of the uvea: tum or progression 39-40. M olecular prognostic testing in uveal m elanoma: has teri3 l hepatic fotcm ustinc for high-risk uveal m elanom a patients. O phthalm ology 2006; 113: mesylate against uveal m elanom a in vitro and in vivo. Diagnosis The diagnosis of retinoblastoma is essentially clinical and is based on fundus examination by ophthalmoscopy and ultrasonography. As the tumor bccomes larger it may induce retinal detachment (exophytic pattern), vitreous and subretinal seeding (endophytic pattern), or a mixed pattern (Fig. Diffuse infiltrating retinoblas­ toma is a rare variant of this tumor characterized by lack of a circumscribed mass. The tumor in this case masquerades as uveitis, hyphema, and secondary glaucoma and presents Figure 49. Six-cycle chemoreduction using three agents (vincristine, etoposide, and carboplatin) is generally prescribed. Note pretreatment appearance of mixed pattern with exophytic tion is not without its problems. After chemotherapy (B) while on chemotherapy has been observed/0,51 Immediate there is marked rep/ession of the exophytic tumor and com plete clinical resolution of the endophytic component (vitreous seeo si. Ested here may differ from those оt other centers tased on physician preference», patient preferences. A cuff of subretinal fluid extending no more than S mm from the base of the tumor f. Iar*e avascular plaques on the underside of the cetauhed retina, or extensive subretinal masses (exoplv/fcc disease). In the case of familial retinoblastoma, the initial event or "hit" is a germline mutation that is inherited and found in all cells of the offspring (A). He proposed that the development occurs in a retinal cell, then retinoblastoma develops (B). In the case of familial retinoblastoma, the initial event or “hit” is a germline familial cases of retinoblastoma, all cells in the body are mutation that is inherited and found in all cells of the off­ predisposed to possible tumor development since a germline spring (Fig. The second “hit” occurs some time mutation (“first hit”) has been inherited in all cells of the during development, and if it occurs in a somatic cell such body, including the ovaries and testis-hence the heritability as a retinal cell, then retinoblastoma develops. In contrast, in most Histopathologic High-Risk cases of unilateral sporadic retinoblastoma, the “two hits” Features and the Role of occur during development of the retina and both “hits" are Adjuvant Chemotherapy somatic mutations. Ophtha- System ic and Sub-Tenon Imoscopically and histopathologically, heritable and non­ Chemotherapy for Groups heritable retinoblastoma cases are indistinguishable/’1The С and D Intraocular Retinoblastoma major differences between heritable and nonheritable retin­ oblastoma are that the heritable tumor usually occurs at a 0 3 2 1 Extraccular disease A Trial of Intensive Multi- younger age and is more likely to be bilateral and multi- Modality Therapy for Extraocular Retinoblastoma centric, and the affected patient is at higher risk for nonocular tumors than is the nonheritable case. Hereditary In addition, there is contribution of other genes, which cases are also at an increased risk of second malignancies, leads to continued tumor progression. This finding subsequently Newer evidence suggests that recent treatment methods of prompted a retinal examination, which revealed unilateral systemic chemoreduction for retinoblastoma may prevent multifocal tumors in both cases. DeRose Like everyone else, visually impaired people want full, depends on the severity and chronicity o f the disease. Severe productive lives, and any intervention that may help them and chronic impairments cause disabilities. Again bilitation services arc scarce,1and most patients with low depending on their severity and chronicity, disabilities may vision arc left in the hands of eye care practitioners who turn into handicaps. Handicaps arc barriers to functioning often do not attend to their rehabilitative needs. However, while their medical care is flawless, with the objective of eliminating or limiting handicaps. The thorough clini­ In treating visual disorders, ophthalmologists sec cian should question patients about their functional problems patients at the start of this hierarchy. If the clinician cannot resolve the thought of as a rehabilitation effort, not a procedural one. One desired benefit o f this change in attitude Vision rehabilitation has many parallels with rehabilitation may be better third-party funding for low-vision care, medicine. Unfortunately, patients often are not referred for care because the practitioner believes cine that vision rehabilitation could better emulate is a that they cannot afford the services. Decisions about services comprehensive team approach that exploits each member’s and assistive devices should be based on medical necessity, specialty. The rehabilitation medicine team is led by the physiatrist, Better funding may allow all patients to receive the reha­ who evaluates the patient, diagnoses disabilities, and coor­ dinates the rehabilitation effort. Ihe analog to the traditional occupational therapist tion caused by the disease results in an im pairm ent that is the occupational therapist trained in low vision, or a vision 845 rehabilitation teacher. The analog to the physical therapist is rehabilitation objectives, goals, and tasks. The social worker mine that the patient’s objectives arc education, career, performs in an identical crossover role. For creating the interdisciplinary team is to provide better example, for an objective of completing college, the goals patient care, standardize low-vision services, and integrate might be traveling to and around the college, attending low-vision rehabilitation into mainstream medicine. Patients are considered to have low vision when they are Clearly, patients should receive early and proper reha­ unable to perform desired or required tasks because of bilitation to improve their quality of life, enable them to visual disability. Employed persons typically seek low-vision meet as many o f their objectives as possible, and enhance care sooner than retired persons because o f the visual their medical management. Patients’ attitude patients’ life state improves, their need for intervention toward their vision loss may also contribute to their func­ lessens. People differ in their ability to deal with impairment and vary in their ability to keep it from becom ing a disability or handicap. Because of patients’ individual tempera­ In contrast to medical history taking, which seeks infor­ ments and requirements, it is difficult if not impossible to mation about the disease process, low-vision history taking quantify criteria numerically, as by visual acuity, contrast seeks to determine how the disease is affecting the patient’s sensitivity function, visual field size, etc.

During the pro- mucosal injury or perforation within the tunnel cedure 5mg proscar, minor bleeding is often treated by may lead to mediastinal sepsis because the forced coagulation using the tip of the knife proscar 5 mg. After care- operation 5 mg proscar, they are precoagulated using the ful hemostasis within submucosal tunnel proscar 5mg, the hemostatic forceps in soft coagulation mode mucosal incision site is closed with several (Figs . Sufficient submucosal injection is important to prevent mucosal injury (arrows show the mucosal rupture) 6 . Note after clinging to the the distal esophagus longitudinal muscle fibers and lifting them up toward the a Fig . On the basis of more believe that in future myotomy length in patients than 600 cases’ experiences in our center , short- will be tailor made to manometric findings . Long-term data on remission will confirm his offer a significant anti-reflux barrier . In these cases, the submucosal Complete Myotomy space may be obliterated and an obvious esopha- Although a circular muscle myotomy preserving geal diverticulum may occur at the original myot- the longitudinal outer esophageal muscular layer omy site as a result of previous surgery; it may is often recommended (which is different from the therefore be very difficult to create submucosal usual full-thickness myotomy performed surgi- tunnel, as can be accomplished easily in those cally) in order to avoid entering the pleural space who have not had prior surgery. It is often difficult to preserve should therefore be placed in an unscarred area the longitudinal muscle as they are extremely that has not been previously myotomized. Given the fact that an incom- with such megaesophagus, both interventional plete myotomy with possible fibrotic healing can and surgical treatments are relatively complex and result in postoperative recurrence, a full-thickness difficult. As a new minimally time and facilitate reinjection possibly contribut- invasive endoscopic treatment, we propose hat ing to a lower rate of minor intra-procedural achalasia in children should be treated with bleedings [27] (Fig. Re-myotomy was performed at the opposite side of the (h) Closure of mucosal entry. Second, regular follow-up enables the clini- nursed in a semi recumbent position, given oxy- cian to detect symptomatic recurrences at an gen. Clinical or tele- lance after 10–15 years of disease duration might phone follow-up is planned to assess the Eckardt be beneficial because of the increased risk of can- score at every 1–2 years. However, manometric examinations are also recommended further studies are needed to determine how fre- to recognize recurrence of disease. This left such patients with two the same place, thus offsetting mucosal defect choices: surgical resection or endoscopic fol- and tumor resection site. However, these techniques are under the completely direct visualization and more aggressive and can result in damage to the decrease the recurrence rate. Therefore, laparoscopic application of traditional endoscopic resection and endoscopic cooperative surgery is recom- (Fig. The maximum resectable lesion size is 5 cm because of the limited space of the sub- 6. Tumors with large size, irregu- lar shape, and difficult location (such as cardia 6. A standard single extraluminal growth); (4) to initially distinguish accessory-channel gastroscope is used during benign from malignant lesions (Fig. In addition, it also pro- vides information of local invasion and distant metastasis (Fig. Care is taken to cedure includes four steps: tumor locating, muco- avoid damage to esophageal adventitia or gastric sal incision, submucosal tunneling, tumor serosa. A the right direction of the submucosal tunnel dual-channel gastroscope is sometimes needed (Fig. If pneumoperitoneum is Afterwards a 2-cm, longitudinal mucosal incision developed, a 20-gauge needle is inserted in the made with a hook knife as an entry point. Patients are ited space in the tunnel, tumor only up to the kept nil by mouth for 1 day. This not only causes less bleeding (due to Complications less vascular bed), but also maintains mucosal integrity. Utmost care should be taken to avoid Like any other surgical procedure, there is the injury to the overlying mucosa. Procedure-related compli- space, the mucosal injury or even perforation cations can be intraoperative or postoperative. This area can often be clipped with metallic clips after myotomy or tumor resection. If mild subcutane- subcutaneous emphysema exacerbate, subcutane- ous emphysema, mediastinal emphysema, or ous puncture is needed. If severe intraoperative pneumothorax (volume of lung compression pneumothorax occurs (airway pressure exceeding <30 %; patient not breathless and SpO2 >95 %), 20 mmHg, SpO2 <90 %, confirmed by X-ray film the patient is observed closely and given con- at bedside), closed thoracic drainage is consid- servative treatment. If the abdo- pression of more than 30 %, thoracic drainage men is excessively distended during the proce- is carried out. A central venous catheter is dure, abdominal puncture is performed using a inserted at the third or fourth intercostal space 20-gauge needle. After men and allows successful completion of the pro- 2–3 days of the drainage, the decompressed cedure. Pearl anterior to the external sphincter, whereas in women it is a less well-defined insertion area of woven muscle fibers slightly superior to the external sphincter. The significance of this finding is that during vaginal delivery this tissue becomes markedly attenuated predisposing it to obstetrical tears which can clearly impact fecal continence. This ana- tomic arrangement in women along with the fact that the puborectalis muscle is absent anteriorly should alert the sur- geon to exercise caution when encountering an anterior fis- tula tract. Unless the fistula is extremely superficial, primary fistulotomy should probably be avoided. The Geography of the Anorectal Spaces There are several spaces and potential spaces surrounding the rectum and anal canal that are of surgical significance Fig. The perianal space surrounds serves as a window to the left and right ischiorectal spaces, which is the lowest portion of the anal canal and is confined by the how horseshoe abscess/fistula forms. The location and course of the radiating elastic septae of the conjoined longitudinal muscle inferior rectal nerves are shown to emphasize how they can be readily avulsed by overaggressive spreading of curved clamps during drainage attachments to the anoderm and perianal skin and contains of ischiorectal abscesses finely lobulated fat, delicate branches of hemorrhoidal vessels, nerves, and lymphatics. When blood or pus accumulates in this closed space the stretching and irritation of the many inexperienced clinician into making the diagnosis of celluitis nerve endings results in the severe anal pain associated with rather than a drainable abscess with disastrous results espe- perianal abscesses and thrombosed external hemorrhoids. The abscess cavity may extend around one-half the anal canal to the level of the anorectal ring. The roof of this circumference of the anus (horseshoe) or extend completely pyramid-shaped space is composed of the levator ani mus- around the anus (floating freestanding anus). It is filled with large ischiorectal abscess is inadvertently mistaking the coarsely lobulated fat and contains the inferior rectal vessels fanned out array of branches of the inferior rectal nerve as and nerves. It is a relatively large space and can harbor a “loculations” inhibiting adequate drainage. Tearing these substantial abscess with only minimal involvement of the branches by recklessly spreading a large curved clamp can overlying gluteal skin. These clinical findings can mislead an result in significant injury to the nerve supply to the external sphincter. If this procedure is carried out on both sides as in the case of a horseshoe abscess, complete denervation of the sphincter can occur. The superficial postanal space is located in the posterior midline between the skin and anococcygeal ligament and is frequently involved with anorectal abscesses. The deep post- anal space (retrosphincteric space of Courtney) located deep to the anococcygeal ligament and the upper portions of the external sphincter and levator muscles is of special surgical significance first because of the frequency of abscesses occurring in this region, and secondly because the deep postanal space serves as a window to the left and right ischio- rectal spaces which can result in horseshoe abscesses or fistulas (Fig. The presence of a high fistula in a patient with Crohn’s surface of the levators and pelvic peritoneum. In these instances, it is prudent to mark the fistula presenting in this location may be difficult to diagnose tract with a long-term seton such as a silastic vessel loop to especially when there are no visible clinical findings around promote drainage and deter the development of recurrent the perineum. A marking seton should be placed whenever there is a which resulted from the septic process eroding through the reasonable clinical suspicion that primary fistulotomy adjacent levator ani muscle resulting in an hourglass-shaped will disrupt fecal continence. Summary When to Avoid Primary Fistulotomy Anatomic considerations relating to fistula surgery Anorectal examination under anesthesia is an important step Sphincter architecture based on refined imaging techniques to assess the location and extent of the abscess/fistula pro- Geography of the anal glands and anorectal spaces cess, as well as a means to determine how much sphincter When to avoid primary fistulotomy muscle is encircled by the tract. There are several circum- stances where these findings can be particularly helpful in preventing overly aggressive fistulotomy which may result in References fecal incontinence. In each of the following cases complete primary fistulotomy should be avoided and be replaced by 1. The riddle of the sphincters, the morphophysiology of more conservative procedures such as the judicious use of the anorectal mechanism reviewed. Surgical anatomy and physiology of the colon, rec- setons, fistula plugs, fibrin glue, or eventually mucosal or tum, and anus. A new concept of the anatomy of the anal sphincter mech- be defined as involving more than 50 % of the external anism and the physiology of defecation. Surgical anatomy of the pelvic tomic landmarks have been severely distorted such that autonomic nerves, a practical approach. Relationship of Abscess to Fistula 3 Herand Abcarian the role of anal glands in the pathogenesis of anorectal infec- Introduction tion [7]. Similarly, Kratzer in 1950 stressed the clinical signifi- cance of anal glands and ducts [8]. Eisenhammer in 1956 The true incidence of the anorectal abscess fistula is not known stated that all fistulas originate from intermuscular gland because most reports come from a large colorectal surgery infections [9]. In addition many cases of ano- secreting, columnar epithelium of anal glandular type in rectal abscess are drained in the office, outpatient clinic, surgi- biopsy material from 21 of 30 patients with fistula in ano. In centers, or emergency departments and as such no formal 13 this formed part of the lining of the internal opening of the records, e. In one large series of anorectal abscesses in 1967, Goligher and colleagues challenged this etiologic treated in the operating room, the incidence of fistula was theory. In two single-institution series, the incidence of anal, eight ischiorectal, one perirectal) they carefully inspected fistula was similar at 26 % [2] and 37 % [3]. If one extrapo- the lining of the anal canal and its valves with a bivalve ano- lates the number of abscesses based on fistula data, the inci- scope. In only five out of 29 cases (all perianal) they found commu- nication with the crypt region, supporting the argument that in Etiology about two-thirds of the anorectal infections the cryptoglandu- lar etiology does not apply [11]. Anorectal abscess is believed to originate from infected anal The infection, originally an intersphincteric abscess, finds a glands. These were originally described by Hermann and path of least resistance to spread. If it extends caudad between Desfosses in 1880s who demonstrated that the anal glands the internal and external sphincter to reach the anal verge, it opened into the anal crypts, and branched within the internal produces a perianal abscess. If it ruptures through the external sphincter and ended in the space between the internal and sphincter to reach the ischiorectal fossa, it is called ischiorectal external sphincters. If the abscess extended in a cephalad direction between infection in these glands spread through the intersphincteric the layer of the rectal smooth muscle it will produce a high space to the perianal skin [5]. In 1933 Tucker and Hellwing intermuscular abscess which on occasion is labeled a submuco- published on the histopathology of the anal gland, and demon- sal abscess. Rarely, infection may spread above the levator strated conclusively that anal sepsis originates in the gland space producing a supralevator abscess (Fig. A deep ducts and extends from anal lumen into the walls of the anal postanal abscess may spread to one or both ischiorectal fossae canal [6]. Hill and colleagues in 1943 published and stressed and results in a horseshoe abscess Aside from cryptoglandular origin, the other causes of ano- rectal suppuration may result from the downward spread of pel- H. They its transmural pathologic nature may extend to perirectal produce pain and swelling but are not associated with many or perianal space as an abscess fistula. These abscesses are easier to diagnosis chicken and fish bones as well as swallowed toothpicks may and do not need any imaging modalities (Fig. Anorectal abscesses may drain spontaneously or may External penetrating injuries (stab, gunshot, shot gun require surgical incision and drainage. Once the abscess is wounds) may result in an overt injury though at times more drained, there are three possible outcomes: minor or subtle injuries gradually proceed to full-blown 1. Perforation of low rectal cancers may also This signifies possible lack of communication with the present with large ischiorectal abscesses. Occasionally a thin film of epithelium may cover the external opening, causing collection of fluid, blood, or pus underneath it. This may give the patient a false sense Clinical Manifestation of recovery until the area swells, causes pain or ruptures and the fistula reappears. The clinical picture of anorectal abscess depends on the level Anorectal fistulas are associated with preexisting (height) of the abscess. In a study of 100 recurrent the anorectal ring, supralevator and high intermuscular anorectal abscesses, an underlying fistula was demonstrated abscess are located above this level. These etiologies may cause a fistula, which 3 Relationship of Abscess to Fistula 15 Table 3. Fistula (%) find communication with the anal canal with gentle probing Intersphincteric 219 104 (47. All patients underwent examination under abscesses and fistulas: a study of 1023 patients. Sur la muquese de la region cloacole de unroofing of abscess and primary fistulotomy when deemed rectum. Role of anal gland in the patho- of fistulas found in 100 patients with recurrent anorectal genesis of anorectal disease. A critique of the anal glandular infection in the aetiology and treatment of idiopathic anorectal from patients with small anorectal abscess treated with a abscesses and fistulae. The literature is widely Introduction disparate with regard to the natural history of acute anorectal abscesses becoming chronic anal fistula. The incidence of Anorectal infections are classically categorized at specific abscesses forming anal fistula varies between 5 and 83 % versus nonspecific. Corman [1] credits the cryptoglandular “theory” the five types of abscesses and their potential to form five to Herrmann and Desfosses [2] and Chiari [3 ] first elucidated types of fistula is fundamental to the successful evaluation in 1880s. Most of the literature credits Eisenhammer [4] and and treatment of nonspecific anorectal infections. While impossible to prove, the cryptoglandular theory as the origin of nonspecific anorectal infections is widely accepted by the world’s surgeons.

proscar 5 mg

Cellular elements may also be uals in only trace amounts in the plasma 5mg proscar, with a median level produced in addition to the acute-phase proteins proscar 5 mg. Once the condition that induced its elevation has ment components proscar 5mg, and interferons 5 mg proscar. These proteins , which migrate reacts with phosphoryl choline in the C polysaccharide of figure 2 . Among by binding to C1q , agglutination of particulate ligands , and the numerous factors that contribute to natural resistance are participation of insoluble ones , as well as neutralization of the skin , mucous membranes, and other barriers to infection; biological activity. The dermis is the skin layer below the epidermis and base- Humans are confronted with a host of microorganisms with ment membrane that comprises blood vessels, lymphatic ves- the potential to induce serious or fatal infections. Yet nature sels, nerve fbers, scattered fbroblasts, macrophages, mast has provided appropriate molecules, cells, and receptors that cells, dendritic cells, and αβ T cells. Many of these defenses beneath the basement membrane and the reticular dermis is are general or nonspecifc and do not require previous expo- beneath the papillary dermis. Both the papillary and reticular sure to the offending pathogen (or closely related organism). Another important defense system is acquired immunity, which can develop after previous contact The epidermis is the upper layers of the skin that contain with the organism through infection (overt or subclinical). Early induced responses are nonadaptive host responses induced by infectious agents early in infection. Their induc- The keratin layer is an external structure of skin that pro- tive phase differentiates them from innate immunity and their tects the body from microorganisms and resists penetrating failure to involve clonal selection of antigen-specifc lympho- stimuli. It is comprised of keratin protein flaments synthe- cytes distinguishes them from adaptive immunity. Innate defense system is general or nonspecifc and does Keratinocytes are squamous epithelial cells of the epider- not require previous exposure to the offending pathogen (or mis that are produced in waves. The keratin layer is a Constitutive defense system: Refers to innate or constitu- consequence of the orchestrated death of a wave of keratino- tive defense system. In the lower epidermis, these cells secrete pro-infam- matory cytokines and complement components. Innate immunity is a natural or native immunity that is present from birth and is designed to protect the host from Desmosomes are specialized junctions that connect kerati- injury or infection without previous contact with the infec- nocytes to one another and guarantees that each keratinocyte tious agent. It is attributable to physical, chemical, and layer divides and migrates upward as a unit. It includes such factors immunity, may have a signifcant role in resistance of a group as protection by the skin, mucous membranes, lysozyme in (herd) of humans or other animals against an infectious dis- tears, stomach acid, and numerous other factors. Elimination of reservoirs of the disease agent natural killer cells, complement, and cytokines represent key may be as important as specifc immunity in diminishing participants in natural innate immunity. The successful vaccination infected under normal circumstances by selected microor- of most members of a population against a selected pathogen ganisms that usually infect animals. This may be altered in may protect non-immune individuals in the group, whose the case of profound immunosuppression of humans, as in vulnerability is diminished because the pathogen cannot the case of acquired immune defciency syndrome in which become established in the vaccinated population. Molecules, Cells, and Tissues of the Immune Response 153 Lactoferrin is a protein that combines with iron and com- Protective immunity refers to both natural, nonspecifc petes with microorganisms for it. This represents a nonanti- immune mechanisms and actively acquired specifc immu- body humoral substance that contributes to the body’s natural nity that result in the defense of a host against a particular defenses against infection. By combining with induced either by active immunization with a vaccine pre- iron molecules, it deprives bacterial cells of this needed pared from antigens of a pathogenic microorganism or by substance. Lactoperoxidase is an enzyme present in milk and saliva that may be inhibitory to a number of microorganisms and Protective antigens are the antigenic determinants of a serves as a nonantibody humoral substance that contributes pathogenic microorganism that stimulate an immune response to nonspecifc immunity. Its mechanism of action resembles that can protect a host against an infection by that microor- that of myeloperoxidase. Thus, these particular antigenic specifcities can be used for prophylactic immunization in vaccines to immunize Nonsterile immunity: See premunition. Acquired immunity is protective resistance against an Protective epitopes are antigenic determinants of a patho- infectious agent generated as a consequence of infection with genic microorganism that stimulate a protective immune a specifc microorganism or as a result of deliberate immu- response against that same microorganism. Preemptive immunity refers to resistance shown by virus- Cellular immunology is the study of cells involved in infected cells to superinfection with a different virus. Artifcial passive immunity refers to the transfer of immu- Active immunity is protection attained as a consequence of noglobulins from an immune individual to a nonimmune, clinical or subclinical infection or deliberate immunization susceptible recipient. It is a type of adaptive immunity in which lymphocytes are activated in response to Passive immunity is a form of acquired immunity induced a foreign antigen to which they have been exposed. Compare by the transfer of immune serum containing specifc anti- with passive immunity. Examples of passive immunity Artifcially acquired immunity is the use of deliberate are the transfer of IgG antibodies across the placenta from active or passive immunization or vaccination to elicit pro- mother to fetus or the ingestion of colostrum-containing anti- tective immunity as opposed to immunity which results from bodies by an infant. Antitoxins generated to protect against unplanned and coincidental exposure to antigenic materials, diphtheria or tetanus toxins represent a second example of including microorganisms in the environment. The trans- fer of specifcally sensitized lymphoid cells from an immune Specifc immunity refers to an immune state in which anti- to a previously nonimmune recipient is termed adoptive body or specifcally sensitized or primed lymphocytes recog- immunization. By contrast, immunologically serum can be used for the temporary protection of individu- competent cells may interact with antigen to produce specifc als exposed to certain infectious disease agents. Humoral immune response is a host defense mediated by antibody molecules found in the plasma, lymph, and tissue Gravity and immunity: Space fight has been associated fuids. This type of immunity protects against extracellular with the development of neutrophilia, slight T cell lym- bacteria in foreign micromolecules. Humoral immunity may phopenia, and diminished blastogenic responsiveness of be transferred passively with antibodies or serum containing T cells in postfight blood samples. Changes have also Humoral antibody is found in the blood plasma, lymph, been observed in postfight concentrations of immuno- and other body fuids. Humoral antibody, together with com- globulins, complement components, lysozyme, interferon, plement, mediates humoral immunity which is based upon and α2-macroglobulin. IgA and IgM rose but IgG remained constant dur- immunodefciency worldwide is malnutrition. No defects in humoral immunity have malnutrition has an adverse effect on immunity, increases been noted. Serum antibody responses are usually unaffected in Exercise-induced immunosuppression or immunoenhance- protein-energy malnutrition. But cytes is intact and metabolic destruction of microorganisms these increases return to preexercise levels within a few is decreased as is synthesis of various cytokines including hours following its cessation. Acute exercise is followed cell-mediated immunity and diminished antibody responses. It leads to impaired lympho- have been demonstrated following long distance running. IgG, IgM, and IgA levels as well as the ability to synthesize antibody to tetanus toxoid antigen are not compromised by Vitamin A and immunity: A defciency of vitamin A com- exercise. Exercise prior to exposure to infection diminishes promises acquired, adaptive, antigen-specifc immunity. The morbidity or mortality, yet exercise during an infection pro- defciency has been linked to atrophy of thymus, spleen, duces the reverse effect. Prolonged intense exercise is fol- lymph nodes, and Peyer’s patches pointing to major alterations lowed by some immunosuppression. Vitamin A defciency altered by physical exercise are related to the neuroendocrine is also associated with impaired ability to form an antibody changes such as those that occur in response to physical or response to T cell-dependent antigens such as tetanus toxoid, psychological stress. It is also linked to decreased antibody responsiveness to T cell-independent antigens such Nonspecifc immunity: Refers to mechanisms such as as pneumococcal polysaccharide and meningococcal poly- phagocytosis that nonspecifcally remove invading micro- saccharide. Vitamin A defciency also compromises natural organisms, as well as the action of chemical and physical innate immunity since it is necessary for maintenance of barriers to infection such as acid in the stomach and the mucosal surfaces, the frst line of defense against infection. Other nonspecifc protective factors include lysozyme, Immune effector cells that mediate nonspecifc immunity β lysin, and interferon. Nonspecifc or natural immunity include polymorphonuclear cells, macrophages, and natural does not depend on immunologic memory. Neutrophil phagocytosis is diminished by cells represent an important part of the natural immune Vitamin A defciency, and viral infections are more severe cell system. Molecules, Cells, and Tissues of the Immune Response 155 Immunologically, vitamin A may serve as an adjuvant to ele- Vitamin E and immunity: Vitamin E is required by the vate antibody responses to soluble protein antigens in mice. It is a major antioxidant that protects cell The adjuvant effect is produced whether vitamin A is given membranes from free radical attack. Vitamin and stimulates cytotoxic cells, natural killer cells, phago- B defciency induces marked changes in immune function, cytosis by macrophages, and mitogen responsiveness. Thymic hormone activity is dimin- immunostimulatory action of vitamin E renders it useful for ished and lymphopenia occurs. Vitamin B defciency sup- therapeutic enhancement of the immune response in patients. Folate and vita- lation of immunity is particularly important in the elderly in min B12 defciencies are linked to diminished host resistance whom infectious disease and tumor incidence increase with and impaired lymphocyte function. Vitamin E facilitates host defense by inhibiting increases ciency suppresses humoral antibody responses to antigens. In vitro, vitamin E has been shown to stimulate ate interfering action on immune function. Intake of micronutrients, including the B complex which may be a mechanism to enhance immune responses vitamins two to three times higher than the U. Zinc is an element of great signifcance to the immune sys- tem as well as to other nonantigen-specifc host defenses. Vitamin C and immunity: Ascorbic acid (vitamin C) is The interleukins of the immune system play a role in zinc needed for the cells, tissues, and organs of the body to func- distribution and metabolism in the body. It is an antioxidant and a cofactor in many the active site in multiple metalloenzymes, zinc is critical in hydroxylating reactions. The immune system is sensitive to chemical prothesis within lymphocytes and leukocytes. Leukocytes have high concen- role in the reproduction of cells is of critical signifcance trations of ascorbate that is used rapidly during infection and for immunological reactions since nucleic acid synthesis phagocytosis, which points to vitamin C’s role in immunity. Zinc facilitates Vitamin C facilitates neutrophil chemotaxis and migration, cell membrane modifcation and stabilization. Zinc def- induces interferon synthesis, maintains mucous membrane ciency is associated with reversible dysfunction of T lym- integrity, and has a role in the expression of delay type hyper- phocytes in man. High dose vitamin C supplementation is believed lymphoid organs and is associated with diminished num- bers of lymphocytes in the T cell areas of lymphoid tissues. Anergy develops in zinc-defcient nonspecifc extracellular free radical injury and autotoxicity patients; this signifes disordered cell-mediated immunity as after the oxidative burst activity of stimulated neutrophils. There is also a decrease in further enhances immune function indirectly by maintaining the synthesis of antibodies to T cell-dependent antigens. Thymic hor- of vitamin D, has a signifcant regulatory role in cell differ- monal function requires zinc. It mediates also associated with decreased formation of monocytes and its action through specifc intracellular vitamin D3 receptors macrophages and with altered chemotaxis of granulocytes. Among calcitriol’s numerous effects on the immune Wound healing is impaired in these individuals who also system are the inhibition of cytokine release from monocytes; show greatly increased susceptibility to infectious diseases, the prolongation of skin allograft survival in mice; the inhi- which are especially severe when they do develop. Zinc is absorbed all along 156 Atlas of Immunology, Third Edition the small intestine but is taken up primarily in the jejunum. Thus, one gene can encode two or more Its function falls into three categories: catalytic, structural, versions of the same protein. This process leads to proteins In humans, zinc defciency causes severe growth retarda- with alternative C-terminal amino acid sequences tion and sexual immaturity. Acute zinc toxicity may occur with intakes in the range of 1 to 2 g, which leads to gastric An anti-sense oligonucleotide is an oligonucleotide created distress, dizziness, and nausea. Diagnosis of zinc defciency is dif- Transcription factors are proteins that unite with motifs in fcult because of the lack of a sensitive specifc indicator promoters and initiate, support, or block gene transcription. Chromatin remodeling complexes: Enzymatic complexes that are large, variable, and comprised of multiple compo- A eukaryote is a cell or organism with a real nucleus con- nents. Proteomics: The study of the total proteins that an organ- Genomics is the study of an organism’s complete genome. Study of the proteins that an organism manifests is termed “expression proteomics. It is the total genetic material found in the Transcriptomics is the branch of knowledge concerned haploid set of chromosomes. Germ line refers to unaltered genetic material that is trans- de novo pathway of nucleic acid synthesis: A biosynthetic mitted from one generation to the next through gametes. An mechanism whereby new nucleotides are constructed from individual’s germ line genes are those present in the zygote amino acids. TdT inserts a few nucle- otides in T cell receptor gene and immunoglobulin gene An intron is a structural gene segment that is not transcribed segments at the V-D, D-J, and V-J junctions. The base sequence in one strand is identical to of fragments terminating in a 5′-phosphoryl nucleotide. The term may also refer to the ordering Homologous recombination describes the exchange of of gene segments. Homologous recombination sequence of a true gene but does not encode a protein due to is employed to introduce a mutated version of a gene into defects that inhibit gene expression. It constitutes the combined genetic constituents inherited from both par- Nonproductive rearrangement refers to rearrangements ents and refers to the alleles present at one or more specifc in which gene segments are joined out of phase leading to loci. When referring to microinjected transgenics, mice can failure to preserve the triplet-reading frame for translation. Complementarity is a genetic term that indicates the A karyotype is the number and shape of chromosomes requirement for more than one gene to express a trait. Diploid is a descriptor to indicate dual copies of each auto- some and two sex chromosomes in a cell nucleus. In genet- numerous different signal transduction pathways in a variety ics, this refers to genes with only one allele.

5mg proscar

Selection o f surgical technique for retinal detach­ Implications of missed diagnosis in the peri-operative period proscar 5mg. M orning glory syndrom e: unusual congenital optic disk trum of hybrid disc anom alies in a single eye proscar 5 mg. Pseudodoubling of the E ntartung dcs Sehnerven m il bcsondcrer Bctciligung der optic disc: a colour Doppler im aging study 5 mg proscar. Pituitary stalk anom alies coarctation of the aorta and cardiac defects 5mg proscar, and eye duplication in association with moya moya disease and bilateral abnorm alities . Torres M , Gom ez-Pardo E , Gruss P Pax2 contributes to inner ear traction-rhcgm atogcnous retinal detachm ent in the m orning glory patterning and optic nerve trajectory . M orning glory disc expansion and contraction m utations of РЛХ2 and inclusion of anom aly in neurofibrom atosis type 2 . Arch O phthalm ol 1999;117: Chiari 1 m alform ation as part o f renal-colobom a syndrom e . O cular m alform ations, moy- apoptosis in fetal kidneys o f Pax2(lN cu) + / - m utant mice. M orning glory disk anom aly—m ore than meets the prenatal detection and clinical spectrum in a large family. Renal colobom a syn­ oidal colobom a, and congenital constrictive m alform ations of the drom e. R edefining papillorenal ol the optic disc associated with m oyam oya disease: case report). A case of peripapillary m idline cranial defects and abnorm al carotid circulation: an asso­ staphyloma. M orning glory syndrome: asso­ staphyloma with light stim ulus to the contralateral eye. Arch ciation with moyamoya disease, m idlinc cranial defects, central O phthalm ol 2001;119:1216-7. Contractile peripapillary staphy­ gioma with ipsilateral peripapillary excavation in girls: a variant of loma. O phthalm ic Genet 2001;22: o f posterior fossa brain m alform ations, hem angiom as, arterial 219-53. Ann O phthalm ol iris defects, optic pit and the m orning glory disc anom aly in a 1985;17:382-4. D om inant inheritance of graphic oscillatory potentials, optic atrophy, and dysplasia in optic pits. I Icidelbcrg retinal tomography patients with optic nerve pit and serous retinal detachm ent of the o f optic disc and nerve fiber layer in Singapore children: variations macula. La pseudo-atrophic optique dcs nouveaux-nes (dys- Gracfcs Arch Clin Exp O phthalm ol 1987;225:311-4. Prepapillary vascular m docvaninc green angiographic findings in congenital optic disk loops. A study based on nerve fibers associated with ipsilateral myopia, amblyopia, and stra­ review o f stereo fundus photographs and fluorescein angiographic bism us. A clinical analysis o f pscudo­ A sign of m ultiple basal cell nevi (G orlins) syndrom e. Chronic papilledema sim ulating hyaline bodies o f the ria centralis retinae in an eye with a persistent hyaloid artery and optic disc. H em orrhagic com plications of branch arterial occlusion associated with preretinal arterial loops. Klin Monbl Augcnheilkd Augcnarztl Fortbild defect and Marcus G unn pupillary phenom enon (Acromedical 1962;140:75-88. Л clinicopathologic classification of these active pseudostratified columnar epithelium. In some cases, correspond to the location and size of the fundus abnor punchcd-out areas of depigmentation develop in the center mality. In younger patients the scotoma tends to be relative, of the lesion and coalesce in a cauliflower like fashion. Electroretinographic and electro- lesions may involve almost one quarter of the fundus. Ihe normal choriocapillaris flush predilection for any particular fundus quadrant. There with obliteration of the retinal capillary bed, retinal capillary may be occasional focal intra-retinal pigmentation near the leakage, and presumed neovascularization in five cases. Although no histopathologic evi­ pigmentation and increases in size of the lesion should not dence is yet available, the tumor probably represents either be considered as signs of malignant degeneration. Congenital hypertrophy of the retinal features, such as hypertrophy, segmental hyperplasia, and pigment epithelium. The boundaries of larger conditions such as rubella retinopathy, pigment prolifera­ lesions may hyperfluoresce. No leakage of dye is observed tion secondary to trauma or hemorrhage, congenital during any phase of the angiogram. These lesions are local, round, sharply circumscribed at a greater risk of developing intestinal cancer. Histopathology Underlying large choroidal vessels arc usually visible The histopathologic features of grouped pigmentation of within the lesions. These cells have virtually no pigment sition between hyperpigmented and surrounding normal granules. Melanosomes were larger in the hyperpigmented cells but retained their normal football-shaped configura­ Pathogenesis tion. They The electroretinographic and electro-oculographic may be uniformly thick or may have a dimpled appear­ recordings are normal in patients with this condition. There is a focal narrowingof the major retinal vessels Dark adaptation is also normal. Choroidal shows early hyperfluorescence corresponding to most of vessels may be visible beneath some lesions. There is no leakage of dve or staining of partially melanotic spots may accompany albinotic ones. In this disorder, the retinal flecks are described as “dirty yellow” in color, and they do not affect the peripapillary or macular areas. The most common type of lesion is small, children, it is possible that this complication, reported in a flat, round, and hyperpigmented. Larger lesions, usually ovoid to round, are present close to the posterior pole and vary in color from light be exercised when interpreting pigmented fundus lesions grayish-brown to black. The results give an Visual field scotomas corresponding to large lesions indication of the severity of the intestinal disease and allow have been reported. As Chen and associates reported, carc must tion, and chorioretinal anastomoses are occasionally w. Orbital osteo­ mas have been reported in a few patients with Gardners syndrome,164,65 and epidermal cysts of the eyelid skin may occur in these patients. The combination of genetic analysis and fundus examination offers a 100% diagnostic predictability. Herrera and associates reported a patient with Gardner syndrome and a deletion involving the long arm of chromosome 5. A ciinlcopathoiogic study of the eyes in familial were found al the site where both forms of alternative splic­ adenom atous polyposis with extracolonic manifestations {Gardner’s syndrome). The absence of the ocular trait, however, does not rule out the disease in any family. Benign m elanom as o f the retinal pigment epithe­ None of the three subjects with mutations in codon 437 had lium. Prevalence and im portance o f pigm ented ocular fundus lesions in G ardners syndrom e. Enlargem ent o f congenital hypertrophy of focal lesions are explained by the overlying photoreceptor the retinal pigm ent epithelium. Retinal vascular changcs in congenital normal values recorded in patients with Gardner syndrome hypertrophy of the retinal pigm ent epithelium. Br J Ophthalm ol indicate the absence ofa detectable functional diffuse abnor­ 1976;60:499-503. Photoreceptor loss overlying congenital hypertrophy of the retinal pigm ent epithelium by optical coherencc tomography. Klin symptomatic, highly specific (> 90%), and relatively Monbl Augenheilkd 2002;219:644-8. Retina trophy of the retinal pigm ent epithelium associated with familial 2006;26:470-2. Lack of association am ong pattern o f congenital grouped albinotic retinal pigm ent epithelial typical congenital hypertrophy o f the retinal pigm ent epithelium , spots. Elcctrorctinographical studies on 1992;99:1709-13, "fleck retina with congenital nonprogressive nightblindness. A genetic and clinical study o f intestinal polyposis, a O phthalm ol 20()0;118:637-41. Arch Ophthalm ol phy o f the retinal pigm ent epithelium : clinical features and frequency 1975;93:1153. Ophthalm ologic and elcctro-oculographic find­ trophy o f the retinal pigm ent epithelium follows developm ental ings in G ardners syndrom e. M acular colobom a with bilateral grouped pigm enta­ sis of familial adenomatous polyposis. Occult radiopaque jaw congenital grouped pigm entation of the retinal pigm ent epithelium lesions in familial adenom atous polyposis coli and hereditary non associated w ith subm icroscopic deletions of 13q33. Ann O ttalm ol Clin Ocul serves as a clinical m arker in a family with familial adenom atous 1938;66:543-52. A syndrom e of multiple fundal anom alies in of the retinal pigm ent epithelium : a functional and imaging study). A histopathologic study of spots: a rare anom aly of the retinal pigm ent epithelium. G ardner syndrom e in a m an with result in a variable phenotype in familial adenom atous polyposis. Congenital hypertrophy of the terization o f the familial adenom atous polyposis coli gene. Prcdictivc diahgnosis in familial retinal pigm ent epithelium in familial adenom atous polyposis. Novel adenom atous polyposis: evaluation of m olecular genetic and oph­ criteria of assessment and correlations with constitutional adenom a­ thalmologic m ethods. Pigm ented fundus lesions in a preterm o f familial adenom atous polyposis in 11 unrelated families with infant with familial adenom atous polyposis. Singh Reports of prenatal diagnosis of eye and adnexal anomalies Using transvaginal sonography, the eyes can be detected appeared in the literature in the 1980s. By 14 weeks of age, the lens should be with multiple ocular findings; we chose to categorize them detected in all fetuses. It appears as an oval structure in the by the ocular finding that was most emphasized in the center of the orbit and is characterized by a thin cchogcnic original report. The hyaloid artery can be anomalies, we utilized only reports of cases that included a detected in almost all fetuses by the 14th week of gestation specific description of the ocular imaging. These results conflicted with those of the Helsinki Ultrasound Trial,’’ which reported a lower perinatal mortality rate in the women routinely screened by ultrasonography due to the detection of anomalies and the subsequent termination of affected pregnancies. Current recommendations from the American Institute of Ultrasound in Medicine, the American College of Radiology, and the American College of Obstetricians and s Gynecologists4 do not recommend examination of the globe, orbit, or adnexal structures as part of the routine У fetal anatomy examination. Ultrasonographic appearance of normal phy include operator experience variability, fetal position, fetal globe at 20 weeks of gestation (coronal view). Retinoblastoma: at risk replicated epidemiologic data do not exist that suggest pregnancies. An additional invagination occurs that forms a groove fully characterize a finding from fetal ultrasonography, from the ventral aspect of the optic cup posteriorly to the and nondiagnostic prenatal ultrasonography. The anterior chamber absence of the eyes, the morphology of the lens and vitreous, angle, the stroma, and the endothelium covering the poste­ and performing ocular biometry. ЛИ were diagnosed would deliver enough radiation to justify termination of ultrasonographically with characteristic features including pregnancy. Three cases were unilateral,30 32 two of which were associated with contralateral microphthalmia. Associations included “anophthalmia-plus” syndrome,33ophthalmo-acromclic syn­ drome, type Waardenburg,14otocephaly," limb body wall complex,31 holoprosencephaly,17 a family history of anophthalmia,1"" and a maternal history of recurrent miscarriages. Clinically, the differentiation between anophthalmia and extreme microphthalmia is dif­ figure 11. Prenatal ultrasonographic characteristic features include an absent or small orbit, an absent globe, ana an absent lens. Nomograms for ocular biometric measurements based Gestational age at diagnosis has ranged from 14 to 32 weeks. Sixteen of the cases were associated with holoprosencephaly, 13 of which were alobar. The ocular diameter was demise;* the other case resulted in death shortly after birth. Causes include chromosomal aberrations, single gene mutations, and of other anomalies by ultrasonography (Fig. Gestational age at diagnosis the ocular globe at the insertion of the optic nerve. Note rocal showing proboscis (arrow), absent nasal bone, flat facial profile, and bulging of posterior pole of globe (arrow).

The suprascapular nerve and artery pass proximally beneath the superior transverse scapular 276 ligament through the suprascapular notch proscar 5 mg. Coronal proton density–weighted image of the right shoulder demonstrating a cyst in the spinoglenoid notch proscar 5mg. It is arising in relation to a posterosuperior labral tear (long arrows) and extends into the spinoglenoid notch region 5mg proscar. Proximal nerve compromise results in weakness 5 mg proscar, fatty degeneration , and ultimately atrophy of both the supraspinatus and infraspinatus muscles , often associated with pain . More distal nerve compromise results in selective atrophy of only the infraspinatus muscle resulting clinically in the infraspinatus syndrome (Fig . Since the sensory branch of the suprascapular nerve exits the nerve above the spinoglenoid notch , patients suffering from compression of the nerve at this distal point will complain of weakness in external rotation of the upper extremity , infraspinatus muscle atrophy, but rarely pain. This constellation of signs and symptoms has been termed volleyball shoulder as it is most commonly seen in elite volleyball players, although it has also been reported in weight lifters, baseball pitchers, and videographers who hold heavy cameras on their shoulders for long periods of time (Fig. Patients with compromise of the distal suprascapular nerve will often exhibit a positive dropping sign (Fig. More distal compression of the suprascapular nerve presents clinically as weakness in external rotation of the upper extremity, infraspinatus muscle atrophy, but rarely pain. This constellation of signs and symptoms has been termed volleyball shoulder as it is most commonly seen in elite volleyball players, although it has also been reported in weight lifters, baseball pitchers, and videographers who hold heavy cameras on their shoulders for long periods of time. A: With the patient seated the shoulder is placed in 0 degrees of abduction and 45 degrees of external rotation with the elbow flexed to 90 degrees. The examiner holds the patient’s forearm in this position and instructs the patient to maintain it when he lets go of the forearm. B: On releasing the forearm a positive test is recorded when the patient’s forearm drops back to 0 degrees of external rotation, despite efforts to maintain external rotation. With the patient in the neutral sitting position, the spine of the scapular is palpated and a high-frequency linear ultrasound transducer is then placed in a coronal position just above and parallel to the scapular spine (Fig. Since the spine of the scapula runs at approximately a 33-degree angle to the superior margin of the scapula, the transducer should be rotated with the superior end of the transducer aimed toward the medial aspect of the coracoid process. When the ultrasound transducer is parallel to the scapular spine, the spine will appear as a bright, linear hyperechoic line that does not move with respiration like the pleura does. After the spine of the scapula is identified, the ultrasound transducer is then slowly moved in a cephalad and lateral trajectory until the U-shaped suprascapular notch is identified (Fig. The suprascapular nerve should be visible within the notch appearing as a 2- to 3-cm hyperechoic area beneath the suprascapular ligament. The suprascapular artery and vein should lie just above the suprascapular nerve and their exact location relative to the suprascapular nerve can be identified with the use of color Doppler (Fig. The suprascapular notch and the suprascapular nerve within are evaluated for the presence of osteophyte, ganglion cysts, or other mass that may be entrapping the suprascapular nerve (Fig. The superior transverse scapular ligament is then evaluated for hypertrophy or other abnormality (Fig. Proper coronal ultrasound transducer placement for ultrasound evaluation of the proximal suprascapular nerve as it passes through the suprascapular notch. Transverse ultrasound image of the suprascapular notch, nerve and overlying ligament and musculature. Use of color Doppler can aid in the identification of the suprascapular artery and vein. The cyst lies on the floor of the fossa deep to the deltoid (Del) and supraspinatus (SupS) muscles. Transverse ultrasound image of the suprascapular notch and superior transverse scapular ligament. To perform ultrasound evaluation of the more distal portion of the suprascapular nerve as it passes through the spinoglenoid notch, the patient is then placed in the sitting position with the affected extremity reaching across the chest to hold the contralateral shoulder (Fig. The high-frequency linear ultrasound transducer is then placed in the transverse plan just below the acromion and slowly rotated until the spinoglenoid notch is visualized (Figs. A: To perform ultrasound evaluation of the more distal portion of the suprascapular nerve as it passes through the spinoglenoid notch, the patient is then placed in the sitting position with the affected extremity reaching across the chest to hold the contralateral shoulder. B: Proper placement of the linear ultrasound transducer 281 to evaluate the distal suprascapular nerve as it passes through the spinoglenoid notch. Transverse ultrasound image of the distal suprascapular nerve at the spinoglenoid notch. Proximal nerve compromise results in weakness, fatty degeneration, and ultimately atrophy of both the supraspinatus and infraspinatus muscles, often associated with pain. More distal nerve compromise results in selective atrophy of only the infraspinatus muscle resulting clinically in the infraspinatus syndrome (Fig. Since the sensory branch of the suprascapular nerve exits the nerve above the spinoglenoid notch, patients suffering from compression of the nerve at this distal point will complain of weakness in external rotation of the upper extremity, infraspinatus muscle atrophy, but rarely pain. Because the bony components of the spinoglenoid notch can limit thorough evaluation of the distal suprascapular nerve, magnetic resonance imaging may provide additional useful clinical information. It should be remembered that complete rupture of the infraspinatus musculotendinous unit may present in a clinically similar manner to compromise of the distal suprascapular nerve (Fig. Sagittal T2-weighted, fat-suppressed magnetic resonance image demonstrating a full-thickness infraspinatus tendon tear at its insertion site on the humerus (arrow). The effectiveness of ultrasonography-guided suprascapular nerve block for perishoulder pain. The shoulders of professional beach volleyball players: high prevalence of infraspinatus muscle atrophy. Injection technique for suprascapular nerve block In: Atlas of Pain Management Injection Techniques. In: Comprehensive Atlas of Ultrasound-Guided Pain Management Injection Techniques. The space lies just below the inferior aspect of the capsule of the glenohumeral joint (Fig. Contained within the quadrilateral space is the axillary nerve which is a branch of the brachial plexus and the posterior circumflex humeral artery. Compromise of either of these structures by tumor, cyst, hematoma, aberrant muscle, fibrous bands, stretch injury, compression by inferior migration of the humeral head, or by heterotropic bone can produce the constellation of symptoms known as quadrilateral space syndrome (Figs. Illustrations of the intimate relationship of the teres minor nerve with the inferior capsule (blue) in the sagittal (A) and coronal (B) orientations. Isolated teres minor atrophy: manifestation of quadrilateral space syndrome or traction injury to the axillary nerve? A: T1-weighted short inversion time inversion-recovery coronal oblique image, showing to inferior labrum. Intraoperative view of the left shoulder from the posterior aspect, showing the decompressed axillary nerve within the quadrilateral space. A case of quadrilateral space syndrome with involvement of the long head of the triceps. Diagrammatic representation of a rotator cuff tear with humeral decentering and traction on the teres minor nerve. There is resultant denervation of the muscle with fatty atrophy and sparing of the other terminal branches of the axillary nerve. Isolated teres minor atrophy: manifestation of quadrilateral space syndrome or traction injury to the axillary nerve? Cahill and Palmer first described this syndrome in 1983, with most patients being young athletes in their second and third decade of life. The use of magnetic resonance scanning and ultrasound imaging of the quadrilateral space has made the diagnosis of quadrilateral space syndrome more objective and it is now being diagnosed in all age groups (Figs. Arteriography, color Doppler, and magnetic resonance arteriography will aid in identification of compromise of the posterior circumflex humeral artery (Figs. T1-weighted paracoronal image identifying the quadrilateral space (black arrow) bound by the humerus laterally, teres major inferiorly, triceps medially and, in this case, an atrophied teres minor superiorly (white arrow). Isolated teres minor atrophy: manifestation of quadrilateral space syndrome or traction injury to the axillary nerve? Magnetic resonance imaging demonstrates fatty atrophy of the teres minor in patient with quadrilateral space syndrome (white arrow). With the affected extremity in neutral position, the posterior humeral circumflex artery fills normally (white arrow). With abduction of the affected extremity, the posterior humeral circumflex artery is truncated (black arrow). Ultrasound findings of teres minor denervation in suspected quadrilateral space syndrome. Magnetic resonance angiogram showing narrowing of the posterior circumflex artery as it passes 288 through the quadrilateral space consistent with a clinical diagnosis of quadrilateral space syndrome (arrow). A case of quadrilateral space syndrome with involvement of the long head of the triceps. The pain is often described as aching in nature with superimposed dysesthesias often present. Adduction and external rotation of the affected upper extremity will make the symptoms worse. If the compromise of the axillary nerve remains untreated, the patient may begin to notice the gradual onset of weakness of the affected upper extremity, particularly when abducting and externally rotating it. In some patients, the weakness is progressive and permanent atrophy of the deltoid and teres minor muscles can be identified on physical examination and medical imaging studies (Fig. Paresthesias and numbness in the distribution of the axillary nerve may be present with the patient often illustrating a positive sergeant chevron sign. There often is tenderness to palpation of the quadrilateral space on physical examination. As with other entrapment neuropathies such as carpal tunnel syndrome, electromyography may help identify compromise of the axillary nerve and help distinguish quadrilateral space syndrome from the brachial plexopathies and cervical radiculopathy which may both mimic quadrilateral space syndrome. Point tenderness with compression over the quadrilateral space (looped line area) and deltoid atrophy. C: One of the wounds from the operation (third intercostal space midaxillary line) is seen. With the patient in the above position, at the middle of the posterior arm, a high- frequency linear ultrasound transducer is placed in a longitudinal orientation over the medial side of the posterior humerus (Fig. An ultrasound survey image is obtained and the hyperechoic margin of the shaft of the humerus and the adjacent triceps muscle is identified (Fig. The transducer is then slowly moved toward the axilla while tracing the hyperechoic margin of the humerus until the hyperechoic margin curves outward as the transducer approaches the inferior head of the humerus. Color Doppler may be helpful in identifying the posterior circumflex humeral artery (Fig. The quadrilateral space is then evaluated for the presence of abnormal mass, inferior migration of the humeral head and joint capsule, aneurysm, and tumor. Dynamic scanning utilizing color Doppler with the arm in neutral and hyperabduction (over 120 degrees) and external rotation of the arm may aid in identification of occlusion or compromise of the posterior circumflex humeral artery (Fig. Comparison imaging of the teres minor muscles to identify muscle atrophy should also be carried out in all patients suspected of suffering from quadrilateral space syndrome (Fig. Proper longitudinal placement of the ultrasound transducer for ultrasound-guided axillary nerve block in the quadrilateral space. Longitudinal ultrasound view of the margin of the midhumeral shaft and adjacent triceps muscle. The ultrasound transducer is slowly moved toward the axilla while tracing the hyperechoic margin of the humerus until the hyperechoic margin curves outward as the transducer approaches the inferior head of the 290 humerus. Below the point of this outward curve lies the axillary nerve and posterior circumflex humeral artery within the quadrilateral space. Use of color Doppler can aid in the identification of superior circumflex humeral artery. A: Transverse sonogram of right teres minor muscle (arrows) shows increased muscle echogenicity with slight loss of muscle bulk. B: Transverse sonogram of the normal left teres minor muscle (arrows) is shown for comparison. Ultrasound findings of teres minor denervation in suspected quadrilateral space syndrome. It occurs when the axillary nerve and/or posterior circumflex humeral artery are compromised as the traverse the quadrilateral space. Whether the ensuing constellation or signs and symptoms that comprise quadrilateral space syndrome are purely neurogenic or vascular or a combination of both can be best ascertained by careful physical examination combined with the targeted use of ultrasound and magnetic resonance imaging (Fig. Ultrasound-guided injection of the axillary nerve at this level with local anesthetic and steroid may serve as both a diagnostic and therapeutic maneuver (Fig. Ultrasound findings of teres minor denervation in suspected quadrilateral space syndrome. Proper needle placement for ultrasound-guided axillary nerve block in the quadrilateral space. The anatomy of the quadrilateral space with reference to quadrilateral space syndrome. As the radial nerve exits the axilla, it passes between the medial and long heads of the triceps muscle and then curves across the posterior aspect of the humerus, giving off a motor branch to the triceps muscle. Continuing its downward path, the radial nerve gives off a number of sensory branches to the upper arm as it travels in the intermuscular septum separating the bellies of the brachialis and brachioradialis muscles. It is at this point that the nerve is often damaged by fractures of the shaft of the humerus or by subsequent open reduction and internal fixation of the fracture (Figs. At a point above the lateral epicondyle, the radial nerve divides into deep and superficial branches, with the superficial branch continues down the arm along with the radial artery to provide sensory innervation to the dorsum of the wrist and the dorsal aspects of a portion of the thumb and index and middle fingers and the deep branch provides the majority of the motor innervation to the extensors of the forearm (Fig. The radial nerve arises from fibers from the C5–T1 nerve roots of the posterior cord of the brachial plexus. The radial nerve passes through the axilla lying posterior and inferior to the axillary artery.

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