Its prognostic impact has been demonstrated in univariate analysis but had no inde- tric branch of the left gastroepiploic vein purchase silagra now erectile dysfunction hypertension drugs. Tumor infiltration (arrowhead) is also noted in the pendent prognostic significance in the multivariate 19 gastrosplenic ligament purchase 50mg silagra overnight delivery erectile dysfunction zoloft. Imaging studies may demonstrate the penetration of tumor outside the wall of the stomach buy 100 mg silagra with mastercard impotence remedies, butitisdifficulttospecifyifitisduetoperineural may have prognostic implications in univariate analy- invasion (Fig. In imaging studies, diag- nosis should be suggested when tumor nodules or tracks of tumor can be identified extending from the primary tumor along the perigastric arteries (Fig. In Stranding S (ed) Gray’s Anatomy, the Anatomical Basis of Clinical Practice, 40th ed. Perineural invasion in a patient with carcinoma at Livingstone, Elsevier, London, 2008, pp 1099– the gastric antrum. Thieme Medical tumor thrombus growing into the perigastric veins Publishers, Inc, New York, 1997, pp 1–23. In Stranding S (ed) Gray’s Anatomy, Prognostic impact of lymphatic and/or blood ves- the Anatomical Basis of Clinical Practice, 40th ed. Shiraishi N, Sato K, Yasuda K, Inomata M, identifies early-stage high-risk radically resected Kitano S: Multivariate prognostic study on large gastric cancer patients. P atterns of S pread of D isease from theP ancreas ventral diverticulum is associated with the liver and Introduction bile duct diverticulum. It branches out proximally from the liver diverticulum close to the foregut and it The pancreas is considered a secondary extraperito- is located in the ventral mesoduodenum. During embryologic development, the the two diverticula evaginates from the foregut cepha- body and tail of the pancreas are suspended in the lad to the liver diverticulum. It branches into the peritoneal cavity in the dorsal mesoduodenum and dorsal mesoduodenum and extends into the dorsal the head in the ventral mesoduodenum. These diverticula develop into pan- of the foregut, outpouching of the dorsal mesogas- creatic ducts; the pancreatic acinar cells and endocrine trium, and migration of the midgut, the posterior cells are also formed from this ductal element. The ventral pancreatic bud becomes the cau- the small and large bowel below the transverse meso- dal portion and the uncinate process of the head of the colon, and the extraperitoneal organs. In this chapter, pancreas, while the dorsal bud becomes the cranial we review developmental anatomy of the pancreas and portion of the head, body, and tail of the pancreas. The dorsal duct Embryology and Anatomy that drains the body and tail of the pancreas as well as of the Pancreas the cephalad portion of the head may persist and drain into a minor papilla; this segment is known as the accessory duct of Santorini. Development of the Pancreas As the rotation of the foregut progresses, the dorsal The pancreas develops from two endodermal diverti- mesoduodenum and mesogastrium fuse with the par- 1–3 ietal layer of the peritoneum and become the posterior cula from the foregut that form the duodenum. Outpouching of the dorsal meso- The head of the pancreas connects to the liver and gastrium between the stomach and pancreas occurs to the lesser curvature of the stomach via the hepatoduo- form the omentum. The posterior leaf of this extension denal and gastrohepatic ligaments (part of the ventral fuses with the mesentery of the midgut that develops mesogastrium), while the tail communicates with the into the transverse colon to form the transverse meso- hilum of the spleen and the greater curvature of the colon over the pancreas. The pancreas is embedded in stomach via the splenorenal and gastrosplenic liga- 2–6 the extraperitoneum except for the most distal portion ments (part of the dorsal mesogastrium). The splenic ior peritoneal layer that forms the posterior wall of the artery and vein course within the ligament to the lesser sac and the posterior peritoneal layer that covers spleen. The root of the trans- verse mesocolon traverses the second portion of the Anatomy of the Pancreas and Peritoneal duodenum, the head of the pancreas, and the caudal Ligaments Around the Pancreas, surface of the body and tail of the pancreas. Mesentery, and Mesocolon The small intestinal mesentery is formed by the posterior peritoneal layers that cover the ascending The pancreas lies transversely along its long axis in the and descending mesocolon. It runs obliquely to the right iliac fossa terior surface of the head is separated from the inferior crossing the horizontal portion of the duodenum, vena cava by only extraperitoneal fat and on occasion abdominal aorta, inferior vena cava, and right ureter. Thus, precisely identifying the spe- cific vascular architecture underlies the position and course of major peripancreatic ligaments and perito- neal folds. Table 10–1 lists the vascular landmarks of these ligaments and peritoneal folds around the pancreas. The head of the pancreas is supplied by a network of arteries around the head of the pancreas and this network originates from three 2–4 major arteries: The gastroduodenal artery descends from the com- Fig. The gastroduodenal artery continues its course in the craniocaudal direction anterior to the head of the pancreas and then bifurcates to form a branch coursing anteriorly in the gastrocolic ligament to become the right Table 10–1. Vascular Landmarks of Pancreatic Ligaments and Peritoneal Folds Peritoneal ligaments and fold Relation to organs Landmarks Hepatoduodenal From duodenum to right hilar fissure Hepatic artery, portal vein, bile duct ligament Gastrohepatic ligament Lesser curvature of stomach to liver hilum Right gastric artery and vein Gastropancreatic fold Posterior wall of the lesser sac above the Left gastric artery pancreatic body Splenorenal ligament From extraperitoneum anterior to left kidney to Splenic artery and vein splenic hilum Transverse mesocolon Transverse colon to pancreatic head Middle colic artery and vein, gastrocolic trunk Along caudal surface of pancreatic body and tail Left middle colic vein to splenic vein or inferior mesenteric vein Root of small bowel Duodenojejunal junction to right iliac fossa Superior mesenteric artery and vein, ileocolic mesentery artery and vein 262 10. The other branch con- body and tail of the pancreas and anastomoses tinues in the craniocaudal direction close to the with small branches of the splenic artery. The anatomy of these veins is relatively a branch of the dorsal pancreatic artery. It can be identified behind the proximal proximal segment of the jejunum frequently drains portion of the body of the pancreas. The gastrocolic trunk, vein, which runs medially along the head of the which is formed by the right gastroepiploic vein, the pancreas and anastomoses with a peripancreatic middle colic vein, and the right colic vein, runs in the arcade around the head of the pancreas. The portal vein then ascends usually develop in the subhepatic recess or peritoneal behind the head of the pancreas to enter into the hepa- recess below the transverse mesocolon. Venous drainage of the body and tail of the pancreas The primary tumors in the tail and body frequently is more variable, but it consists of multiple small present with advanced disease because they lack symp- branches draining into the splenic vein along the tail toms and they are more likely to disseminate into the and body of the pancreas. Patterns of Spread of Disease from the Pancreas Subperitoneal Spread Intraperitoneal Spread Contiguous Subperitoneal Spread Even though the pancreas is an extraperitoneal organ, This mode of spread is very common in acute pancrea- it is covered by peritoneal lining of the posterior wall titis. Leakage of pancreatic enzymes may dissect into of the lesser sac and posterior peritoneal layers that the subperitoneal space of the peritoneal ligaments, 7,8,24 form the ascending and descending mesocolon. Hematoma in the lesser sac developed after aspiration biopsy of a neuroendocrine carcinoma of the pancreatic body. Note displacement of vessels (arrow) in the transverse mesocolon laterally and caudally. Note anterior displacement of the gastroepiploic vessels in the gastrocolic omentum, the anterior boundary of the lesser sac (arrow). Pancreatic ductal adenocarcinoma commonly posterior pancreaticoduodenal nodes by following invades the adjacent peritoneal ligaments. However, the inferior pancreaticoduodenal artery to the unlike pancreatitis that can spread further away superior mesenteric artery node (Fig. The sionally, they may also drain into the node at the contiguous spread in pancreatic adenocarcinoma proximal jejunal mesentery (Fig. This feature will be described later in lects lymphatics along the medial border of the this chapter. Lymphatic Spread and Nodal Metastasis Lymphatic drainage of the head of the pancreas is dif- The lymphatic drainage of the body and tail of the ferent from that of the body and tail. The head of the pancreas follows the dorsal pancreatic artery, the sple- pancreas and the duodenum share similar drainage nic artery, and vein to the celiac lymph node. They can be divided into three major and duodenal cancer and they carry a poor prog- routes: the gastroduodenal, the inferior pancreaticoduo- 11–13 nosis. Because of Around the head of the pancreas, multiple lymph the lack of accuracy, peripancreatic lymph nodes and nodes can be found between the pancreas and duo- the nodes along the gastroduodenal artery and inferior denum above and below the root of the transverse pancreaticoduodenal artery are included in radiation mesocolon and anterior and posterior to the head field, and they are routinely resected at the time of of the pancreas. However, it is important these nodes such as the inferior and superior pan- to note when an abnormal node, such as one with low creaticoduodenal nodes, they can be designated density and/or irregular border, is detected beyond the peripancreatic nodes. Periarterial and perineural invasion is common in The inferior pancreaticoduodenal route also pancreatic ductal adenocarcinoma (Figs. Pancreatitis with pancreatic inflammatory tissue at the gastropancreatic fold, splenorenal ligament, gastrosplenic ligament, transverse mesocolon, and in the anterior pararenal space.

Light freezing with liquid nitrogen has been advo- in hair-bearing areas of skin and does not occur on the palms cheap 100mg silagra amex impotence nutrition, cated for smaller flat lesions (1) silagra 50mg with visa how to treat erectile dysfunction australian doctor. With time 50mg silagra free shipping erectile dysfunction drugs in ayurveda, it may become more elevated, and even assume a dome configuration (1–5). It is usually discrete and movable, and has been compared to a but- ton stuck on the surface of the skin. A characteristic fea- ture is the presence of intraepithelial keratin cysts (horn cysts or pseudohorn cysts) that should not be confused with the pearl cysts, which are often present in squamous cell carci- noma. These cysts gradually coalesce and migrate superfi- cially, forming a rough surface to the lesion (3). Management Treatment is generally observation or excision, depending on the clinical circumstances (4,11–14). In the eyelid area, a lesion can be removed for cosmetic considerations or because Chapter 1 Benign Tumors of the Eyelid Epidermis 7 Selected References 1. The lesions are mainly in the periocular area, but some are present on the face and the neck. Seborrheic keratosis at lateral aspect of right upper eyelid upper eyelid in an 82-year-old man. Arch Oph- lesion that occurs mainly in middle aged to older adult men thalmol 1963:69:698–707. Am J Ophthalmol licular keratosis” was introduced by Helwig in 1954 because of 1979;87:810. Inverted follicular keratosis clinically mim- later reports suggest that it is not of hair follicle origin, but icking malignant melanoma. Carbon dioxide laser surgery of the papillomatous and pigmented, and is often located on or very eye and adnexa. Of the 65 eyelid cases studied by Boniuk and Zimmerman (1), the mean age of the patienThat diagnosis was 69 years; 43% occurred at the eye- lid margin, and 5 presented as a cutaneous horn. In another series that included all locations, 34 of 40 cases occurred on the face and only 2 were on the eyelids (3). It should include any keratotic pigmented skin lesion, particularly melanoma, melanocytic nevus, and pigmented basal cell carcinoma (1–5,9). The epithelium typically shows localized, ill-defined squamous eddies within the acanthotic epithelium. However, there has been no con- vincing histopathologic evidence to support that concept and it is now believed to be an inverted irritated seborrheic keratosis. The lesion can be observed or excised depending on the clinical circumstances (1–8). In the eyelid area, a lesion can be removed for cosmetic considerations or because of interference with wearing of glasses. Removal can be accom- plished by curettage, shaving excision flush with the skin sur- face, and/or cryotherapy (1). Chapter 1 Benign Tumors of the Eyelid Epidermis 11 ■ Eyelid Inverted Follicular Keratosis Figure 1. Inverted follicular keratosis on upper eyelid in a 24-year- in an 80-year-old man. Histopathology of inverted follicular keratosis showing invasive acanthosis and keratin cysts. It can also occur at the margins of some malignant neo- J Cutan Pathol 1974;1:231. However, the squamous cells usually are better differentiated and not so invasive as with squamous cell carci- noma. Inflammatory cells are frequently presenThat the base of the lesion and microabscesses are often detectable in the acanthotic epithelium (1,2). The adjacent inflammatory infiltrate should be studied carefully to rule out granulomatous inflammation secondary to tuberculosis, blastomycosis, and other predisposing condi- tions (4–6). Chapter 1 Benign Tumors of the Eyelid Epidermis 13 ■ Eyelid Pseudoepitheliomatous Hyperplasia Figure 1. Pseudoepitheliomatous hyperplasia secondary to blasto- thus simulating basal cell carcinoma. Local injection of chemotherapeutic ranged from 27 to 78 years, with a mean of 59 years (6). In the eyelid, it can assume a variety of forms from Clinical Features a sessile keratotic plaque to a cutaneous horn. Well-differentiated squamous cell carcinoma of the eyelid arising during a 20-year period. Aggressive keratoacanthoma of the eyelid: “malignant” keratoacanthoma or squamous cell carcinoma? Most authorities now recommend resection rather Chapter 1 Benign Tumors of the Eyelid Epidermis 15 ■ Eyelid Keratoacanthoma Figure 1. Histopathology of another eyelid keratoacanthoma showing proliferating squamous cells, with acanthosis, hyperkeratosis and a cen- well circumscribed lesion with central keratin-filled crater. Chapter 1 Benign Tumors of the Eyelid Epidermis 17 ■ Eyelid Keratoacanthoma: Clinicopathologic Correlation in an Elderly Man Figure 1. Higher power histopathology showing necrosis (pink area) cumscribed mass with hyperkeratosis and acanthosis. Pigmented cutaneous horn in an 70-year-old African upper eyelid in a 65-year-old woman. Histopathology of a cutaneous horn, showing layers of the white appearance due to extensive layers keratin comprising the eosinophilic keratin. C 2 Premalignant and Malignant T umors of Eyelid Epidermis 20 Part 1 Tumors of the Eyelids Eyelid Actinic Keratosis General Considerations (1). Topical chemotherapy using 5-fluorouracil cream has been applied twice daily for 2 to 3 weeks (1). Many aging changes in the skin, including the eyelid, are sec- There has been recent interest in treatment of actinic ondary to gradual damage from lifelong exposure to ultravio- keratosis of the face and bald areas of the head with topical let light. In a randomized, is a common precancerous cutaneous lesion that affects face, double-blind, parallel-group, vehicle-controlled trial of 492 dorsa of the hands, bald areas on the head in men, and com- patients, complete and partial clearance rates for imiquimod- monly the eyelids (1). A report from Japan found a cally and statistically significantly higher than for vehicle- mean patient age at diagnosis of 62 years and a slight predilec- treated patients (7. If untreated, approximately 20% are percentage reduction of baseline lesions was 86. It was concluded that 5% imiquimod cream applied 3 Clinical Features times weekly for 16 weeks is safe and effective for the treat- Actinic keratosis has several clinical variations, but is usually ment of actinic keratosis (11). As mentioned, they generally occur an excellent prognosis because local invasiveness is minimal on sun-exposed areas of older Caucasians who have had and metastasis occurs in only 1% to 3% of cases (10). The differential diagnosis includes most of the benign and malignant epidermal lesions mentioned in this atlas. Some actinic keratoses are pigmented, making the clinical dif- ferentiation from lentigo maligna and early melanoma difficult. The presence of multiple actinic lesions in the adjacent skin can facilitate the diagnosis.

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Shoulder joint synovial membrane • Lines the deep surface of the fbrous capsule silagra 100mg low price erectile dysfunction from smoking, covering the articular cartilage purchase 50 mg silagra otc ayurvedic treatment erectile dysfunction kerala. Divided into types 1–3 depending on the proximity of the anterior capsular insertion to process and deltoid muscle (the subacromial- the glenoid articular surface order 50 mg silagra mastercard erectile dysfunction treatment for diabetes. The ligaments are static stabilizers, namely: Subacromial · the glenohumeral ligaments ( Fig. The subacromial- in the bicipital groove and prevents it from dislocating subdeltoid bursa lies superfcial to the rotator cuf tendons and deep to the acromion and deltoid muscle. As a result, on movement of the shoulder out (particularly during abduction) it can become easily impinged. Biceps pulley system/rotator interval Glenoid labrum • Rotator interval: • Fibrocartilaginous rim around the periphery of the · triangular interspace between the supraspinatus and glenoid. Buford complex: normal variant anatomy of the glenoid labrum which mimics a labral tear. There is defciency of the anterosuperior glenoid labrum (black arrowhead) with associated thickening of the middle glenohumeral ligament (white arrow). Sublabral foramen: normal variant anatomy of the glenoid labrum which mimics a labral tear. There is separation of the anterosuperior glenoid labrum from the underlying glenoid with contrast seen in between (white arrow). The foramen never extends posterior to the attachment of the long head of the biceps tendon. Capsule Coracoacromial arch Middle glenohumeral ligament • Formed (from anterior to posterior) by the coracoid Anterior band process, coracoacromial ligament and acromion. Inferior glenohumeral ligament • Located within it (from superior to inferior) are the Posterior band subacromial-subdeltoid bursa, supraspinatus tendon and Inferior glenohumeral ligament long head of biceps tendon. Axilla Cross-sectional imagingThe axilla is a pyramidal-shaped potential space through which structures from the neck and chest pass into the upper limb and vice versa. Subscapularis tendon B Acromio-clavicular joint Acromion of scapula Clavicle Coracohumeral ligament Supraspinatus m. Long head Coracoid process of biceps tendon scapula Glenohumeral ligament Glenohumeral ligament (inferior) posterior band (middle) Subscapularis m. Supraspinatus Long head of biceps tendonThe contents of the axilla include the major neurovascular Coracoid process structures (subclavian and axillary artery and vein and the brachial plexus) of the upper limb, lymphatics and the proxi- Glenoid cavity Labrum mal parts of the biceps brachii and coracobrachialis muscles ( Figs. Fibrous capsule Brachial plexus Infraspinatus Nerve plexus made up of: • fve roots (anterior rami of C5 to T1), which form into Subscapularis • three trunks (upper, middle and lower), which divide Teres minor into • six divisions (anterior and posterior from each trunk), which form into • three cords (lateral, medial and posterior): · the musculocutaneous nerve is the continuation of the lateral cord · the ulnar nerve is the continuation of the medial cord · the radial and axillary nerves are the continuation of the posterior cord · the median nerve is the continuation of a Fig. B Acromion of scapula Acromio-clavicular joint Clavicle Supraspinatus tendon Glenoid labrum Supraspinatus m. Pectoralis 3rd–5th ribs Coracoid process Depresses • Each compartment contains its own muscles and minor of scapula shoulder; elevates 3rd–5th ribs if neurovascular supply ( Fig. Arm Posterior (extensor) fascial compartment Plain radiographic anatomy • Muscular contents: triceps brachii muscle Humerus ( Fig. Transverse humeral ligament Greater tuberosity Long head of biceps of humerus tendon Lesser tuberosity of humerus Bicipital groove humerus Head of humerus Subcutaneous fat Subcutaneous B C fat Deltoid m. Superior glenohumeral ligament Long head of Subscapularis tendon biceps tendon Long head of biceps tendon Head of humerus Supraspinatus tendon Head of humerus Subcutaneous fat D E Subcutaneous fat Deltoid m. Brachial plexus Clavicle divisions Brachial plexus Brachial plexus trunks divisions Subclavian a. Brachial plexus divisions Roots (anterior rami of C5-T1) Trunks (superior, middle, inferior) Divisions (anterior, posterior) Musculo- C5 cutaneous Lateral Superior Cords (medial, lateral, posterior) Anterior Terminal n. C6 Median Posterior Middle Posterior C7 Radial C5 C8 C6 Medial Inferior Anterior C7 C8 Ulnar T1 Clavicle T1 Roots Trunks Subclavian a. Brachialis Anterior lower Coronoid Flexes • When fully extended, the long axis of the forearm lies half of humerus process and forearm laterally at an angle to the long axis of the forearm, termed tuberosity of the carrying angle. Humerus Anterior (flexor) compartment Posterior (extensor) compartment Intermuscular septae Deep fascia Skin Medial Lateral Fig. Cross-sectional anatomy • Base: imaginary horizontal line connecting the epicondyles Elbow joint ligaments ( Fig. Radius and ulnaThe radius and ulna are long triangular-shaped bones with Median nerve at the elbow anterior, posterior and interosseous borders, the latter of • Located in the anterior cubital fossa, anterior to the which allow attachment of the interosseous membrane which brachialis muscle and deep to the bicipital aponeurosis. The proximal radius is formed of the: • Gives of the anterior interosseous nerve branch near the • head, for articulation with the capitellum of the humerus bifurcation of the brachial artery. The distal radius is formed of the: • broad distal surface, for articulation with the carpal bones at the wrist joint and with the distal ulna at the distal radio- ulnar joint • styloid process, which provides attachment for the brachioradialis muscle and the radial collateral ligament of the wrist joint. The proximal ulna is formed of the: • olecranon process with its trochlear fossa for articulation with the trochlea of the humerus • coronoid process, a proximal and lateral protuberance that allows articulation with the radial head. The distal ulna is formed of the: • head for articulation with the carpal bones at the wrist joint and with the distal radius at the radio-ulnar joint • styloid process, which provides attachment for the ulnar collateral ligament of the wrist joint. The normal carrying angle of 15–20° is the angle formed between the long axes of the arm and forearm. Lateral supracondylar Humerus ridge humerus Medial supracondylar Triceps brachii m. Pronator teres Common extensor Olecranon fossa tendon origin humerus Lateral epicondyle Common flexor tendon humerus origin Anconeus Medial epicondyle humerus Olecranon ulna Ulnar n. Radial diaphysis 8 weeks gestation • Nerve supply: median nerve except for the fexor Proximal radius 4–6 yrs 13–16 yrs carpi ulnaris and the medial part of the fexor Distal radius 1 yr 16–18 yrs digitorum profundus which are supplied by the ulnar Ulnar diaphysis 8 weeks gestation nerve. Proximal ulna 8–10 yrs 13–15 yrs • All four muscles in the superfcial layer have a common origin from the medial epicondyle of the humerus, the Distal ulna 5–7 yrs 16–18 yrs common fexor origin (Tables 15. Cross-sectional anatomy Posterior (extensor) fascial compartment of the forearm • Similarly to the arm, the forearm is encircled by a sheath of •The muscles of the posterior compartment can be deep fascia, attached to the posterior subcutaneous border divided into superfcial and deep layers. This is a synovial ellipsoid joint with articulation between the distal radius and the scaphoid, lunate and triquetral carpal bones. Important radiographic anatomical features at the wrist • Variance refers to the articular surfaces of the radius There are important anatomical measurements to evaluate on and ulna at the level of radiolunate articulation and is posteroanterior and lateral radiographs of the wrist that help classifed into: orthopaedic surgeons plan surgery following trauma. A Radius B Ulna Anterior (flexor) compartment Posterior (extensor) compartment Intermuscular septum Interosseous membrane Deep fascia Skin Fig. E Biceps brachii Brachialis Extensor carpi radialis longus Brachioradialis Flexor digitorum profundus Flexor pollicis longus Flexor digitorum superficialis Pronator teres Flexor carpi radialis Palmaris longus Flexor carpi ulnaris Fig. Tis is a aspect of the wrist into six compartments, each with a thickening of fascia that attaches to the scaphoid and single synovial sheath containing one or more tendons trapezium bones on the radial side and to the pisiform ( Table 15. Guyon tunnel • This is a fbro-osseous tunnel that permits the passage of the ulnar nerve as well as the ulnar artery and vein. A horizontal line is drawn perpendicular to the longitudinal axis of the radius at the articulation with the ulna. A second line is drawn joining the radial styloid • It has a proximal radial wall formed by the pisiform and a process and the ulnar aspect of the articular surface of the radius. A horizontal line is drawn at the level of the radial styloid, perpendicular to the The radial styloid process lies 9–12 mm distal to the ulnar articular surface. A second line is drawn joining the dorsal and The radial and ulnar articular surfaces are at the same level. The palmar inclination is the angle formed between these two lines, normally 15–20°.

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In one study [7] dental abnormalities were identi- tyly and the severity of dental and urologic fndings 100 mg silagra with amex icd 9 code for erectile dysfunction due to diabetes, but not fed in 33 of 73 patients cheap 50 mg silagra with visa impotence hypertension. Camptodactyly may be discolored teeth buy 100 mg silagra fast delivery erectile dysfunction in diabetic subjects in italy, hypoplastic enamel, multiple caries, and en- masked by syndactyly and will become manifest after releasing larged pulp chambers of the permanent teeth [13]. Camptodactyly in combination with clinodac- ifestations include a narrow nasal bridge with narrow nostrils. Early release of these Systemic Neurologic disorders are common including neu- digits is necessary due to the discrepant growth of the ring and rogenic or spastic bladder. Teeth were the phalangeal level, the fexion deformity of the ffth digits was cor- small but good color was preserved with good hygiene. Oculodentodigital dysplasia: ulnar-sided syndactyly and its associ- ated disorders. Juvenile open angle glaucoma with microcornea in oculo-dento-digital dysplasia (Meyer-Sch- wickerath-Weyers syndrome). The question of hypertelorism in oculodentoos- plete simple syndactyly extend up to the distal phalangeal level of the seous dysplasia (Letter). The genotypic variations seen clinically are directly related to the extent of the alanine residues. They are presenThat birth and have a Synpolydactyly with foot anomalies very high phenotypic presentation within the family pedigree (. Children present primarily with hand and foot abnormalities and no other malforma- Background Muragaki et al. The level of the arborization – or level at which the synpolydactyly is an inherited human abnormality of the polydactyly starts – lies within a soft tissue syndactyly and hands and feet caused by expansions of a polyalanine stretch is diffcult to determine without radiographs. This heterozygous individual lost a digit following stunted metacarpals, and extra carpal bones. Note the similar type General musculoskeletal Abnormalities involving the distal the lumbrical muscles in the central portion of these hands portions of all four extremities, and the hands more severely have their normal skeletal origin as long as the bone is nor- than the feet. They then attach to abnormal interrupting skeletal seg- ments as they progress along the longitudinal axis of the Upper extremity The polydactyly predominantly involves ray. Distally, lateral bands may be present along the exten- the ring fnger followed by the long and, to a much lesser sor mechanism at the phalangeal level, but they are rarely in extent, the index rays of the hand. After skeletal correction these rays often have determines the classifcation of this entity (. Similarly, side-to-side fusion of phalangeal families with very high penetrance, radial (preaxial) poly- segments may create long trapezoidal-shaped phalanges. Homozygous individuals are rare, Longitudinal epiphyseal brackets and associated clinodac- but their hands and feet are easily distinguished by a number tyly are common along the ring ray in particular, but can be of features including very small and short hands and feet, seen in the long and index rays as well [13]. Much like the polydactyly throughout the entire hand, loss of the tubular thumb in Apert syndrome, the growth of its bones is dimin- structure of phalanges and metacarpals, abnormal structure ished despite correction (. Enough phalanges of the midfoot with intact talus and calcaneus, and very small for two or more separate digits may be supported by only phalanges with or without symphalangism. The ring ray of the hand is consistently the hands and feet are severely impaired. Polydactyly of the ffth digit may also be seen, and clinodactyly of this digit is present on all severely Lower extremity In the most extensive series, a Turkish involved hands. Ankle and hind foot hands are often misaligned and the lack of their function were normal. Simple syndactyly between fourth and ffth toes postoperatively causes signifcant functional disability. Occasional metatarsal syn- Both the dorsal and palmar interosseous muscles as well as ostosis is seen in the lateral midfoot region. This classifcation system parallels a complete ray within the central portion of the hand and could often that for radial polydactyly, and designates the type to the level of arbo- be interpreted as a complete index duplication (With kind permission rization of the extra skeletal parts. Synpolydactyly: clinical and molecular gitudinal epiphysial bracket extending along the entire radial border of advances. A molecular pathogen- esis for transcription factor associated poly-alanine tract expansions. Synpolydactyly 2 Syndrome 387 Synpolydactyly 2 Syndrome central digits of the hands is an inevitable consequence of the asymmetric skeletal structures (. Syndactyly 3/3-prime/4 with metacarpal and metatarsal syn- ostosis Lower extremity Foot size is smaller and there are fre- quently malformations at the hindfoot level of the calcaneus Hallmarks Complex syndactyly involving the 3rd and 4th and talus. Metatarsal fusions are present and toes are short rays of the hand with associated synostosis at the metacarpal and deviated. These children also often have polydactyly and/or phalanges with Background De Smet et al. Because metacar- pal branching and synostoses are seen, these polysyndactyly digits tend to be more severe, usually involving the long and rings rays of the hand (. In both hands the long digit has been formations involving polydactyly branching from the distal portion of pulled ulnar as part of the complex syndactyly. Metatarsal synostoses the long metacarpal and extending to involve the ring digit. Popliteal Pterygium Syndrome 389 Popliteal Pterygium Syndrome Systemic Bifd scrotum and cryptorchidism in the male and hypoplasia/aplasia of the labia majora in the female. Pubic hair distribution Faciogenitopopliteal syndrome may have abnormal extension to the inner thigh. Popliteal pterygium syndrome: a clinical study of three extremity webbing that extends from the ischial tuberosities families and report of linkage to the Van der Woude syndrome locus to the heels. Birth which extended from the buttocks to the feet with severe fex- Defects Orig Art Ser. Popliteal pterygium syndrome: a syndrome comprising cleft lip-palate, popliteal and intercrural pte- Etiology This condition is caused by a mutation in the rygia, digital and genital anomalies. Variable expression of the popliteal pterygium syndrome in two 3-generation families. Lower extremity contractures are pres- ent and more than half of the children have simple syndactyly involving the hands and/or feet (. The digital webbing helps distinguish them from other conditions such as the Escobar and Van der Woude syndromes. Upper extremity Syndactyly is present in almost two thirds of these children, may affect all four web spaces and is invari- ably simple, either complete or incomplete. Craniofacial The facial clefts may be complete or incom- plete and associated with palatal clefts. One year later the child stands only with nail plate of the great toes are small with notched eponychial folds. At the time of surgical release the c Incomplete simple syndactyly involves the thumb-index web space. Cryptophthalmos syndrome Cryptophthalmos syndactyly syndrome Upper extremity Cutaneous syndactyly occurs in more Meyer-Schwickerath’s syndrome than 50 % and may affect multiple fngers. The third interdigital web space is most commonly affected in the hand but multiple spaces Hallmarks Cryptophthalmos, ear and genital anomalies. Background Fraser syndrome [1] was named after the Cana- Lower extremity Cutaneous syndactyly occurs in more than dian geneticist C.

By R. Avogadro. Mississippi State University.