Bisphosphonates are first line for both not have sufficient power to demonstrate efficacy in hip frac- prophylaxis and treatment; calcitonin may be considered tures All post-menopausal women with a history of hip or where bisphosphonates are contraindicated or not verterbral fracture order imodium online pills, or with osteoporosis based on bone min- tolerated cheap imodium on line. It is probably less effective than combined contribution of hyperphosphataemia order 2mg imodium with amex, vitamin bisphosphonates but no direct comparisons have been D deficiency and secondary hyperparathyroidism. Raloxifene reduces the risk of breast cancer (see D deficiency in chronic renal failure results from reduced p. It In addition, reduced calcitriol results in decreased intesti- is indicated for severe post-menopausal osteoporosis or nal absorption of calcium and the subsequent hypocalcae- where bisphosphonates have proved to be ineffective. The aim of treatment is to maintain normal serum phosphate and calcium Oestrogen–progestogen. Though now out of favour (see levels and suppress secondary hyperparathyroidism in or- above), oestrogen–progestogen therapy may yet be indi- der to prevent disordered bone metabolism. Phosphate binders are the first step in the management of hyperphosphataemia and prevention of renal osteody- 3In a pivitol 19-month trial, teriparatide increased bone mineral density in strophy. The aim of treatment has been to prevent the spine and femoral neck, and rates of new vertebral fractures and non-vertebral fractures were 5% and 6. It is indicated for patients with end- hyperphosphataemia and adverse clinical outcomes, pos- stage renal disease with secondary hyperparathyroidism sible secondary to accelerated vascular calcification, pro- refractory to standard treatment. Calcium-based phosphate binders, such as calcium carbonate and calcium acetate,arethemostcom- Osteomalacia monly used agents with similar efficacy. Newer non cal- cium-based phosphate binders include the anion Osteomalacia is due to primary or secondary vitamin D de- exchange resins sevelamer hydrocholoride and sevelamer car- ficiency (see above). These have a similar phosphate lowering effect compared to calcium based agents but are associated with reduced risk of hypercalcaemia. Sevelamer hydrochloride Paget’s disease of bone may worsen metabolic acidosis thus sevelamer carbonate This disease is characterised by increased bone turnover (re- is the preferred agent. Lanthanum carbonate is a non-alu- sorption and formation) – as much as 50 times normal. The newer nitrogen-containing bispho- uating lanthanum; short-term trials suggest increased ad- sphonates (pamidronate, zoledronic acid, risedronate, alendro- verse effects compared with other binders. These bisphosphonates Phosphate binders alone may not be sufficient to control suppress bone turnover without impairing bone mineralisa- phosphate levels and prevent secondary hyperparathyroid- tion. Single fetus, longitudinal lie, cephalic presentation, 3/5th of the head is palpable P/A. Any woman who has been supervised (examined and advised) during pregnancy in an institution at least three times. What investigations are commonly advised to a pregnant woman in the antenatal clinic? Ultrasound examination in the frst trimester and/or routine anomaly scan at 18–20 weeks. Generally a pregnant woman is seen at an interval of 4 weeks upto 28 weeks, at interval of 2 weeks upto 36 weeks and thereafter weekly till the expected date of delivery. To detect any high-risk factor from the history, examination and investigations, each time she attends the clinic. Live virus vaccines (rubella, measles, mumps, varicella, yellow fever) are contraindicated. The top of the centralized uterine fundus is measured from the superior border of the symphysis pubis with a measuring tape. Engagement (by palpating the sincipital and occipital poles in cephalic presentation). Detailed fetal anatomy, viability, number of fetuses liquor volume and placental localization can be assessed. In cephalic presentation, it is heard by placing the fetoscope or the bell of the stethoscope on the spinoumbilical line depending on the side where the fetal back is. In occipitoposterior position, it is more towards the flank and is difficult to locate. What are the different methods that can help to estimate the gestational age and predict the expected date of confinement? Date of fruitful coitus — if this date can be remembered, 266 days are to be added to this date. Date of quickening — to add 22 weeks in a primigravida and 24 weeks in a multipara to this date of quickening. Review of previous antenatal records and to add the required weeks to make it 40 weeks. Preconceptional counseling means examination of the woman and counseling her about pregnancy and its outcome before the actual conception occurs. It can also reduce the complications of pregnancy as care (preventive measures) can be provided well ahead. Anemia, infections, diabetes mellitus can be detected and treated before the actual onset of pregnancy. If there is a prospect for her going into labor (ripe cervix) soon, it would be better for her to stay back specially when stays far away from the hospital. Otherwise, she may go home and come back with onset of labor pain for delivery in this institute. Ripe cervix means a transition phase of the cervix from the state of pregnancy to labor. Score: 6-13 suggests cervix is favorable and success of induction of labor is high. How can you exclude the pathologies that hinder or prevent the engagement of a normal head? Presence of placenta in the lower uterine segment (placenta previa), usually manifests with episodes of painless vaginal bleeding which is fresh and recurrent. However, ultrasonography would be helpful to exclude placenta previa or any pelvic tumor. Occipitoposterior position of the head also causes deflexion and delayed engagement. Alteration in the size and/or shape of the pelvis (due to shortening of pelvic diameters) to sufficient degree so as to alter the normal mechanism of labor for an average-sized baby. Disproportion may be either due to an average-sized baby with a small pelvis or due to a big baby with normal-sized pelvis or due to a combination of both. There are some differences in the course of labor compared to occiput- anterior position (Fig. What happens as regard to the mechanism of labor in a case with occiput posterior position? In majority (90%) of cases, there is favorable outcome as the occiput rotates anteriorly with correction of deflexion. In about 10% of cases, the outcome is unfavorable as the occiput fails to rotate anteriorly. The important determinants to this outcome are : (i) Shape of the pelvis, (ii) force of the uterine contraction, and (iii) correction of deflexion of the head. She would be investigated to rule out any cause of floating head (as discussed above) specially placenta previa, pelvic tumor or cephalopelvic disproportion. Once pelvic pathology is ruled out, we would wait for spontaneous onset of labor and by that time the fetal head is expected to be engaged.
Chest x-rays ciples used for nonpregnant patients order cheap imodium, that is discount 2mg imodium otc, aggres- are safe throughout pregnancy cheap imodium online american express, but alkaline phos- sively for cure in most cases. Low-dose bone treatment for pregnancy-associated cancers, and the scans reduce the fetal radiation exposure by half choice of operation is based not only on the same (from 0. Thus, breast radiation during preg- nancy is contraindicated, and lumpectomy and ra- diation should be offered only if the radiation can Diagnosis and Recommendation be given postpartum (i. Because ter stages of the pregnancy), or if the radiation will this patient was already in her second trimester and be delayed by prior chemotherapy. Otherwise, a was likely to be a candidate for chemotherapy, she is mastectomy is typically chosen. Reconstruction af- offered either mastectomy or lumpectomy and radia- ter mastectomy should be delayed until after deliv- tion. With either approach, the standard axillary ■ Approach evaluation for invasive cancer is still two-level axil- Once thought to be rare, pregnancy-associated lary dissection. The accuracy and safety of sentinel breast cancer (breast cancer during, or within a year node biopsy in pregnancy is unknown, and preg- after, a pregnancy) is expected to increase in fre- nant patients are excluded from pending national quency as women delay childbearing until later in trials of this technique. Further, while technetium life, when the general risk of breast cancer begins to does not cross the placenta and the standard dose of rise. At present, breast cancer is the second most 1 mCi (or less) may give a very low dose to the fetus, common malignancy in pregnancy (after cervical isosulfan blue dye is a class C drug, and has not cancer), occurring in 1 in 5,000 deliveries. No data exist to compare patient outcomes after General anesthesia and breast operations are gener- chemotherapy in patients who received it during ally safe throughout pregnancy. Nevertheless, for pregnancy with patient outcomes for those in whom pregnancies more advanced than 32 weeks, consid- systemic treatment was delayed until after delivery. Anthracyclines are considered safer than general principles of operation during pregnancy alkylating agents in pregnancy. Taxane use was apply; the surgical team should be aware of the found safe in one case report. Recent studies of physiologic changes of pregnancy that can com- dose-dense schedules excluded pregnant patients. The pregnant patient should be ing increased plasma volume, decreased albumin given preoxygenation, antacids or acid-decreasing concentration, increased liver and kidney function, medications, fetal monitoring, rapid-sequence in- and decreased gastric motility, may affect chemo- duction with cricoid pressure, and elevation of the therapy dosing. Many drugs be decreased, and that lactation after radiation is of- typically used to treat chemotherapy side effects are ten difficult due to changes in the nipple and milk safe in pregnancy, including ondansetron, haloperi- ducts. The patient recovers well and is seen in consultation by The patient begins chemotherapy at 24 weeks’ ges- a medical oncologist who advises four cycles of tation. She receives two cycles, and then, following chemotherapy with doxorubicin (Adriamycin) and a rest period, delivers a healthy 7-pound baby girl by cyclophosphamide, followed by a taxane. The patient expresses an interest in possi- Discussion bly having another child in the future. Although all chemotherapy drugs are category D (ter- atogenic), these risks have generally been seen only in Discussion the first trimester; later in pregnancy they are surpris- ingly safe, with only a 1. In the only prospec- There is no evidence that therapeutic abortion im- tive trial of chemotherapy in pregnancy to date, proves the outcome in pregnancy-associated breast Berry, at the M. A large meta-analysis recently showed no tions were few, but did include preterm delivery link between previous abortion and subsequent in- (three cases), transient newborn tachypnea (two creased risk of breast cancer. The long- malities, is not recommended during pregnancy, 244 Case 55 and is usually stopped if a breast cancer patient Suggested Readings subsequently becomes pregnant. Oophorectomy has not been shown to improve the prognosis in Beral V, Bull D, Doll R, et al. Similarly, future rative reanalysis of data from 53 epidemiological studies, in- pregnancies do not appear to increase the likelihood cluding 83,000 women with breast cancer from 16 countries. J Clin On- gest that breast cancer survivors who subsequently col 1999;17:855–861. Management of general sur- become pregnant have a better 5-year survival than gical problems in the pregnant patient. A review of A 45-year-old asymptomatic woman with no signifi- her current mammogram, with magnification views, cant past medical history undergoes annual screen- demonstrates indeterminate microcalcifications in ing mammography and presents to your office with the upper outer quadrant of the right breast. Within these cat- egories, the percentages of cases with a surgical pathologic diagnosis of malignant involvement were as follows: benign calcification (0% malignant); in- determinate calcification (22%); malignant calcifica- tion (92%); smooth mass (1%); irregular mass (40%); architectural distortion (47%); asymmetric breast tissue (3%); smooth mass with calcification (0%); irregular mass with calcification (66%); architectural distortion with calcification (57%); and asymmetric breast tissue with calcification (29%). If this procedure is not available, then a nee- onstrates indeterminate calcifications in the upper dle-localized excisional biopsy should be performed. Magnification view demonstrates amorphous calcifications in a grouped distribution. The pathology demonstrates lobular car- Differential Diagnosis cinoma in situ and fibrocystic changes including Indeterminate microcalcification may be benign, sclerosing adenosis. Calcifications are seen in associ- though the likelihood of malignancy (noninvasive ation with benign fibrocystic changes. It does not form a mass, pro- hyperplasia, noninvasive carcinoma, and invasive car- duce nipple discharge, or routinely produce mammo- cinoma, and there may be a relationship between the graphic findings such as calcifications or architec- amount of calcification and the activity of the epithe- tural distortion. Stated an- breast cancer, albeit a higher rate than the general other way, 87% of patients at 10 years and 74% of pa- population. Observation with mammography and tients at 20 years will remain free of invasive disease history and physical examination is an acceptable during the 15 years subsequent to diagnosis. It is important to note that complete history and physical examination, in addi- this risk is equal for both breasts, regardless of the tion to breast self-examination and awareness. In all situations, however, one must review in more than 13,000 women at increased risk for the reason for recommending the original biopsy: if breast cancer, tamoxifen decreased the incidence of the microscopic pathologic findings in addition to breast cancer by 49%. If there are equivocal find- further, its use reduces the risk of osteoporosis-in- ings on core biopsy, then an open surgical biopsy is duced fractures. A thorough discussion with the patient should ensue so that she may par- Case Continued ticipate in the decision-making process regarding management of her risk. After the options have been explained, the patient As stated previously, patients diagnosed with elects to pursue chemoprevention with tamoxifen. Efficacy of bilateral pro- Suggested Readings phylactic mastectomy in women with a family history of breast cancer. The breast: comprehensive manage- ous categories of mammographically detected, nonpalpable ment of benign and malignant disorders. Because areas of invasion may be ing mammogram and is found to have clustered minute, the accurate diagnosis of in situ cancer ne- microcalcifications in the upper outer quadrant of cessitates the analysis of multiple microscopy sec- the left breast. In 1941, Foote and a mastectomy for “breast cancer” at age 47 and is Stewart published a landmark manuscript, which alive and free of disease. In the late 1960s, Gallagher and Martin pub- tems and physical examination are normal. There is lished a descriptive study of whole breast sections no palpable breast mass or axillary/supraclavicular and described a stepwise progression from benign lymphadenopathy. If they are scattered, the most important deter- women and up to 5% of breast cancers in men. It mination is whether or not there are casting occurs most commonly in the fifth decade of life. If so, malignancy cannot be The term intraductal carcinoma is frequently applied excluded. Granular or casting calcifications are malig- tered microcalcifications are detected on screening nant.
This component of nonelastic resistance is gener- ally underestimated and ofen overlooked purchase 2mg imodium, but may C discount imodium 2mg visa. It Measuring vital capacity as an exhalation that is as seems to be primarily due to viscoelastic (frictional) forceful and rapid as possible (Figure 23–10 ) resistance of tissues to gas fow discount imodium 2mg with mastercard. Excessive amounts of expiratory resistance also activate expiratory mus- Tissue resistance work cles (see above). Airway resistance work Respiratory muscles normally account for only 2% to 3% of O2 consumption but operate at about. Ninety percent of the work is dissi- pated as heat (due to elastic and airfow resistance). In pathological conditions that increase the load on the diaphragm, muscle efciency usually progressively decreases, and contraction may become uncoordi-. The work required to overcome elastic resis- tance increases as Vt increases, whereas the work 0 required to overcome airfow resistance increases as 0 –1 –2 Change in pleural pressure (mm Hg) respiratory rate (and, necessarily, expiratory fow) increases. Work of Breathing on Pulmonary Mechanics Because expiration is normally entirely passive, both the inspiratory and the expiratory work of breathing The efects of anesthesia on breathing are complex is performed by the inspiratory muscles (primarily and relate to changes both in position and anesthetic the diaphragm). Volumes & Compliance Respiratory work can be expressed as the prod- Changes in lung mechanics due to general anes- 6 uct of volume and pressure (Figure 23–11 ). During exhalation, the stored potential collapse and compression atelectasis due to loss of energy is released and overcomes expiratory airway inspiratory muscle tone, change in chest wall rigidity, resistance. The mechanisms tory resistance are compensated by increased inspi- may be more complex; for example, only the depen- ratory muscle efort. When expiratory resistance dent (dorsal) part of the diaphragm in the supine increases, the normal compensatory response is to position moves cephalad. The higher position Awake of the dorsal diaphragm and changes in the thoracic cavity itself decrease lung volumes. Tus, the risk of increased intra- pulmonary shunting under anesthesia is similar to that in the conscious state; it is greatest in the elderly, in obese patients, and in those with underlying pul- monary disease. At end-expiration, the dorsal thesia would be expected to increase airway resis- portion of the diaphragm is more cephalad and the tance. Increases in airway resistance are not usually ventral portion is more caudal than when awake, the observed, however, because of the bronchodilating thoracic spine is more lordotic, and the rib cage moves properties of the volatile inhalation anesthetics. Position of airway Neck extension ↑ Neck ﬂexion ↓ E ﬀ ects on the Work of Breathing Increases in the work of breathing under anesthesia Age ↑ are most ofen secondary to reduced lung and chest Artiﬁcial airway ↓ wall compliance, and, less commonly, increases in airway resistance (see above). The problems of Positive-pressure ventilation ↑ increased work of breathing are usually circum- Drugs—anticholinergic ↑ vented by controlled mechanical ventilation. Pulmonary perfusion E ﬀ ects on the Respiratory Pattern Pulmonary emboli ↑ Hypotension ↑ Regardless of the agent used, light anesthesia ofen results in irregular breathing patterns; breath hold- Pulmonary vascular disease ing is common. Inhalation agents generally pro- duce rapid, shallow breaths, whereas nitrous–opioid techniques result in slow, deep breaths. Dead space Ventilation is usually measured as the sum of all can be afected by a variety of factors (Table 23–3). Tis ratio can be derived by the Bohr equation: Minute ventilation = Respiratory rate × Tidal volume Vd Paco2 − Peco2 For the average adulThat rest, minute ventilation = Vt Paco2 is about 5 L/min. Terefore, even with a normal inspiratory (becomes less negative) per 3-cm decrease in lung time, abnormalities in either compliance or resistance height. Because of a higher Time Constants transpulmonary pressure, alveoli in upper lung areas Lung infation can be described mathematically by are near-maximally infated and relatively noncom- the time constant, τ. In contrast, the smaller alveoli in depen- dent areas have a lower transpulmonary pressure, Regional variations in resistance or compliance are more compliant, and undergo greater expansion not only interfere with alveolar flling but can cause during inspiration. In reality, inspira- lung can be demonstrated in normal individuals tory time is necessarily limited by the respiratory rate breathing spontaneously during abnormally high and the time necessary for expiration; consequently, respiratory rates. Hypercapnia and acidosis have a constrictor efect, whereas hypocapnia causes pulmonary vaso- 2. Pulmonary Perfusion dilation, the opposite of what occurs in the systemic circulation. Of the approximately 5 L/min of blood fowing through the lungs, only about 70–100 mL at any one time are within the pulmonary capillaries undergo- Distribution of Pulmonary ing gas exchange. At the alveolar–capillary mem- Perfusion brane, this small volume forms a 50–100 m -sheet of2 Pulmonary blood fow is also not uniform. Moreover, to Regardless of body position, lower (dependent) areas ensure optimal gas exchange, each capillary perfuses of the lung receive greater blood fow than upper more than one alveolus. Tis pattern is the result of a Although capillary volume remains relatively gravitational gradient of 1 cm H2O/cm lung height. Large increases in lation allow gravity to exert a signifcant infuence either cardiac output or blood volume are tolerated on blood fow. Also, in vivo perfusion scanning in with little change in pressure as a result of passive normal individuals has shown an “onion-like” layer- dilation of open vessels and perhaps some recruit- ing distribution of perfusion, with reduced fow at ment of collapsed pulmonary vessels. Small the periphery of the lung and increased perfusion increases in pulmonary blood volume normally toward the hilum. A shif in posture from not uniform across the lung, the alveolar distending supine to erect decreases pulmonary blood volume pressure is relatively constant. The interplay of these (up to 27%); Trendelenburg positioning has the pressures results in the dividing of the lung into four opposite efect. Changes in systemic capacitance distinct zones (ie, the West Zones) (Figure 23–15 ). In in obstruction of blood fow and creation of alveolar this way, the lung acts as a reservoir for the systemic dead space. In lower areas of the lungs, Pa 7 autonomic system in infuencing pulmonary progressively increases due to lower elevation above vascular tone (above). In zone 2 (Pa > P a > Pv), Pa is higher lus for pulmonary vasoconstriction (the opposite of than Pa, but Pv remains lower than both, resulting its systemic efect). Both pulmonary arterial (mixed in blood fow that is dependent on the diferential venous) and alveolar hypoxia induce vasoconstric- between Pa and Pa. The bulk of the lung is described tion, but the latter is a more powerful stimulus. Tis by zone 3 (Pa > Pv > Pa), where both Pa and Pv are response seems to be due to either the direct efect of higher than Pa, resulting in blood fow independent hypoxia on the pulmonary vasculature or increased of the alveolar pressure. Zone 4, the most dependent production of leukotrienes relative to vasodilatory part of the lung, is where atelectasis and/or intersti- prostaglandins. Inhibition of nitric oxide production tial pulmonary edema occur, resulting in blood fow may also play a role. Hypoxic pulmonary vasocon- that is dependent on the diferential between Pa and striction is an important physiological mechanism pulmonary interstitial pressure. V/Q for individual lung units (each alveolus and increase in alveolar ventilation. An appreciable com- its capillary) can range from 0 (no ventilation) to pensatory increase in O2 uptake cannot take place infnity (no perfusion); the former is referred to as in remaining areas where V/Q is normal, because intrapulmonary shunt, whereas the latter constitutes • pulmonary end-capillary blood is usually already alveolar dead space. Because perfusion increases at a greater rate than ventilation, nonde- • pendent (apical) areas tend to have higher V/Q ratios 3. Absolute shunt refers to ana- with the law for the conservation of mass for O • 2 tomic shunts and lung units where V/Q is zero.
Clinical variation in dys- chondrosteosis: a report on 13 individuals in 8 families order 2 mg imodium with mastercard. Ulnar Dimelia (Mirror Hand) 15 Ulnar dimelia is categorized under duplication and typically lateral and involve only one upper extremity 2mg imodium visa. The condition consists of replication of the ulna best buy imodium, absence of radius, poly- however can be bilateral affecting both hands and both feet. There is great variation of the clinical phenotype but the most This results in a bizarre appearance that is often called “mir- important clinical reality is that the entire upper extremity is ror hand” because the hand, wrist, and forearm on the ulnar involved (. The frst description of this condition with illustrations was All elements are duplicated with the exception of the radial in 1587, but the frst well-documented case was presented by ray, including scaphoid, trapezoid, trapezium, metacarpal, Jackson,  who described a mirror hand with duplicated and thumb phalanges. The patient was a German machinist who be deviated to one side, depending upon the confguration of found the extra digits and wide span useful at work and while existing carpal bones and the length of the two ulnae. The presence of sworth dissected the limb with subsequent description by eight digits is usual along with occasional syndactyly. The specimen is now in the Warren Museum at cases the accessory index ray is often absent, hypoplastic, or the Harvard Medical School (. Most digits have a fexed Experimental embryologic studies with polarizing region posture because the extensor muscles are often absent or hy- grafts of the avian limb buds have shown three planes of poplastic . The second plane is each ulna is rotated and each olecranon fossa faces the other. Wolpert The elbow joint is held in an extended position and its mo- has produced “mirror hands” by grafting polarizing zones tion is often restricted. Upper arm fexor muscles including to different positions along the anteroposterior axis of the biceps and brachialis are poorly developed. He suggested that the condition may result are frequently attached to the distal humerus and do not cross from an additional polarizing region in the anterior margins the elbow joint. The forearm is foreshort- This is the rarest of all congenital upper limb differences ened and the extensor muscles are poorly developed as is the with probably less than 100 cases reported in the literature, wrist, which is held in a fexed and, usually, ulnarly deviated  thus it is impossible to ascertain its incidence. In mirror hand there is limited function which is mirror hand is not inherited but may occur with fbular di- achieved by the ulnar digital components, whereas the radial melia of the lower extremity and absence of the tibia. The three or four digits often obstruct the more functional ulnar Laurin-Sandrow syndrome designation has been given to digits (. Multiple carpal bones are present and vard Medical School) a The medial side of the arm, elbow and fore- articulate from two well-formed capitates. On the dorsal surface two arm and palmar surface of the dissected specimen (ulnar dimelia) are sets of dorsal extensors have run through two separate retinacular sys- demonstrated. Two ulnar arteries (red) and a number of fexor tendons tems, which have been removed. Large muscle bellies in the forearm and views of the elbow demonstrate a proximal synostosis (fusion) of the arm have not been preserved. The medial of these two forearm bones has a more normal b Palmar (left) and dorsal (right) views of the hand show four well- appearing proximal ulna, trochlea, and trochlear notch aligned digital rays with an additional three rays, which become more 15 Ulnar Dimelia (Mirror Hand) 199 Fig. Typically, abnormalities exisThat all tion due to the overwhelming palmar (volar) fexor forces and the ab- levels of the upper limb. The glenohumeral joint is intact but hypoplas- sence of strong radial wrist extensors. These deformities and possible tic with no shoulder abduction and little foreword fexion or extension. The wrist and hand are held in tight fexion and ulnar devia- 200 15 Ulnar Dimelia (Mirror Hand) Fig. No fexor mechanism was present and a pectoralis muscle ulnae articulating with the distal humerus. In this patient the synchon- transfer was used to restore fexion drosis between the two bones has been excised. The ulnar four digits are to the left and the three radial digits with their more fexed posture on the right. Hypoplastic fexor tendons, lumbrical and interosseous muscles, and neurovascular structures are demonstrated. All fexors funneled into one carpal canal 15 Ulnar Dimelia (Mirror Hand) 201 References Associated Syndromes 1. Positional signalling along the anteroposte- rior axis of the chick wing: the effects of multiple polarizing region grafts. Bilateral absence of the radius and tibia with bilateral reduplication of the ulna and fbula: a case report. Hereditary ulnar and fbular Presentation Very rare syndrome with approximately ten dimelia with peculiar facies. Johns Hop- and mental retardation in a surviving child was reported in kins University. The hand and feet contain either seven or eight metacarpals and metatarsals respectively and there is an absence of the radius and tibia in the upper and lower extremities. The hands and feet are cupped with digits and toes giving a rosebud ap- pearance (. These limbs are described in the genetic and pediatric literature as both polydactyly and syndactyly. Lower extremity There is fbular and foot duplication giving the appearance of mirror feet (. Craniofacial Major abnormalities related to the midline of the nose vary from containing a broad nasal tip to a V-shaped cleft and associated hypertelorism. Congenital carpal fusion is due the carpal bones becomes more evident radiographically to failure of segmentation during embryonic limb develop- (. However, Isolated carpal fusions especially of the distal carpal row some would argue that carpal coalition might be a refection are usually asymptomatic, do not adversely affect the wrist of a failure of differentiation because early in embryonic life range of motion, and often discovered accidentally. Synos- cartilaginous anlages of many more than eight carpal bones tosis across the midcarpal joint results in some limitation of are present. The incidence in Caucasian populations has these appear predictably with growth and, in fact, radiologic been described as 0–2% compared to a much higher preva- atlases of wrist ossifcation are used to determine the chron- lence in African populations described as 9. When the coalition is identifed as an isolated fnd- triquetral was classifed by DeVilliers et al. When the synostosis involves and triquetrum that appears radiographically as a pseudo- the distal carpal row, the fnding may be an isolated one but arthrosis; in type 2 there is a notch of varying depth at the there is a much greater chance that there is a syndromic as- site of fusion between the two carpal bones; in type 3 there sociation. When both carpal rows are involved there is close is complete synostosis of the two bones; whereas in type 4 to a 90 % chance of a syndrome, particularly if the condition is bilateral and if there are multiple coalitions . The Apert syndrome commonly shows synostoses at the carpal, meta- carpal, metatarsal, and phalangeal levels (. Carpal fusions crossing the proximal and distal carpal rows as between the trapezium and scaphoid are rare in hands with developed thumb and four digits. These, however, are com- monly seen in hypoplastic hands or limbs with limited num- bers of digital rays (. In one recent publication a case was reported of synostosis between the trapezoid and trapezium, fusion of the radius and scaphoid, and hypoplasia of the thumb . Very little mo- between lunate and triquetrum in the proximal carpal row fol- tion has been lost by the fusion of these two carpals.
K. Sobota. University of California, Hastings College of Law. 2019.