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Echogenic material in the fetal gallbladder and fetal disease.

The presence of echogenic material within the gallbladder is probably a rare finding in the fetus, and the list of predisposing factors known for postnatal life seems not to be applicable to prenatal diagnosis. In the present study 1656 obstetric scans were performed on referrals to the Unit of Fetal Medicine. No echogenic gallbladder contents were found before 28 weeks. In the subgroup of 523 fetuses who were examined during gestational weeks 28-42, six fetuses were found to have echogenic material in the gallbladder. The echogenicities were found in patients who had the following: extra-amniotic hematoma with intrauterine growth retardation and oligohydramnios, tetralogy of Fallot, trisomy 21 with atrioventricular septal defect and transient ascites, early abnormally distended fetal gallbladder, chromosomal aberration (translocation 10; 11) with bilateral clubfoot, and gastroschisis. Echogenic densities had disappeared at ultrasound scans performed during early postnatal life in four cases, were absent at five weeks in one case, and still present as calculi 8 months after birth in one case. Although no causative conclusions can be drawn from the report, these conditions are suggested as possible predisposing factors for the presence of echogenic material in the fetal gallbladder.

Adult↗

Progesterone and 5 alpha-pregnane-3,20-dione in human amniotic fluid.

Progesterone and 5 alpha-pregnane-3,20-dione (5 alpha-DHP) were determined by radioimmunoassay in 242 amniotic fluid samples from 16-19 weeks of gestation. 165 samples fulfilled the criteria of the normal collective. There is a positive correlation (r = 0.359, p less than 0.001) between progesterone and 5 alpha-DHP. The mean concentration (+/- SD) of progesterone for normal pregnancies was 68.04 +/- 35.56 ng/ml, the mean for 5 alpha-DHP was 6.6 +/- 4.76 ng/ml. A slight decrease of the hormone concentrations with increase of the week of gestation was observed. 7 cases, who later developed EPH-gestosis showed a significant higher progesterone concentration (p less than 0.05). 16 women with premature labor had a significant higher 5 alpha-DHP concentration (p less than 0.05). Significantly elevated progesterone and 5 alpha-DHP values were found in 36 cases of bleedings in early pregnancy. Pregnant women older than 35 years proved to have a significant higher 5 alpha-DHP concentration (p less than 0.05). Also women, who delivered a child weighing less than 2,500 g (n = 8), showed a significant higher progesterone concentration (p less than 0.01). There was no difference in amniotic fluid progesterone and 5 alpha-DHP concentration depending on the sex of the child. The hormone concentrations of 3 cases with Morbus Langdon-Down were slightly below the mean concentration for progesterone and 5 alpha-DHP. Progesterone and 5 alpha-DHP concentrations were found to be normal in one case each of open Ductus Botalli, esophagial atresia, conjunctival bleeding with eyelid edema, teleangiectasia, Morbus Gaucher, sicklefoot, omphalocele, clubfoot, and stillbirth respectively.(ABSTRACT TRUNCATED AT 250 WORDS)

5-alpha-Dihydroprogesterone↗

[Tetra-X syndrome with epilepsy, mental retardation and multiple dysmorphias].

It is reported on a 21-year-old female with super-female syndrome in association with an early childhood brain damage exhibiting idiopathic epilepsy, severe oligophrenia, emotional instability, Little syndrome, areflexia of the legs, strabismus, telecanthus, broad nose, epicanthic fold, clinodactyly of little fingers, radio-ulnar-synostosis, retardation of ossification, aortic insufficiency, erythro-cyanosis, one-side clubfoot and nephrosclerosis, also menstrual cycle dysfunction.

Abnormalities, Multiple↗

[Aplasia of the tibia - a case report with observation for 38 years (author's transl)].

The paper reports the result of operative treatment in a case of total congenital absence of the tibia. The boy was treated conservatively by plaster casts and apparative supports until he was six years old. Then the upper end of the fibula was set into the intercondylic fossa of the femur and two months later a talofibular fusion correcting the clubfoot deformity was performed. 32 years after this procedure the leg is now 28 cm shorter. The knee joint is absolutely stable in extended position and active flexion is possible to 45 degrees. The fibula shows a remarkable hypertrophy and has changed its form into a tibia-like bone. The patient is able to walk 15 km without any pain using a supporting apparatus. In congenital absence of the tibia or fibula we prefer orthopedic - surgical devices which preserve the dysplastic part of the limb instead of primary amputation.

Braces↗

Fetal myelomeningocele repair: short-term clinical outcomes.

OBJECTIVE: The study was undertaken to evaluate short-term clinical outcomes with antenatal myelomeningocele (MMC) repair. STUDY DESIGN: Retrospective review of 50 fetuses that underwent open fetal MMC closure was performed. Inclusion criteria included less than 26 weeks' gestation, thoracic to S1 level defect, absent clubfoot deformity, Arnold-Chiari malformation, ventriculomegaly less than 17 mm, normal karyotype, and no other anomalies. RESULTS: Perinatal survival was 94% (47/50 fetuses). Mean age at delivery was 34 weeks 3 days. All fetuses demonstrated reversal of hindbrain herniation. Forty-three percent of the 50 fetuses have required ventriculoperitoneal shunting compared with 100% thoracic, 88% lumbar, and 68% sacral (85% overall) in 297 historic controls. Better-than-predicted leg function was seen in 57% of thoracic and lumbar level lesion patients. CONCLUSION: Early experience with fetal MMC repair suggests a decreased need for ventriculoperitoneal shunting, arrest or slowing of progressive ventriculomegaly, and consistent resolution of hindbrain herniation. However, further long-term follow-up is needed to evaluate neurodevelopment and bladder and bowel function.

Adult↗

Embryotoxic and teratogenic effects of indium chloride in rats and rabbits.

Daily indium chloride doses of control (0), 50, 100, 200, or 400 mg/kg were administered orally to Sprague-Dawley rats by gavage, on d 6-15 of gestation, and daily metal doses of control (0), 50, 100, or 200 mg/kg were administered to New Zealand rabbits on d 6-20 of gestation. Further groups of pregnant rats were treated with control (0) or 400 mg/kg indium chloride orally on one of d 8, 9, 10, 11, 12, 13, 14, or 15 of gestation. The dams and fetuses were examined on d 21 (rats) and 30 (rabbits) of gestation, using standard teratological methods. Indium concentration was determined in the maternal and fetal blood, as well as in the amniotic fluid, by atomic absorption spectrometry. Indium was found to cross the placenta and appeared in fetal blood in proportion to the metal concentration of the maternal blood. In the amniotic fluid, indium concentrations remained below the detection limit. In rats, indium chloride produced dose-dependent maternal toxic effects, with a dose of 400 mg/kg inducing embryotoxicity (embryolethality) and teratogenicity. Doses of 200 and 100 mg/kg were embryotoxic (retarding) and teratogenic, causing skeletal and visceral anomalies in addition to external anomalies (rudimentary or missing tail, syndactylia, clubfoot, exencephalia) in rats. In rabbits, 200 mg/kg indium chloride was lethal for the dams and the embryos (some of the animals died, and the number of abortions and full resorptions increased). This dose was found to be teratogenic (caused gross renal anomalies) and increased the frequency of fetuses with skeletal retardation. In rats, the effects of indium chloride causing fetal retardation was found to be independent of exposure time. The teratogenic effects were the highest on d 11 and 12 of gestation, when indium chloride caused gross external malformations. Data suggest that the teratogenic effects of indium chloride can be attributed primarily to a direct cytotoxic action of indium resulting from placental transfer, but the effect is not a selective one, as it appears only in the presence of maternal toxic effects.

Abnormalities, Drug-Induced↗

Thomas Dent Mutter, MD: early reparative surgeon.

Thomas Dent Mutter (1811-1859) was one of the early American pioneers of plastic surgery. He studied under some of the great teachers in Europe during the early 19th century and returned to the United States as a professor of surgery at Thomas Jefferson University in Philadelphia. He was renowned for his operations on clubfoot, cleft lip and palate, congenital anomalies, and mutilating injuries. He is best known for the cervical flap that bears his name. His contributions include an endowment for a medical museum that bears his name to which he donated a large collection of his medical specimens. The Mutter Museum prospers to this day. His ideas and innovations have influenced many students of plastic surgery both during his lifetime and today.

History, 19th Century↗

Biomechanical study of tibialis anterior tendon transfer.

The tibialis anterior tendon transfer has been used in the treatment of recurrent congenital clubfoot and paralytic equinovarus foot deformities in cerebral palsy. This study attempts to determine the optimal site of tibialis anterior tendon insertion for ankle and foot motion and to compare the split and whole tendon transfer. Ten fresh normal anatomic leg specimens were used. The lateral half of the tibialis anterior tendon was detached from its insertion, passed beneath the extensor retinaculum, and anchored to the appropriate tarsal bone by a barbed staple. Tension was applied, and ankle and foot motions were measured. The experiment was done by anchoring the tendon to the tarsal bones along the axis of the second metatarsal and serially through to the axis of the fifth' metatarsal. The entire experiment was repeated using the whole tibialis anterior tendon. For split tendon transfer, insertion onto the fourth metatarsal axis was the most effective route; it produced maximal dorsiflexion with minimal supination and pronation. For whole tendon transfer, the ideal site of insertion was along the third metatarsal axis. However, the difference between the average maximum dorsiflexion achieved by the split tendon transfer and that of total tendon transfer is not statistically significant.

Ankle↗

Complications of midfoot and hindfoot arthrodesis.

Midfoot and hindfoot arthrodeses traditionally have been done to treat deformities resulting from paralytic disorders, residual clubfoot deformity, and posttraumatic arthritis. The surgical indications for midfoot and hindfoot arthrodeses more recently have been expanded to include painful arthritic deformities associated with neuroarthropathy, seropositive or seronegative arthropathies, and neurologic disorders. Regardless of the joint fused or the technique used, the goal of each remains similar: the creation of a painless, plantigrade foot capable of being fitted into, at the very least, a custom shoe. The aim of the current study is to describe the major complications associated with midfoot and hindfoot fusions in adults, and the prevention and the treatment of these complications.

Arthrodesis↗

Consistent arterial abnormalities associated with a variety of congenital malformations of the human lower limb.

A number of seemingly unrelated congenital deformities of the lower limb have been presented which include clubfoot, fibular deficiency, tibial aplasia, and diplopodia. Although the bony morphology in these limbs is quite different, they all share a strikingly similar arterial pattern, that being deficiency or absence of the anterior tibial artery, and of its derivative, the dorsal pedis artery. Since all of these diverse conditions share a similar aberrant arterial pattern, we suspect that the arterial changes are important in the pathogenesis of those conditions. Study of the soft tissue anatomy of these specimens suggests that the etiologic teratogenic event occurred early in embryonic development. In those limbs that contain the remnant of a missing structure, it is concluded that injury occurred after the mesenchyme was instructed to form that structure. These are termed "post-specification" defects. In those circumstances where limb duplication occurs, the injury affected the signal before instruction of mesenchyme to develop into a specific structure was completed and these abnormalities are termed "pre-specification" malformations. The musculotendenous and neurologic abnormalities seem to be reactive to the pre-existing bony pattern.

Angiography↗

Congenital dislocation of the patella. Part II: orthopaedic management.

Five patients (eight knees) with diagnosed congenital dislocation of the patella and well-documented charts were reviewed. Age at diagnosis ranged from 4 days to 6 years. A flexion contracture of the knee and femorotibial rotatory dislocation of varying degrees were present in all the cases. The quadriceps was active in all the cases, producing knee flexion in four cases. Foot deformity was associated in all the cases (clubfoot, calcaneovalgus, or congenital vertical talus). Gradual correction of knee flexion contracture with serial casting was attempted in five cases leading to an almost complete extension in two cases. Treatment of patellar dislocation was surgical in all the cases, consisting in extensive quadriceps release (seven knees) or V-Y lengthening (one knee), division of lateral soft tissues, and reefing of the medial retinaculum and capsule. Intraoperative anomalies were recorded. At an average follow-up of 6.9 years, all the patients are able to walk on their operated limb, and the patella is centered in the trochlea in all the cases. Knee mobility, rotatory dislocation, and daily function were improved in seven cases.

Child↗

Dynamic plantar pressure measurement for the normal subject: Free-mapping model for the analysis of pediatric foot deformities.

In measuring plantar pressures during gait, prior methods have divided the foot into five regions and neglected forefoot alignment as it is involved in intoeing and outtoeing. The authors' proposed free-mapping method divides the foot into nine regions and incorporates a pedobarograph foot progression angle. The purpose of the study was to provide normal pressure parameter data during stance phase using a free-mapping model. Sixty-six normal children, ages 6 to 16 years, were recruited and walked along the 5-m walkway at self-selected speeds. The mean and standard deviation for the plantar contact area, contact time, peak pressure, maximal mean pressure, pressure-time integral, force-time integral, instant of peak pressure, and instant of maximum force in nine foot regions are reported. These normative data will provide a basis with which assessment of foot deformities involved in clubfoot, pes planus, and cavus foot will be more accurately defined.

Foot↗

The newborn foot: diagnosis and management of common conditions.

An assessment of the foot should be a part of every newborn physical examination. The newborn foot, although complex in structure, can be examined quickly in any office setting. Many foot deformities are diagnosed immediately after birth, allowing for earlier and often more successful treatment. A neonate with a foot deformity can be a source of anxiety to parents. Adequate knowledge of commonly encountered neonatal foot problems enables the nurse to give appropriate anticipatory guidance to the parents. Foot abnormalities usually occur as isolated findings in an otherwise healthy newborn. However, these deformities can also be seen in newborns with underlying neuromuscular disorders and syndromic conditions. Common newborn foot abnormalities include equinovarus deformity (clubfoot), metatarsus adductus, calcaneovalgus, congenital vertical talus, polydactyly (supernumerary digits), and syndactyly (webbed toes). These common foot disorders, their treatment, and their prognosis are discussed. This article also discusses fundamental nursing implications needed to care for these young patients and their families.

Algorithms↗

Tarsal coalition.

Tarsal coalition is a common abnormality of the hindfoot skeleton that only rarely leads to symptoms. These symptoms occur most commonly in adolescence but rarely can be found also in adults. Although most coalitions are congenital, as the consequence of autosomal dominant inheritance, coalitions also can be acquired by degenerative joint disease, inflammatory arthritis, infection, and clubfoot deformities. Fifty percent of all coalitions are bilateral. Talocalcaneal and calcaneonavicular coalitions are most commonly found, and patients frequently have more than one coalition in the same foot. Clinical symptoms of the tarsal coalition frequently follow a sequence of sprains or other minor injuries to the involved foot. This leads to a rigid, painful foot. The pain is worsened by continued activities. The frequently cited peroneal spastic flatfoot is an uncommon means of identifying a tarsal coalition. The diagnosis of the tarsal coalition is made on the oblique radiograph of the foot, which demonstrates the calcaneonavicular coalition. Computed tomography (CT) and magnetic resonance imaging scans show the presence and extent of other coalitions. Secondary signs for the presence of a coalition are talar beaking, anteater nose sign, and C sign. These secondary signs can be demonstrated best on a lateral view of the involved foot. Local anesthetic blocks under image intensifier or CT guidance can identify areas of joint degeneration, which are caused by the altered biomechanics of the foot. Initial treatment should consist of conservative therapy in the form of support or immobilization of the involved foot, change in the activities of the patient, and nonsteroidal anti-inflammatory medication. Surgical treatment in the form of a resection of the coalition should be reserved for those patients for whom conservative therapy has failed. Subtalar or triple arthrodesis should be reserved for those patients for whom all other therapy has failed.

Adolescent↗

The Grice extraarticular subtalar arthrodesis: a clinical review.

One hundred forty-nine Grice procedures performed between 1955 and 1982 were reviewed. The valgus deformities were secondary to poliomyelitis, cerebral palsy, myelodysplasia, flexible flatfoot, clubfoot, congenital vertical talus, and other neuromuscular diseases. Review consisted of radiographic and clinical follow-up. Overall evaluation revealed 46% unsatisfactory results because of both graft failure and residual clinical deformity. Previous unrecognized ankle valgus and instability prejudiced the results in the myelodysplasia and flexible flatfoot. Recommendations include consideration of the Grice procedure in poliomyelitis and cerebral palsy coupled with appropriate tendon balancing, but not in myelodysplasia or flexible flatfoot in the presence of often unrecognized ankle valgus.

Arthrodesis↗

Fever following orthopedic operations in children.

One hundred fifty-three orthopedic operations in 129 children were analyzed for the significance of postoperative fever (POF) as a predictive factor for possible complications. In 72% of the operations, a temperature of greater than 37 degrees C was recorded. In 63 operations (41%), the temperature was greater than 38 degrees C. Sixteen children had positive clinical signs that might explain the fever, and all of them had a temperature of greater than 38 degrees C. Duration of operation of greater than 1 h, clubfoot releases, open reduction of fractures, and spine fusion operations gave higher incidences of POF. POF indicates a complication only when associated with positive physical findings. A postoperative temperature of greater than 38 degrees C, therefore, mandates repeated physical examination, which is the most reliable method of discovering the presence of complications.

Adolescent↗

Congenital dislocation of the knee.

Twenty-three congenital knee dislocations in 17 patients were reviewed after an average follow-up of 11 years. There was a 30% incidence of breech delivery along with a 41% incidence of clubfoot. Treatment in serial casts was successful in 10 knees; 13 required operations. Prognosis was most favorable in unilateral cases and when surgery was performed before 2 years of age. Quadriceps lengthening during knee surgery facilitated the subsequent reduction of ipsilateral dislocated hip.

Adolescent↗

The Heyman-Herndon tarsometatarsal capsulotomy for metatarsus adductus: results in 48 feet.

Forty-eight Heyman-Herndon tarsometatarsal capsulotomies were performed over an 18-year period for idiopathic metatarsus adductus and residual adductus of clubfoot. There was a 41% overall failure rate and a 50% incidence of painful dorsal prominence at the surgical scar. Sixty-eight percent of the patients had difficulty with proper shoe fitting. Those with associated orthopedic anomalies were at high risk for a poor result. We believe that tarsometatarsal capsulotomy is of questionable benefit in treatment of forefoot adduction deformities of children.

Child↗