An Alternative Surgical Approach to Pseudoainhum: A Case Report.

Pseudoainhum is a disorder of unknown etiology that is usually congenital and nonprogressive. One theory states it is caused by amnion band constriction in utero. These constriction bands lead, not only to a less cosmetically appealing foot, but also to long-term neurovascular compromise, resulting in amputation of the digit.

[Scimitar syndrome. Correlation anatomo-embryological].

Objectives: To describe morphologically a toracoabdominal visceral block of a scimitar's syndrome case. We propose a pathogenetic theory wich explains the development of the pulmonary venous connection in this syndrome.

Method: The anatomic specimen was described with the segmental sequential system.

Two-stage release in Streeter's dysplasia.

Streeter's dysplasia is a rare disorder of congenital constricting bands, occurring in 1-10 000 birth, with no sex predilection. In severe cases, it is associated with autoamputation of distal extremities such as fingers and toes. Recent advances have made it possible, in developed countries, to recognise this condition in the prenatal period and to initiate prompt intrauterine management, however, these, being uncommon procedures, need technical advances and experienced hands.

ADAM "sequence" part II: hypothesis and speculation.

Noted for centuries in humans, a relatively hairless mammal [e.g., Hallero, 1766; Hohl, 1828 in Klunker, 2003], the so-called amniotic deformities, adhesions, mutilations (ADAM) sequence remains causally and pathogenetically incognito.

[Complex of amniotic deformities, adhesions, mutilations: Endless debate].

Amniotic deformities, adhesions, mutilations (ADAM) complex is a broad heterogeneous spectrum of congenital anomalies. ADAM complex is characterized by constriction rings, amputation of fingers or limbs and the presence of the amniotic band. However, it may also involve craniofacial disruptions, body wall defects and internal organ abnormalities.

Amniotic band syndrome.

Amniotic band syndrome is an uncommon congenital disorder without any genetic or hereditary disposition. It involves fetal entrapment in strands of amniotic tissue and causes an array of deletions and deformations. Primary treatment is plastic and reconstructive surgery after birth with in utero fetal surgery also coming in vogue.

Ten-year experience with one-step correction of constriction rings by complete circular resection and linear circumferential skin closure.

Background: In amniotic band syndrome (ABS) constriction rings affecting the limbs are regularly seen at birth. Circular resection and closure with multiple Z-plasties has been described as the treatment of choice. However, direct circular closure creating linear circumferential scars might replace multiple Z- or W-plasties to reduce scars and improve aesthetic outcome.

Human pancreatic cancer fusion 2 (HPC2) 1-B3: a novel monoclonal antibody to screen for pancreatic ductal dysplasia.

BACKGROUND.: Pancreatic ductal adenocarcinoma is rarely detected early enough for patients to be cured. The objective of the authors was to develop a monoclonal antibody to distinguish adenocarcinoma and precancerous intraductal papillary mucinous neoplasia (IPMN) from benign epithelium.

Management of ACL elongation in the surgical treatment of congenital knee dislocation.

Congenital knee dislocation is a rare anomaly associated with a variety of neuromuscular diseases and deformities. The etiology of this condition remains unclear, but it is usually associated with a variety of disorders, such as Larsen's syndrome, arthrogryposis, spondyloepiphyseal dysplasia, Ehlers-Danlos syndrome, Down syndrome, and Streeter's dysplasia. It is rarely an isolated entity, and 60% of patients with congenital knee dislocation had additional congenital anomalies, most commonly hip dysplasia.

Septo-optic dysplasia with digital anomalies--a recurrent pattern syndrome.

A distinct form of septo-optic dysplasia (SOD) comprises limb malformations in addition to the characteristic CNS and ocular abnormalities. To date, there have been 4 reports, citing 5 affected patients with this combined phenotype. We now add a further case and present neuroradiological images of the CNS findings in this condition.

Case of complex craniofacial anomalies, bilateral nasal proboscides, palatal pituitary, upper limbs reduction, and amnion rupture sequence: disorganization phenotype?

We report a case of a dizygotic twin with complex abnormalities of head, body, and limbs. The anomalies include the following: lateral and midline cleft upper lip, ectopic palatal pituitary, natal teeth, bilateral nasal proboscides with an absent nose, left microphthalmia with conjunctival-lined cyst, right ocular dysgenesis, bilateral retinal dysplasia, platybasia with skull asymmetry, hydrocephalus secondary to aqueductal atresia, brain hemispheric asymmetry with a parietal-occipital cortical flap, agenesis of posterior corpus callosum, absence of the olfactory nerves and left anterior cerebral artery, leptomeningeal and intraventricular heterotopias, right radial longitudinal terminal meromelia with constriction rings of fingers, partial syndactyly of the third and fourth left fingers, dorsiflexed great toes and pes equinovarus bilaterally, and multiple skin tags with a sacral appendage. Additionally, this twin's placental disc and extraplacental membranes were devoid of amnion.

Fetal foot length--a new parameter for assessment of gestational age.

Ultrasonographic measurement of fetal foot length, a new parameter, was correlated with the gestation age. One hundred and five ultrasonographic measurement of fetal foot length was performed between 13 and 42 weeks gestation. Comparison of linear regression of foot length versus gestational age demonstrated a strong correlation with an r2 value of 0.

Fetal foot length as a predictor of gestational age.

Ultrasonographic measurement of fetal foot length is useful in the assessment of gestational age. Two hundred twenty-three postpartum and 224 ultrasonographic measurements were performed between 11 and 43 weeks' gestation; 207 postpartum and 160 ultrasonographic measurements met our requirements of secure dates and no physical anomaly or maternal disease. Mean foot length at each week of gestation compared favorably with Streeter's data, based on pathologic specimens, described in 1920.