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Pool R D - - 1986
The term "congenital scoliosis" contributes little to our understanding of aetiology, for "congenital" simply means "to be born with" and is applicable to deformities present at birth whether these are genetically determined or acquired in utero. The presentation of monozygotic twins, one of whom has congenital scoliosis (vertebral anomalies) while ...
Lawhon S M - - 1986
The cases of twenty-eight patients with the VATER association, which consists of various combinations of vertebral anomalies, anal atresia, tracheoesophageal fistula with esophageal atresia, radial dysplasia, and renal anomalies, were reviewed. Associated anomalies included deformities of the ribs and lower limbs, a two-artery umbilical cord, a congenital heart defect, and ...
Aiba K - - 1986
A three-year-old boy who had ataxic diplegia, mental retardation, horizontal pendular nystagmus with head nodding and abnormal auditory brain stem responses showing only waves I and II was presented. His clinical features coincided with recent reports in the Japanese literature of cases of a new syndrome that is congenital in ...
Herman J H - - 1986
Central nervous system hemorrhage is a well-recognized complication of neonatal alloimmune thrombocytopenia attributed to perinatal trauma from passage through the birth canal. That central nervous system (CNS) hemorrhage can occur in utero is not as well recognized, and congenital CNS lesions have only circumstantially been linked to thrombocytopenia. We report ...
Marions O - - 1986
We report two cases of hydrocephalus in adults. The radiological investigations and direct inspection during surgery in one of the cases indicate that the hydrocephalus is caused in both cases by a benign stricture in the region of the foramen of Monro and that this constriction is congenital. This origin ...
Nakayama D K - - 1986
Urethral obstruction due to posterior urethral valves (PUV) is a more lethal condition in the newborn than is commonly recognized. To study its prognosis when the diagnosis of PUV is made shortly after birth, cases of PUV presenting in the first week of life were reviewed. Eleven cases were seen ...
Nishimura M - - 1986
Cranial computed tomography (CT) of 108 cases with dilated lateral ventricles was reviewed to elucidate the relationship between focal vulnerability of developing brain and disproportional dilatation of lateral ventricles. CT findings of 108 cases with symmetrical dilatation of lateral ventricles were classified into three types by morphometry of lateral ventricles: ...
Segal D - - 1986
Unilateral arch hypertrophy secondary to congenital absence of lumbar articular facets is a rare condition of still undetermined etiology. Most symptomatic cases had a history of mild trauma. Radiologic criteria using plain films and CAT scan are available to make the diagnosis of unilateral arch hypertrophy and the specific defects ...
Drut R - - 1986
Using immunoperoxidase PAP technique in 2 cases of congenital fibrosarcoma, a great number of cells showed positive stain for alpha-1-antitrypsin (A1AT) and alpha-1-antichymotrypsin (A1ACT), both considered to be good histiocytic markers. The ultrastructure in 1 case also provides evidence of histiocytic differentiation. These findings suggest that congenital fibrosarcomas are actually ...
Hennekam R C - - 1986
We describe a median "cleft" face anomaly (MCFA) with congenital hypothalamic hamartoma in a newborn girl. The MCFA was associated with a frontal midline skull lipoma and a complex congenital heart defect. Possible pathogenetic mechanisms are discussed, and a review of the pertinent literature is given. It is concluded that ...
Reynolds M A - - 1985
A case of salt-losing congenital adrenal hyperplasia with severe hyponatremic dehydration is presented. Clinical signs and symptoms of cerebral edema with elevated intracranial pressure were present. Conventional treatment was started, and after initial concern regarding future head growth and development, there was a good outcome with normal development at 1 ...
Gouw A S - - 1985
Splenogonadal fusion is a rare congenital malformation in which there is an abnormal fusion between the spleen and the gonad or derivatives of the mesonephros. It can occur in both sexes but has been reported far more frequently in males. There are two types of this malformation: the continuous and ...
Denneny J C JC - - 1985
Congenital bronchomalacia is a disease typically associated with infants between 1 and 12 months of age; cases in children less than 1 month of age are rare. Bronchomalacia is commonly associated with tracheomalacia and rarely warrants operative intervention. Three cases of bronchomalacia are presented, including two unusual cases of bronchomalacia ...
Hughes J L - - 1985
The case of a 4 1/2-year-old boy with congenital vertical ocular motor apraxia who was otherwise developmentally and neurologically normal is reported. The presence of perinatal hypoxia in this patient may have been etiologic. While the presence of a supranuclear vertical ocular motor abnormality usually suggests a serious, acquired neurologic ...
Azzopardi N - - 1985
Out of 21,090 live births registered in the Maltese Archipelago between 1st January 1980 and 31st December, 1983 there were 31 cases of congenital abnormalities that needed emergency surgery in the first few hours of life. The final results obtained locally compare favourably to those registered in larger centres abroad.
Wertelecki W - - 1985
Abnormal centromere-chromatid apposition (ACCA) was noted in a patient with Peters' anomaly. Previous reports of ACCA emphasized its association with tetraphocomelia and other congenital malformations (Roberts, SC Phocomelia, Pseudothalidomide Syndromes). This report expands the array of congenital malformations associated with ACCA and emphasizes the diagnostic importance of ocular defects for ...
Bittencourt A L - - 1985
The isoenzyme profiles of a group of five Trypanosoma cruzi stocks obtained from congenital cases and of a second group of seven stocks obtained from chagasic mothers who did not transmit their infection congenitally were studied by electrophoresis of nine enzymes. All the mothers became infected in Bahia State (Brazil). ...
Pollock R A - - 1985
Congenital hyperplasia is a developmental disorder characterized by marked unilateral asymmetry. The structural disproportions from one side to the other far exceed variations commonly seen in the normal population. Classically, as first described in 1836, the overgrowth of tissue is restricted to one-half of the body, including the extremities. In ...
Bosniak S - - 1985
A severe corneal ulceration that is evident shortly after birth and persists in spite of adequate medical therapy, may be secondary to a congenital entropion. A vertically kinked tarsus is an unusual and frequently overlooked form of congenital entropion. Prompt recognition of this entity is essential to avoid severe corneal ...
Bernard T N TN - - 1985
Forty-two cases of congenital scoliosis, four cases of congenital kyphosis and one case of congenital lordosis were reviewed retrospectively from 1975 through 1982, with an average follow-up of 5.09 years. Associated anomalies occurred in 62.5% of the cases, with Sprengel's deformity and Klippel-Feil syndrome being the most common musculoskeletal anomalies. ...
Sundaram M B - - 1985
Pupils in congenital neurosyphilis differ from the classic Argyll-Robertson pupil in acquired cases, and often tend to be large and unreactive. Constriction to pilocarpine in the reported patient would suggest that peripheral parasympathetic damage is responsible for such pupillary findings. Congenital neurosyphilis should be included among the causes of "tonic" ...
Marchac D - - 1985
The abdominal wall of the infant presents a significant skin excess. Applying techniques of aesthetic abdominoplasty, it is possible to excise extensive abdominal wall lesions, as often seen in children with congenital hairy nevi. Three cases of congenital nevi were handled in this manner and are presented. One case involved ...
Tsukahara S - - 1985
The commonest cause of blindness among the 67 patients at the Yamanashi School for the Blind was congenital cataract (16). Next was retinitis pigmentosa or choroido-retinal degeneration (8), then retinopathy of prematurity (7). Congenital glaucoma and brain tumor each contributed five. Four were due to microphthalmia/micro-cornea and five to high ...
Youssif M - - 1985
Two cases of posterior urethral polyps are reported. The literature was reviewed in regard to its frequency, origin, pathology, clinical presentations and treatment. It is considered that it is a non-neoplastic congenital defect. Because of its obstructive qualities, it is of clinical significance and should be considered as a separate ...
Weider D J - - 1984
This article details case histories of a son and his mother who were studied over a seven-year period before making the diagnosis of bilateral oval window microfistulae in both of them. The boy demonstrated a slowly progressive bilateral and symmetrical sensorineural hearing loss closely resembling that of his mother. The ...
McDonald T J - - 1984
Congenital cholesteatoma of the ear is a capricious and challenging problem for the otologist. We present 21 cases managed at the Mayo Clinic from 1961 to 1983. In all but one unusual case, the cholesteatoma developed behind an intact tympanic membrane and there was no history of aural infections. Preoperative ...
Wang R - - 1984
Congenital middle ear cholesteatomas remain of interest because of their relative rarity and unknown origin. A 4-month-old child presenting with bilateral congenital middle ear cholesteatomas forms the basis for this report. The nature of the epithelial debris found suggests an external origin. An extended transcanal tympanotomy approach for removal, based ...
Verdi G D - - 1984
This report describes a newborn boy with cleft palate and congenital trismus preventing normal feeding. After surgical division of the fibrous bands between the maxillary and mandibular alveolar ridge, oral feedings progressed without difficulty. Historical reports of congenital oral bands are reviewed.
Antebi E - - 1984
A case of a venous malformation of the abdominal wall in a 13-year-old boy is hereby presented. This malformation, manifesting as varicosities which were present from infancy caused no disturbances other than cosmetic. Clinical, laboratory and radiological examinations revealed no underlying pathology linked with this malformation. Considering the patient's history, ...
Schweitzer V G - - 1984
Otosclerosis classically describes an osteodystrophic change in the bony labyrinth and stapes footplate, of autosomal dominant inheritance, reported rare under the age of 5, extremely "rare" in the Oriental and Black race, "non-existent" in the American Indian, and with a clinical incidence of 5 per 1000 Caucasians. The differential diagnosis ...
Belmont J R - - 1984
Congenital laryngeal stridor (CLS) and laryngomalacia are terms used to describe a common disorder characterized by onset, at or soon after birth, of a harsh respiratory sound mostly audible during inspiration. Typically, laryngoscopy reveals flaccid supraglottic structures which tend to prolapse medially toward the glottis during inspiration. Despite reports of ...
Parks M M - - 1984
Surgical alignment of the eyes in congenital esotropia within the first two years of age can result in the development of binocular vision. With one exception, reported here, the binocular vision has been extramacular and never macular. In this one case the patient was proven to be a bifixator. This ...
Fitch R D - - 1984
A congenital anomalous band between the flexor pollicis longus and extensor pollicis longus tendons is, according to the literature, a rare condition. This anomalous junctura was recognized in three children with congenital thumb hypoplasia. Improved thumb function was noted after surgical release of the band in all cases. In the ...
Thomas B J - - 1984
Although physicians may be familiar with terms such as "fibular paraxial hemiamelia," they often do not realize that the defects may involve more proximal levels than are first apparent. The following case report demonstrates a paraxial defect that begins at the level of T2 and extends the remainder of the ...
Zak T A - - 1984
A case of primary congenital upper eyelid entropion ( PCUEE ) is reviewed. The author proposes a congenital shortness of the levator muscle with anomalous insertion onto the tarsal plate as the etiology, and a new simplified surgical correction is described. The classification of PCUEE as a syndrome with multiple ...
Czeizel A - - 1984
The so-called 89 VACTERL cases i.e. three or more combinations of vertebral abnormalities (V), anal atresia (A), cardial abnormalities (C), tracheo-oesophageal fistula and/or oesophageal atresia (TE), renal agenesis and dysplasia (R), limb defect (mainly radial-type reduction or polydactyly) (L) were evaluated in the material of the Hungarian Congenital Malformation Register, ...
Grill F - - 1984
This case report presents a girl with congenital short femur and a limb length discrepancy of 12 cm. After a series of complicated operations failed to lengthen the limb, we applied distraction epiphysiolysis and succeeded in correcting the length discrepancy. This method, rarely mentioned in medical literature, is described and ...
Adeyemo A O - - 1984
An unusual case of congenital lower oesophageal diaphragm (web) associated with achalasia is described. An 18-year-old nulliparous girl presented with severe cachexia and aphagia following progressive dysphagia. A barium swallow demonstrated the achalasia, and the oesophageal diaphragm with a central pinhole opening was seen at endoscopy. Parenteral hyperalimentation was required ...
Weinstein J N - - 1984
Forty-two cases of coxa vara were retrospectively reviewed. All 42 cases were classified based on their history and roentgenographic appearance. Twenty-two cases of true congenital coxa vara were identified and are the primary focus of this review. In this study we have introduced the Hilgenreiner epiphyseal (HE) angle as measured ...
McCarthy S - - 1984
We describe a newborn with multiple congenital anomalies due to the Amniotic Band Disruption Complex. Cerebral computerized tomography and plain films of the extremities corroborated the clinical diagnosis. The purpose of this case report is to alert the radiologist to the varied radiographic appearance of this little known entity and ...
Garat J M - - 1984
2 new cases of blind-ending branches of bifid ureters in children are reported. Clinical and diagnostic features as well as treatment of this pathology are presented. The authors emphasize the differential diagnosis of blind-ending ureteral diverticula, suggesting the possibility that the single congenital diverticulum is actually a dilated blind-ending ureter.
Kikuchi I - - 1984
The cul-de-sac in which the nail is formed determines that it should grow outward instead of upward according to Kligman. This view was questioned by Baran based on clinical observations and review of the literature. A case of vertically growing ectopic nail of congenital origin indicated that the cul-de-sac was ...
Peters A C - - 1984
Two Dutch siblings, diagnosed as suffering from Fukuyama type congenital muscular dystrophy (FCMD) on the basis of clinical, computerized tomography (CT), and muscle and brain biopsy findings, are reported. Hypoplasia of the chorioidea was observed for the first time in FCMD. Autopsy of the first case revealed the major pathological ...
Zancolli E A - - 1984
Two digital congenital deformities produced by failure of differentiation of parts of the retaining ligaments of the skin or the retinaculum cutis are named "congenital cutaneous ulnar drift" and "congenital cutaneous multiple camptodactyly," respectively, based on their pathogenesis and clinical characteristics. A classification of the deformities based on the tissues ...
Ekkelkamp S - - 1983
A case history is presented of a child with bilateral eventration of the diaphragm. In view of the absence of musculature and because there are no signs of neurological disorders, we would call this a bilateral congenital diaphragmatic hernia. The patient was operated upon successfully. Pathogenesis and literature of this ...
Hidalgo J E - - 1983
A case report of a congenital scalp defect with distal limb anomalies is presented. Etiology, associated anomalies, treatment, complications, and mortality are reviewed. The presence of prominent scalp veins may be an indication to defer rotation flaps and to obtain immediate coverage with a split-thickness skin graft after excision of ...
Saia O S - - 1983
A case of congenital bilateral bony choanal atresia treated by surgery is reported. A new method of insertion and retention of the modelling tubes to prevent stenosis is described. This method has the following advantages: 1. it provides a good insertion and retention of the modelling tubes; 2. it doesn't ...
Chijiiwa T - - 1983
Ocular manifestations in two cases of congenital muscular dystrophy of Fukuyama type were reported. This disease is characterized by early onset of hypotonia, generalized muscle weakness and atrophy, mental retardation, and elevated serum creatine-phosphokinase activity. The symptoms include entropion of lower lids, pathological myopia with astigmatism, optic nerve pallor, and ...
Pappas D G - - 1983
The earliest possible diagnostic evaluation of hearing impairment among infants and children is of the utmost importance for their future well-being. The essential features of the family history and the pediatric physical examination are reviewed, discussed, and illustrated in studies and actual case histories of congenital bilateral sensorineural hearing loss.
Dal Monte A - - 1983
The authors report their experience in the treatment of congenital constricting bands by Ombrédanne's two stage operation. Fourteen bands completely encircled the limb and 2 were incomplete. The patients were followed up from 6 months to 15 years (average 3 years) and in all cases the results were excellent. The ...
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