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Pathology of the umbilical cord in adrenal fusion syndrome.

We report the placental findings of a newborn with multiple anomalies that include small and fused adrenal glands, meningomyelocele, lobar holoprosencephaly, cerebellar hypoplasia, short pancreas, and small spleen, fitting with the features of the so-called adrenal fusion syndrome. The placenta was small and presented a short, undercoiled and segmentally uncoiled umbilical cord with a short constriction in the middle of its length. We speculate that adrenal fusion syndrome and abnormal spleen lobulation and short pancreas may represent part of the same complex and probably related to the one including midline anomalies and defects of laterality formation in which horseshoe (fused) adrenal gland also may be found. Undercoiled umbilical cord might represent the placental component of this malformation complex. We also underline the value in identifying abnormalities of the umbilical cord to establish the cause of death.

Abnormalities, Multiple↗

[A case of sarcoid polyradiculopathy with nerve roots swelling demonstrated by myelography].

A 62-year-old woman was admitted to our hospital because of tingling numbness in the trunk and upper extremities. She was well until 18 days earlier, when she began to feel tingling numbness on the ulnar side of the left arm. During the next two weeks it spread gradually over the trunk and ulnar side of the bilateral arms. She had also progressive difficulty in taking hold of objects. On neurological examination she was alert and cooperative with normal articulation. The neck was supple. The cranial nerves were intact. Superficial sensation was bilaterally hypesthetic in the distribution from the 7th cervical through 12th thoracic segments. Mild weakness was distally noted in the upper extremities. Deep tendon reflexes were reduced or absent without laterality. Plantar responses were bilaterally flexor. Coordination and gait were normal. Routine laboratory examinations including blood counts, blood chemistries and urinalysis were unremarkable. Serum angiotensin converting enzyme (ACE) was slightly elevated. A lumbar puncture yielded clear, colorless cerebrospinal fluid (CSF) containing 22 white cells/mm3 and protein of 106 mg/dl. Conventional nerve conduction studies were normal. F-wave conduction studies revealed elevated F-ratios for the upper and lower extremities. Studies of short-latency somatosensory evoked potential showed mild prolongation of N13 recorded after stimulation of the right median nerve. An X-ray film of the spine was unremarkable except for mild narrowing of the C5-6 intervertebral disk space. Postcontrast magnetic resonance imaging of the spine with gadolinium-DTPA was unrevealing as well as a precontrast study. A myelogram disclosed enlarged lateral filling defects corresponding to cervical nerve roots from C6 through C8 bilaterally.(ABSTRACT TRUNCATED AT 250 WORDS)

Cervical Vertebrae↗

Structural anomalies of the reproductive tract.

Structural anomalies of the female reproductive tract may be divided into disorders of lateral fusion and disorders of vertical fusion of the Müllerian duct system. In the past, these disorders were diagnosed at or after menarche or later in life during evaluation of various forms of reproductive tract failure, such as infertility and pregnancy wastage. Newer techniques including pelvic ultrasound and magnetic resonance imaging have allowed diagnosis in the pediatric patient and on occasion, even in utero. Surgical correction of lateral-fusion defects (didelphic, bicornuate, septate, and unicornuate uteri) have remained essentially unchanged except for surgical reconstruction of the septate uterus. Surgical correction of vertical defects (vaginal septi and cervical agenesis or dysgenesis) has received considerable recent interest with the development of newer techniques that may be more effective than the reconstructive procedures of the past.

Adolescent↗

Functional outcomes following palatal reconstruction with a folded radial forearm free flap.

Defects of the soft palate often occur after extirpative procedures are performed to treat oropharyngeal cancers. These defects usually result in velopharyngeal insufficiency and an alteration in speech and deglutition. Palatal prostheses have been used to circumvent this problem in the past. Recently, however, folded radial forearmfreeflaps have been introduced for reconstruction of the soft palate to eliminate velopharyngeal insufficiency and the need for a prosthesis. We conducted a study to evaluate pharyngeal and palatal functions following reconstruction of soft-palate defects with radial forearm free flaps in 16 patients who had undergone resection of the soft palate for squamous cell carcinoma. Nine patients had partial soft-palate defects and 7 had total defects. All patients had lateral pharyngeal-wall defects. In addition, 14 patients had defects of the base of the tongue. Patients were followed for 3 to 40 months. Outcome measures were determined according to several parameters, including postoperative complications, resumption of diet, intelligibility of speech, and decannulation. All patients were evaluated by a speech pathologist and an otolaryngologist with a bedside swallowing evaluation and flexible nasopharyngoscopy. Twelve patients underwent videofluoroscopic studies. There was no incidence of flap failure. One patient developed a transient salivary fistula, which resolved with conservative management. Four patients without dysphagia resumed oral intake 2 weeks after surgery. The 12 patients with dysphagia underwent swallowing therapy. Ten of them responded and were able to resume oral intake, while the other 2 required a palatal prosthesis. Overall, 10 patients resumed a normal diet and 4 tolerated a soft diet within 6 weeks. The 2 patients who required a palatal prosthesis were able to take purees. All patients were decannulated, and all were able to speak intelligibly. Speech was hypernasal in 2 patients and hyponasal in 3. We conclude that the folded radial forearm free flap procedure is a useful alternative for reconstruction of palatal and pharyngeal defects. It is safe and effective, and it results in excellent functional outcomes.

Adult↗

[Incisional hernia repair using full-thickness intraperitoneal mesh].

Prosthetic materials proved to be a real success for incisional hernia repair, still there is a strong debate regarding the best mesh and procedure to be used. Most surgeons avoid full-thickness replacement with intraperitoneal mesh because, occasionally, an intense inflammatory response may lead to severe adhesions or enterocutaneous fistula. Still, interposition of the omentum between the abdominal contents and the mesh is a safe and natural method of visceral protection. Since July 1998, we used this technique in 33 cases treated for antero-lateral abdominal defects, operations that were followed by only 15% early and 3% late complications. Monofilament polypropylene meshes (Bard, Davol Inc.) were used in most cases. Although our results need further evaluation, may be considered favourable on short and medium term comparing to Romanian experience, which is based mainly on extraperitoneal placement of the mesh. The study is a good opportunity to review the literature regarding this issue.

Adult↗

Bilateral variant origin of pectoralis major.

The variable origin of muscles is not uncommon though the complete or partial absence of the clavicular head of pectoralis major muscle is rare. This paper deals with bilateral partial absence of clavicular head of pectoralis major muscle. The clavicular head was arising only in the middle of medial half of the clavicle and was separated from sternocostal head by a gap of 2.5cm and laterally from the origin of deltoid from clavicle by a gap of 2.5cm. Nerve supply of this muscle was from lateral and medial pectoral nerves as usual. The partial absence of the clavicular head of pectoralis major muscle becomes important in cases of reconstructive surgery as this head is required to cover the lateral acromioclavicular defect and in cases of established facial paralysis.

Adult↗

Reversible corneal keratinization following trabeculectomy and treatment with 5-fluorouracil.

A 66-year-old pseudophakic man with primary open-angle glaucoma and ocular cicatricial pemphigoid underwent trabeculectomy and was given postoperative subconjunctival 5-fluorouracil. He developed an inferior corneal epithelial defect and, later, a well-demarcated area of inferior corneal keratinization. Since the corneal keratinization clinically improved with conservative management, surgical intervention was not required.

Aged↗

[High-resolution computed tomography evaluation of congenital anomalies of the temporal bone].

High-resolution computed tomography (HRCT) was used to study 23 patients with congenital temporal bone deformities. One patient had an inner ear abnormality and 3 patients were combined together with inner, middle and external ear malformations. Embryological development of the outer and middle ears is linked. Development of the inner ear occurs independently. These abnormalities included vestibule dilatation in 2 patients, a lateral semicircular canal defect in 1 case, dilatation of lateral and superior semicircular canals in 1 patient, only dilatation of the lateral semicircular canal in 1 case, oval-window non-development in 3 cases, oval aqueduct dilatation in 1 patient and inner ear abnormalities in 1 case. The study also included 17 cases of unilateral external auditory canal atresia, 5 cases of bilateral atresia, and 1 normal external auditory canal. There were 8 cases of unilateral and 2 cases of bilateral bony atresia. There were 9 unilateral and 1 bilateral cases of membranous atresia. Two cases had bony atresia on one side, with contralateral membranous atresia on the other. Nine unilateral and 2 bilateral cases were positioned superiorly and posteriorly of the mandibular condyle. Thick atresic plate were found in 10 unilateral and 2 bilateral cases. Small tympanic cavities were found in 12 unilateral and 2 bilateral patients; 7 unilateral and 2 bilateral cases had acellular and hypoplastic mastoids. Ossicular deformities were seen in 14 unilateral and 2 bilateral patients. One patient had no ossicle. Anteriorly located descending facial nerve canal was noted in 9 unilateral and 2 bilateral cases. Congenital cholesteatoma was identified in 2 patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

[Epiduralography for diagnosis of lumbar intervertebral disc protrusion: report of 304 cases].

The authors used epiduralography in 304 cases for the diagnosis of lumbar intervertebral disc protrusion. The radiopaque contrast media was injected into the epidural space and then X-rays were taken of the 304 cases, 273 cases received surgical intervention. Two hundred and seventy positive epidural X-ray, all were confirmed to have protruded or ruptured intervertebral disc at surgery. Three cases had negative epiduralgram preoperatively, no disc protrusion revealed at operation. Thus the accuracy of this technique in diagnosis of intervertebral disc protrusion was L 100%. The findings of positive epidural X-ray may be classified into 5 categories: (1) Lateral filling defect (21.2%), (2) Irregular filling defect near the center (0.7%), (3) Complete block of epidural space (35.6%), (4) Incomplete block of epidural space (27.4%), and (5) Unilateral block of epidural space (15.1%).

Humans↗

Prosthetic rehabilitation of mandibulectomy and glossectomy patients.

The prosthodontic prognosis in patients with lateral discontinuity defects of the mandible is dependent on a number of complex factors. In some patients only esthetics can be improved, whereas in others mastication and speech improvement can be achieved. Two patients who had undergone the same surgical interventions but presented with different problems are described. The first patient had difficulty with mastication and mandibular movements, which was resolved by building a ramp on a maxillary prosthesis. The second patient had severe speech and swallowing problems, which were corrected with an acrylic, hollow speech prosthesis. In both patients the end result was satisfactory.

Adult↗

[Surgical treatment of recurrent ventral dislocation of the shoulder using the Weber rotation osteotomy].

There are different procedures for surgical treatment of a recidivating ventral dislocation of the shoulder. In our hospital the rotatory osteotomy according to Weber proved good; in accordance with the findings it can be combined with other procedures. The surgical procedures and our results are explained. In our opinion, this surgical treatment will effect, besides known results (rotation of head defect to lateral), an essential stabilisation of the joint, by means of strain exercised on M. infraspinatus and the upper part of M. teres minor.

Adolescent↗

[Operative results of acquired valvular disease with blood cardioplegia followed by terminal cardioplegia].

Cold blood cardioplegia followed by terminal cardioplegia was employed as a method of myocardial protection for acquired valvular disease. Postoperative clinical results of both cardiac iso-enzyme and cardiac function were discussed from the effect of the myocardial protection. In operative procedures of 62 cases, 30 cases underwent mitral valve replacement and other mitral repair, 17 cases aortic valve replacement, 10 cases double valve replacement and 5 cases modified Bentall operation. Iso-enzymes of Creatine-Kinase (CK) and Lactate-Dehydrogenase (LDH) were measured by the constant time-interval. Cardiac function was estimated in acute postoperative phase and late phase. Hospital mortality was 1.5%. The cause of death was thought to be postoperative Graft Versus Host Disease with skin rash and pancytopenia. Cardiac function during acute phase well recovered in 62 cases of which two cases were controlled with intra-aortic balloon pumping. The values of CK-MB were measured during aortic cross-clamp, 30 min, 3 hours, 6 hours and 24 hours after cross-clamp release. Peak CK-MB value was detected 3 hours or 6 hours in almost cases. In contrast, peak LDH-1 value was detected 24 hours after cross-clamp release. Perioperative myocardial infarction was occurred in one case with modified Bentall operation whose CK-MB value was elevated over 150 IU/L at 3rd hour and 24th hour. However, the cardiac radio-isotope data of this case revealed good cardiac function with left ventricular ejection fraction (LVEF) 76% by cardiac pool imaging in spite of small postero-lateral perfusion defect by Thallium 201 scintigram.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Revision total hip arthroplasty with titanium ingrowth prosthesis and bone grafting for failed cemented femoral component loosening.

Fifty-seven hips (55 patients) had revision for failed cemented femoral component loosening using titanium ingrowth femoral components and cancellous bone grafting. The patients' average age was 59 years (range, 25-86 years), and the average follow-up period was 2.8 years (range, two to six years). The preoperative hip score averaged 45.5 (range, 10.0-80.7) and the postoperative hip score averaged 82.5 (range, 43.0-100.0). Complications included dislocation (4.0%), infection (4.0%, one recurrence from a previously infected hip and one acute hematogenous infection), and a 4.0% revision rate for loose femoral component. Another patient had a revision for a loose acetabular component. All parameters of hip function (i.e., pain, limp, activities of daily living, use of support, and distance walked) improved with time. Femoral component loosening is classified into four types based on the severity of loosening and instability. In Type I there is minimal endosteal or inner cortical bone loss, i.e., loosening from the cement-metal-bone interface or a broken stem (seven hips). In Type II there is proximal canal enlargement with cortical thinning of 50% or more and sometimes a lateral wall defect with an intact circumferential wall (23 hips). In Type III there is a posteromedial wall defect involving the lesser trochanter (23 hips). In Type IV there is total proximal circumferential bone loss in varying distances below the lesser trochanter (three hips). The Harris hip scores for the four groups were 93.0, 83.0, 80.0, and 78.0, respectively.

Adult↗

Arthroscopy update #1. Treatment of osteochondrosis dissecans of the knee by arthroscopic curettage, follow-up study.

Thirty-seven patients with osteochondrosis dissecans involving the femoral condyles of the knee were treated with arthroscopic removal of all partially or completely detached osteochondral fragments and with curettage of the crater to bleeding bone. Ten patients underwent a repeat arthroscopy five to 15 months later. All defects had filled with fibrocartilage.

Adolescent↗

[Animal experimental studies on bone regeneration following drilling of the bone].

Experiments in dogs showed that drilling at the surface of bone provokes damage which increases with the number of revolutions per minute. Polychrome sequence marking and histological tests demonstrated that filling up of bony defects started later with turbine-driven drills than with drills of 2,000 r.p.m. and has not even been completed after eight weeks. It is therefore recommended to abstain from using turbines in drilling at the surface of bone.

Animals↗

[Anomalous origin of the left coronary artery. Presentation of 5 cases].

Clinical findings are reported in 5 cases of anomalous left coronary artery originating from pulmonary trunk, with reference of ventricular function evaluated through echocardiographic and angio-haemodynamic methods. All patients were catheterized between 7 and 8 months of age. Left atrial and ventricular dilatation, diminished shortening of minor diameter and free wall thickness and septum normal values were obtained by echocardiography. Quantitative angiography of left ventricle demonstrated an elevation of end-diastolic volumes (mean values: 270% of predicted normal). The ejection phase indexes are diminished. The left ventricular mass/end-diastolic volume ratio was low in all cases. Myocardial imaging with thallium-201 demonstrated perfusion defect in lateral area of left ventricle in one patient. The electrocardiographic signs of myocardial necrosis support the diagnosis, and the echocardiographic and isotopic studies may contribute to it. The angio-haemodynamic method corroborate the diagnosis, and the myocardial pump function evaluation may help to decide the most appropriate treatment for this anomaly.

Aortography↗