Protein-carboxyl methyltransferase of the bovine posterior pituitary gland: neurophysin as a potential endogenous substrate

The problem of the varus hindfoot in children ages six to 10 has been discussed. A preliminary follow-up is presented on six patients treated by extensive medial release, multiple tendon lengthenings, and subtalar intra-articular arthrodesis. Two of our patients revealed incomplete correction of the deformity because of technical errors in the medial release. However, there have been no failures of fusion and no progression of the deformity to date. This is a preliminary report and further follow-up is indicated. It should be noted that we have now extended our series to 40 patients.     

Sequence effects, health profiles, and the QALY model: in search of realistic modeling

In limbs with combined shortening with angulation or malrotation, deformity may be quickly or slowly corrected before lengthening with external fixation. We examined a series of 35 patients with 40 limbs that underwent acute deformity correction and subsequent gradual lengthening. The average deformity corrected was 19 degrees, with subsequent average lengthening of 4.1 cm. Good radiographic callus formation was noted in 34 of the 40 segments studied. The magnitude of deformity correction had no effect on the quality of lengthened bone, incidence of complications, or the healing index. Skeletally mature segments had statistically significant decreased bone formation (p = 0.001), increased prevalence of callus complications (p = 0.001), and a higher healing index (p = 0.003). Based on this experience, it is our conclusion that immediate correction and lengthening is suitable in children and adolescents who have malaligned and shortened lower extremities. Because of poorer results in older patients, we believe that other techniques should be considered in adults.     

Functional involvement of cerebral cortex in Duchenne muscular dystrophy

Proximal femoral varus and derotation osteotomy is a common procedure performed in the management of developmental dysplasia of the hip. This procedure imposes high shear stress on the femoral epiphysis, depending on the degree of varus obtained. We report two cases of proximal femoral epiphyseal slip after varus derotation osteotomy and discuss the management and outcome. Such epiphyseal slip may or may not be symptomatic, and a careful radiologic examination should be carried out in suspected cases. Management should be individualised. Surgical correction of varus may be required.     

Transmetatarsal amputation

We have reviewed the records of 25 patients who underwent a transmetatarsal amputation at San Francisco General Hospital. The average patient age was 63 years old. Twelve of the patients were diabetic, while transmetatarsal amputations were performed in eleven with simple arteriosclerosis. Two patients underwent amputations for either trauma or nonhealing ulcer. Thirteen of the patients healed their amputation, and twelve of these became ambulatory. Eleven required higher amputation, because of nonhealing due to infection in seven and progressive ischemia in four. One patient died on the first postoperative day of pneumonia. The failure group was younger, contained more diabetics, and had a higher incidence of infection. The operative procedure of transmetatarsal amputation is described. We believe that patients with distal gangrene without spreading infection should be considered for transmetatarsal amputation, reserving initial below-knee amputation for those with greater involvement of the foot.     

Roger A. Mann Award. Juvenile hallux valgus: etiology and treatment

In an 11-year retrospective study of 45 patients (60 feet) with juvenile hallux valgus, a multiprocedural approach was used to surgically correct the deformity. A Chevron osteotomy or McBride procedure was used for mild deformities, a distal soft tissue procedure with proximal first metatarsal osteotomy was used for moderate and severe deformities with MTP subluxation, and a double osteotomy (extra-articular correction) was used for moderate and severe deformities with an increased distal metatarsal articular angle (DMAA). The average hallux valgus correction was 17.2 degrees and the average correction of the 1-2 intermetatarsal angle was 5.3 degrees. Good and excellent results were obtained in 92% of cases using a multiprocedural approach. Eighty-eight percent of patients were female and 40% of deformities occurred at age 10 or younger. Early onset was characterized by increased deformity and an increased DMAA. Maternal transmission was noted in 72% of patients. An increased distal metatarsal articular angle was noted in 48% of cases. With subluxation of the first MTP joint, the average DMAA was 7.9 degrees. With a congruent joint, the average DMAA was 15.3 degrees. In patients where hallux valgus occurred at age 10 or younger, the DMAA was increased. First metatarsal length was compared with second metatarsal length. While the incidence of a long first metatarsal was similar to that in the normal population (30%), the DMAA was 15.8 degrees for a long first metatarsal and 6.0 degrees for a short first metatarsal. An increased DMAA may be the defining characteristic of juvenile hallux valgus. The success of surgical correction of a juvenile hallux valgus deformity is intimately associated with the magnitude of the DMAA. Moderate and severe pes planus occurred in 17% of cases, which was no different than the incidence in the normal population. No recurrences occurred in the presence of pes planus. Pes planus was not thought to have an affect on occurrence or recurrence of deformity. Moderate and severe metatarsus adductus was noted in 22% of cases, a rate much higher than that in the normal population. The presence of metatarsus adductus did not affect the preoperative hallux valgus angle or the average surgical correction of the hallux valgus angle. Constricting footwear was noted by only 24% of patients as playing a role in the development of juvenile hallux valgus. There were six recurrences of the deformities and eight complications (six cases of postoperative hallux varus, one case of wire breakage, and one case of undercorrection).     

Clinical efficacy of ultrasound guided percutaneous drainage of abscesses in patients with leukaemia and lymphoma

Ultrasound guided percutaneous drainage (US-PD), a minimally invasive technique, has been reported as highly effective for the treatment of deeply located abscesses, particularly in immunocompromised patients. Therefore, we retrospectively studied its therapeutic efficacy and safety in a series of 14 patients with leukaemia and lymphoma. We collected the clinical and sonographic data of 14 patients with various types of leukaemia and lymphoma. These patients were consecutively observed in four clinical centres with long-term experience with ultrasound guided therapeutic techniques. The cases were analysed according to underlying disease, clinical features, location of the abscess, drainage technique, microbiological data and both short- and long-term outcome. In our series, 11 patients were treated with repeated ultrasound guided needle aspirations (US-NA) and 3 underwent catheter drainage (US-PCD). In 12/14 cases the procedure was successful (86%): the mortality rate was 14%. 5 patients died during the follow-up period because of the underlying disease, without abscess recurrence. No complications were reported. Our data suggest that ultrasound guided percutaneous drainage should be considered the first choice, minimally invasive procedure for the treatment of deeply located abscesses in patients with leukaemia and lymphoma.     

Gold weights for the treatment of lagophthalmos caused by facial paralysis. Our experience and review of the literature

Paralysis of the orbicularis oculi muscle in patients with facial palsy can originate serious functional and esthetic problems. The implantation of a gold weight in the upper eyelid is one of the many surgical techniques that have been described for the correction of lagophthalmos in these patients. In this article we review the literature and present our experience with gold weight implantation for eyelid reanimation in patients with facial palsy. This procedure is technically simple to perform, produces good functional and cosmetic results, has a relatively low complication rate and is reversible should facial function return.     

Low incidence of myelodysplastic syndrome following transplantation using autologous non-cryopreserved bone marrow

Between May 1984 and October 1995 we performed 114 autologous stem cell transplants for lymphoma in our centre; 77/114 (68%) were transplanted after primary therapy. The conditioning regimen varied according to diagnosis; 26 patients were conditioned with melphalan and total body irradiation, 66 received melphalan and etoposide and the remainder (50) were conditioned with melphalan alone. The median follow-up is 62 months. Only two new haematological malignancies have occurred, both in patients with Hodgkin's disease. One patient developed Ph+ chronic myeloid leukaemia 18 months post-transplant. In this case, because of the timing of the haematological disorder, we considered the malignancy to be concurrent with or to have preceded the transplant. A second patient developed acute myeloid leukaemia 20 months post-transplant. She had been treated for Hodgkin's disease for 10 years and was transplanted in third complete remission. Cytogenetic analysis in this case showed trisomy 11. We believe this to have been an unequivocal second malignancy. Our finding of a 1.1% incidence of secondary haematological malignancy (95% CI 0.02-4.96) from a census population adds weight to the hypothesis that haematological problems post-transplant reflects prior chemotherapy rather than toxicity from the transplant procedure itself.     

De novo balanced translocation (6;18)(q21;q21.3 or q22) [corrected] in a patient with heterotaxia

Although alternative therapy for PCP remains limited, the role of TMP/SMX desensitization becomes increasingly important in patients with AIDS. Various successful desensitization protocols have been described in this article. As there are no established guidelines, it appears that the desensitization procedure can occur in small successive doses given each day or one small dose given daily. An evaluation of the severity of allergic reaction can be used to determine the type of dosing regimen. We believe that protocols starting with low doses and slow titration to full-dose therapy, as used at our institution, should be efficacious. Monitoring of the patient after the desensitization procedure should continue, as sensitivity may reoccur. In addition, while the patient is undergoing desensitization, some investigators recommend that alternative therapy be continued until full-dose TMP/SMX therapy is achieved. Also, it is important to realize that once a patient is successfully desensitized, medication compliance must be maintained because, theoretically, reexposure to the drug after a lapse in therapy may result in hypersensitivity reactions. Therefore, this procedure and the possibility of serious adverse effects, such as Stevens-Johnson syndrome and anaphylaxis, should be evaluated carefully and discussed thoroughly with each patient prior to initiation of therapy. Finally, a study of sufficient size should be performed to evaluate the efficacy of desensitization regimens and establish specific dosing guidelines.     

Replacement arthroplasty of the valgus knee. A modified lateral capsular approach with repositioning of vastus lateralis

Total knee arthroplasty (TKR) using a medial capsular approach gives worse results in arthritic knees with valgus deformity than in those in varus, usually because of swelling, poor wound healing and stiffness, instability, recurrent valgus deformity and poor patellar tracking. A technique for replacement TKR of valgus knees using a lateral capsular approach was described several years ago, but was not routinely adopted because of the difficulties with and complexity of the procedure which included deliberate elevation of the tibial tubercle. In order to avoid this we have modified and simplified the procedure. Our preliminary results suggest that this lateral approach is safe and may give a better outcome than that through the medial capsule for the replacement of valgus knees.     

Optimizing laparoscopic splenectomy. Technical details and experience in 59 patients

BACKGROUND: Laparoscopic splenectomy (LS), like other advanced laparoscopic procedures, is still an evolving procedure. The indications for surgery, criteria for patient selection, and operative technique are not yet well defined. We have therefore modified the standard technique for performing LS in an attempt to optimize the procedure. METHODS: Over the past 2 years, we have performed LS in 59 patients. The last 43 patients were operated using a standardized technique that we believe to be optimal. It includes the routine use of the right lateral position, operating through three trocars, the mass transection of the splenic vasculature with a vascular endoscopic stapler, and the use of a self-retaining retrieval bag. RESULTS: The average operating time was 79 min. Average blood loss was 95 cc, and average postoperative hospitalization was 2.3 days. There was one intraoperative complication and one postoperative complication. These results are superior to those we achieved earlier in our own experience, as well as to similar series that have been published recently. CONCLUSIONS: In our experience, the use of this new technique resulted in relatively short procedures with low morbidity. We believe that these results justify the use of LS as the procedure of choice for elective splenectomy in patients with normal or moderately enlarged spleens.     

Review of radiographic measurements following Austin bunionectomy

This study compared preoperative and postoperative results for selected radiographic measurements of 30 patients undergoing the modified Austin bunionectomy procedure for the correction of hallux abducto valgus. Significant reductions in all postoperative radiographic values were demonstrated, including hallux abductus angle, metatarsus primus adductus angle, tibial sesamoid position, and first metatarsal protrusion distance.     

Neonatology radiology casebook. Tracheal agenesis

BACKGROUND: Biliary tract diseases are frequent in heart transplant recipients, with significant morbidity and mortality. Since the first presentation of gallstones in this population is often acute cholecystitis, asymptomatic cholelithiasis should not be considered benign. PATIENTS AND METHODS: We retrospectively reviewed 18 heart transplant recipients who underwent cholecystectomy from January 1991 to June 1997. We intentionally chose to perform a straightforward open procedure when acute cholecystitis was suspected (3 patients). A laparoscopic cholecystectomy was performed in all the other cases (15 patients) without conversion to open procedure. CONCLUSION: Since no significant complications were observed in our patients, we believe that transplant recipients with cholelithiasis should undergo laparoscopic cholecystectomy in their posttransplantation course regardless of the symptomatic status of their biliary tract.     

Frontoethmoidal meningoencephaloceles: a review of 14 consecutive patients

Over a period of 4.5 years, 14 patients with frontoethmoidal meningoencephaloceles were treated. Most patients came from Northern Namibia. Precise delineation of all cranial abnormalities was obtained by modern imaging techniques, and specific patterns of cerebral abnormality were found. The malformation was corrected in a single stage, and significant modifications have been developed to render the procedure simpler and safer. Information from our series favors delayed neural tube closure as the primary pathogenesis of the defect and suggests a common teratogen as the most probable etiological agent. Our experience leads us to advocate early correction of even small defects.     

Iatrogenic metatarsus primus elevatus. Etiology, evaluation, and surgical management

Iatrogenic metatarsus primus elevatus is an infrequent but devastating complication of first ray surgery. The authors address their clinical and radiographic evaluation of metatarsus primus elevatus, and describe a surgical treatment with emphasis on the sagittal plane Z-osteotomy. This osteotomy provides predictable and versatile correction for the treatment of iatrogenic deformities of the first metatarsal. It allows for plantarflexion and lengthening of the first metatarsal while avoiding an interpositional bone graft. The technical aspects of the procedure are thoroughly discussed.     

5-year series of constricted (lop and cup) ear corrections: development of the mastoid hitch as an adjunctive technique

Despite the multitude of corrective procedures described, adequate surgical correction of the congenital constricted ear remains a challenge. The maintenance of the shape and elevation of the reconstructed upper neohelix poses a particular problem. In the present series, experiences with lop ear correction utilizing standard techniques and the use of the mastoid hitch as a useful adjunct to these procedures are described. A total of 19 ears were reconstructed. There were three type 1, eight type 2a, seven type 2b, and one type 3 deformities (Tanzer classification). A graded sequence of procedures was adopted. Mild deformities were corrected by cartilage scoring techniques; a V-Y advancement of the helical root was added for moderate deformities. Cartilage expansion by a banner flap was required for more severe deformities. A mastoid hitch, whereby the refashioned upper neohelix is sutured to the mastoid fascia, should be used as an adjunct to these procedures to maintain helical elevation and prevent recurrence. Severe type 3 deformities may require autologous auricular reconstruction. Mean follow-up time was 1 year. There were six excellent, seven good, four fair, and two poor results. Two patients who had not had mastoid hitch procedures developed a recurrence of the lop deformity. Adequate surgical correction of constricted ear deformities requires a variety of surgical techniques. The mastoid hitch being used for constricted ear correction has not been described elsewhere. The mastoid hitch is a useful adjunctive procedure that may be used effectively in combination with other procedures.     

The twisted nose

To the patient seeking correction of a twisted nose, it is as important to obtain a good shape as it is to achieve normal breathing. The functional problem must be solved regardless of the extent of the resection required. The plastic surgeon may choose among many techniques, some of which have been elucidated. Our preferred approach has been described in detail. We reiterate that experience has shown that a deviated septum which requires stabilization after surgery will recur to a greater or lesser degree at a later date. A twisted nose, to be permanently corrected, should maintain its corrected position without external support at the end of the operation.     

Pregnancy following gastric bypass for morbid obesity

BACKGROUND: Women who suffer from morbid obesity are often infertile. If these women are able to become pregnant, they are considered high risk because of the hypertension, diabetes and other associated risk factors. Following the pregnancy is difficult due to limitations of the physical examinations. More costly ultrasound examinations are needed at a higher frequency. Bariatric surgery reduces the woman's weight and the incidence of obesity related co-morbidities. The number of pregnancies and rate of complications during those pregnancies in our post-bariatirc surgical patients were evaluated. METHOD: Our group has been doing bariatric surgery since the early 1980s. We have over 2000 active patients on our current newsletter mailing list. The patients also have a series of networks through support groups. The patients are informed to contact us when they become pregnant so we may assist the obstetrician with their care. Through these various means, we have been able to identify 41 women in our patient population who have become pregnant. Using personal interview, questionnaire, and review of perinatal records, pregnancy-related risks and complications were studied. RESULTS: With over a 95% follow-up rate on the patients identified as having been pregnant following surgery, we found less risk of gestational diabetes, macrosomia, and cesarean section than associated with obesity. There were no patients with clinically significant anemia. CONCLUSION: Since the patients had an operation that restricts their food intake, some basic precautions should be taken when they become pregnant. With this in mind, our patients have done well with their pregnancies. The post-surgical group had fewer pregnancy-related complications than did an internally controlled group that were morbidly obese during their previous pregnancies.     

The axillo-axillary bypass graft: further experience

The recent utilization of the axillo-axillary bypass graft for revascularization of proximal subclavian or innominate artery occlusions has proved to be a successful operation, technically simple with a low operative morbidity. We have reviewed the reported experience to date and have added our experience of nine additional cases. The procedure has produced excellent results in all patients, with no evidence of postoperative "steal" from the donor limb. Preoperative hemodynamic defects were corrected. Our experience has led us to the conclusion that this simpler and safer extrathoracic approach should be considered as the procedure of choice when arterial lesions allow for its selection and use.     

Orthopaedic aspects of prune belly syndrome

Prune belly syndrome is a relatively uncommon disorder that is characterized by intrauterine urinary obstruction associated with cryptorchisdism, oligohydramnios, and orthopaedic deformations. The oligohydramnios is believed to produce limited intrauterine space, which in turn leads to fetal compression and the resultant deformities. The deformities observed in our patients were developmental dislocation of the hip (DDH), clubfeet, metatarsus adductus, vertical talus, and congenital muscular torticollis. These deformations should be treated aggressively because children with prune belly syndrome may be expected to have a relatively normal life if their renal function is good. The one exception is that as infants these children are very susceptible to pulmonary infections because of their inability to cough. Therefore, treatment of the hip and other deformations should be delayed until the children are old enough to be able to clear pulmonary secretions easily.