Atrial isomerism: a surgical experience.

Most publications on atrial isomerism are autopsy or case reports. The authors review 41 consecutive children operated on from 1980 through to 1996 with emphasis on associated cardiac anomalies, surgical procedures and outcome. Left atrial isomerism was present in 23 patients. Interruption of the inferior vena cava (56%), atrio-ventricular septal defect (47%), common atrium (38%) and cor triatriatum sinistrum (30%) were the most common diagnoses. Biventricular repair was achieved in 17 children and total cavo-pulmonary connection in two. Three underwent staged palliation: modified Blalock-Taussig shunt for two and bidirectional Glenn anastomosis for one. The remainder received a cardiac pacemaker. One patient died early after repair. Two underwent reoperation to correct a regurgitant left atrio-ventricular valve: one of these, in another hospital, had peroperative death. Three died later. Actuarial survival rate after repair and total cavo-pulmonary connection that was stabilized after 2 years was 84%. In the 18 children with right atrial isomerism, pulmonary atresia or stenosis predominated (89%) with discordant ventriculo-arterial connection (72%), atrio-ventricular septal defect (72%), 'single' ventricle (55%) and extracardiac total anomalous pulmonary venous drainage (50%). Biventricular repair was achieved in two patients and complete Fontan circulation in eight. The other eight underwent various staged palliative procedures and correction of extracardiac total anomalous pulmonary venous drainage. Five patients died postoperatively: two in our unit after modified Blalock-Taussig shunt and total cavo-pulmonary connection, three in other hospitals after repair (n = 1) and Fontan (n = 2). Five died later. One was lost for review. Survival after repair and Fontan stabilized after 6 months at 49%. In conclusion, the cardiovascular malformations associated with left atrial isomerism can often be successfully corrected. Those accompanying right atrial isomerism usually preclude a biventricular repair, require staged palliation and carry a poor prognosis.     

Laparoscopic repair of Morgagni-Larrey hernia in a child

Primary laparoscopic repair of Morgagni-Larrey hernia has been described in adult patients but not in children. This is the first report of primary laparoscopic correction in the pediatric age group without using a prosthesis. A Morgagni-Larrey hernia was found incidentally in a 3-year-old-girl. Laparoscopic correction of the defect was performed. After 6 months the patient is doing well. The chest radiograph shows complete resolution of the hernia. The laparoscopic approach allowed repair the hernia with minimal invasiveness. Laparoscopic correction is not difficult except for those hernias in which dense adhesions are present.     

One-stage neonatal corrective repair for d-transposition of the great arteries and complete atrio-ventricular canal.

Association of d-transposition of the great arteries and complete atrio-ventricular canal constitutes an uncommon and complex cardiac anomaly usually associated with poor prognosis. We report our experience on one-stage neonatal repair for d-transposition of the great arteries and complete atrio-ventricular canal. Between August 1997 and 2005, four patients (two males and two females) underwent anatomical correction for d-transposition of the great arteries and complete atrio-ventricular canal using an arterial switch procedure and two-patch repair. Mean age and weight at operation were 20 days (range from 3 to 28 days) and 3.2kg (range from 2.7 to 3.5kg), respectively. None of the patients received preoperative palliative procedure. Associated lesions were left outflow tract obstruction in three patients and multiple muscular ventricular septal defects in two patients. All four patients survived the operation. There was one in-hospitality death due to fungal sepsis. One patient required late re-operation for left ventricular outflow tract obstruction and left atrio-ventricular valve regurgitation. For a mean follow-up of 67 months (range from 51 to 90 months) all patients are asymptomatic and with no residual defects. Corrective repair of d-transposition of the great arteries and complete atrio-ventricular canal can be successfully achieved in this very challenging population during the neonatal period.     

KMFTR (Kotz Modular Femur Tibia Reconstruction System) modular tumor endoprosthesis system for the lower extremity]

206 patients with tumorendoprosteses of the KMFTR-type at the lower extremity were operated at the Institute Rizolli Bologna and at the University Clinic of Vienna. The minimum follow up was 2 years (mean 3.2, max. 8 years). In order to bridge the defect caused by tumor resection the proximal femur was replaced in 42 cases, the distal femur in 119 cases and in 6 patients a total femur prosthesis was used. The proximal tibia was replaced in 35 cases and in 4 cases a total knee prosthesis was implanted. Clinical evaluation according to Enneking revealed 71.6% excellent and good results. In 83.4% the radiological score was higher then 20. We saw deep infections in 8.2% (17 patients), breakage of the prosthesis in 6.8% and aseptic loosening in 5.8%. 3 patients had to be amputated due to infection. In the remaining cases of infection in 66% of the patients healing was observed. All cases of aseptic loosening or breakage could be revised successfully by an exchange of the prostheses and therefore salvage of the leg was achieved.     

Die versorgung von femurfrakturen bei hüftendoprothese

Between 1995 and 1997 we operated 24 patients with implanted hip prosthesis and periprosthetic fractures of the proximal femur using a revision-prosthesis with elastic stem. In 6 patients we performed a primary revision due to aseptic loosening and massive bone loss. In 5 cases fractures occurred intraoperatively during prosthesis revisions, in 6 cases fractures of the prosthesis and/or femur existed preoperatively and in 5 patients we saw pathologic femur fractures. In one patient we performed an osteotomy and correction of the femur axis during prosthesis revision and in another patient we removed a broken stem caused by femur pseudarthrosis. No acetabular revision had to be performed. The used prosthesis stem (ESKA-Implants, Lübeck) was made of a proximal massive and a distal elastic part with the possible interlocking screw placement. During postoperative mobilisation partial weight bearing was allowed for 3 months, then full weight bearing was aimed after removal of the interlocking screws. In all treated patients we saw a good prosthesis ingrowth. We present the elastic stem as good implant, especially in case of femur fractures or bone stock loss.     

Surgical repair of double-orifice of the mitral valve in cases with an atrioventricular canal defects

OBJECTIVE: A Double-orifice in the mitral valve is an uncommon congenital cardiac lesion which occurs as an isolated anomaly or in association with other cardiac malformation. This report deals with our surgical experience of a double-orifice of the mitral valve in cases with an atrioventricular canal defect. PATIENTS AND METHODS: From 1991 through 1999, ten patients were diagnosed to have a double-orifice of the mitral valve at Shizuoka Children's Hospital. Each patient had associated major cardiac malformations, among which atrioventricular canal defect underwent surgical management, with five of these undergoing complete correction with or without previous pulmonary artery banding. Of these 10, the five cases were enrolled in this study. Two of these had a complete type, and the other three had a partial type. The cleft in the left-sided atrioventricular valve was closed partially in four and left untouched in one. Bridging tissue, when present, was left intact. There was no regurgitation from any accessory orifice and no repair for an accessory orifice was needed. RESULT: There was no late death and no replacement of the valve with prosthesis. During follow-up ranging from 1 to 4 years, none of the patients developed severe stenosis or progressive regurgitation in the left-sided atrioventricular valve. CONCLUSION: Meticulous surgical management of a double-orifice in the mitral valve in association with atrioventricular canal defect an achieve an acceptable midterm result without developing severe dysfunction in the left-sided atrioventricular valve.     

Results of surgical correction of congenital talipes equinovarus]

Different surgical procedures in the treatment of clubfoot were analyzed, especially in correlation to over-correction and inadequate correction. Indications for surgery, surgical errors and their influence on outcome were assessed. 82 children (28 females and 54 males) with 130 congenital equinovarus underwent surgery between 1988 and 1994. Age at the time of operation ranged from 6 to 13 months (average: 9 months). Posterior release (i.e. partial subtalar release) was conducted in 68 cases of clubfeet and complete subtalar release in 62 cases. During follow-up 44 children with 64 clubfeet were reviewed. Magone's criteria were used to assess final results. 15 (23%) feet showed very good results, 26 (41%)--good results, 14 (22%)--satisfactory and 9 (14%)--poor or no correction.     

Transgenicular amputation with myocutaneous gastrocnemius flap in arterial occlusive disease]

41 knee disarticulations in patients with gangrene due to periphere vascular disease were performed using a myocutaneous flap of the gastrocnemius muscle as described by Klaes and Eigler in 1985. In many cases disarticulation was preceeded by reconstructive vascular procedures or amputation at a lower level. Primary healing of the stump was achieved in 80% of the cases. 29% of the patients were able to walk with the aid of a prosthesis.     

Autodermoplastic closure of defects of donor's zone after taking free revascularized autotransplants

Problems of autodermoplasty in donor's zone after taking of free revascularized autotransplants for closure of skin defect in 85 patients are regarded. All the transplants were complex. Free autodermoplasty was used in the zone of forearm -- 50 (58.8%) cases, foot - 22 (25.9%), posterior surface of thorax -- 9 (10.6%), and also in the zone of cnemis, lateral surface of femur, deltoid and scapular region -- one (1.2%) case each. In 52 (61.2%) cases free autoskin required for complete closure of defect in donor's zone and in 33 (38.8%) -- for partial closure. Region of taking of free autoskin was anterior-lateral surface of femur in majority of cases - 73 (85.9%). Split-thickness free autoskin was used the most often -- 79 (92.9%) cases. Graft retention was complete or partial in 77 (90.6%) cases, complete necrosis of free autoskin was seen in 8 (9.4%) clinical cases. It is concluded that method of free autodermoplasty is effective for closure of defects including in combination with microsurgical transplantation of tissues.     

Surgical correction of congenital heart defects in infants and young children]

The results of surgical treatment of congenital heart diseases in 595 children under 3 years of age are generalized. There were no fatal outcomes after correction of coarctation of the aorta (9 cases), patent ductus arteriosus (312), pulmonary and aortic stenoses (7). Among 62 patients who underwent removal of an atrial septal defect 3 (4.8%) died. Operations were performed on 140 patients for a ventricular septal defect with high pulmonary hypertension, 31 (22.1%) of them died from various causes. Operations for complicated heart diseases were acts of despair in children whose condition was critical.     

Gastrocnemius myotendinous flap for patellar or quadriceps tendon repair, or both

The authors' experience with simultaneous reconstruction of the quadriceps femoris or patellar tendon or both and soft tissue defect using a musculotendinous unit of the gastrocnemius muscle is presented. Five patients with a partial or complete defect of the quadriceps or patellar tendon or both and additional large soft tissue defects underwent reconstruction applying this technique as a one-stage surgical procedure in different variations. In cases with a partial defect of the tendon or loss of tendon thickness, the thick aponeurosis from the deeper aspect of the gastrocnemius was dissected and transferred as a pedicled tendon flap to reconstruct the tendon defect. In cases with a complete defect of the tendon, the superficial layer of the Achilles tendon together with the deep aponeurotic layer of the gastrocnemius muscle served to reconstruct the tendon. In both procedures the gastrocnemius muscle belly provided soft tissue coverage and was covered with a split thickness skin graft. One patient had a marginal deep necrosis develop that had to be covered with the other gastrocnemius muscle in a second operation. One patient with chronic polyarthritis and infection of his knee prosthesis declined additional reconstruction surgery and had the leg amputated. The average followup was 3.5 years. All patients achieved good results in active extension of the knee with an extension deficit of only 5 degrees to 15 degrees. The range of flexion was at least 90 degrees. The surgical technique described in this report provides functional tendon reconstruction and adequate soft tissue repair simultaneously.     

Remodelling after femoral shaft fracture in children.

Fifty-two children in the 1-14 year age range who had a femoral fracture and were treated by traction and casting, were re-examined 2-17 years later. The results of radiological measurements of femoral length and alignment are analysed by computer. The femoral over-growth following fracture 10.7+/-6.6 mm, did not depend on site or dislocation of fracture. Lateral dislocation completely corrected, varus deformity on an average only up to 40% and valgus deformity up to 60% of the initial defect. Minor deviations corrected less. Repairing continued for over 5 years. Ante- and recurvatum could correct nearly 70%, if the original deformity was over 10 degrees, otherwise less. In neither plane was the correction of axial deviation influenced by site or type of fracture. There were individual cases of complete correction of axial dislocation and cases of complete failure to correct.     

Revision of infected knee arthroplasties in Denmark

Background and purpose — The surgical treatment of periprosthetic knee infection is generally either a partial revision procedure (open debridement and exchange of the tibial insert) or a 2-stage exchange arthroplasty procedure. We describe the failure rates of these procedures on a nationwide basis.

Patients and methods — 105 partial revisions (100 patients) and 215 potential 2-stage revision procedures (205 patients) performed due to infection from July 1, 2011 to June 30, 2013 were identified from the Danish Knee Arthroplasty Register (DKR). Failure was defined as surgically related death ≤ 90 days postoperatively, re-revision due to infection, or not reaching the second stage for a planned 2-stage procedure within a median follow-up period of 3.2 (2.2–4.2) years.

Results — The failure rate of the partial revisions was 43%. 71 of the partial revisions (67%) were revisions of a primary prosthesis with a re-revision rate due to infection of 34%, as compared to 55% in revisions of a revision prosthesis (p = 0.05). The failure rate of the 2-stage revisions was 30%. Median time interval between stages was 84 (9–597) days. 117 (54%) of the 2-stage revisions were revisions of a primary prosthesis with a re-revision rate due to infection of 21%, as compared to 29% in revisions of a previously revised prosthesis (p = 0.1). Overall postoperative mortality was 0.6% in high-volume centers (> 30 procedures within 2 years) as opposed to 7% in the remaining centers (p = 0.003).

Interpretation — The failure rates of 43% after the partial revision procedures and 30% after the 2-stage revisions in combination with the higher mortality outside high-volume centers call for centralization and reconsideration of surgical strategies.     

Surgical treatment of congenital heart defects with pulmonary hypertension in children during their 1st year of life under the conditions of extracorporeal circulation

In the period between 1983 and 1987 operations were carried out on 204 children aged from 25 days to 12 months (body weight of 3 to 10 kg) for congenital heart diseases and pulmonary hypertension. Surgery for ventricular septal defect was undertaken on 173 patients (total mortality 6.9%), transposition of the major vessels with concurrent ventricular septal defect was corrected by Senning's method in 9 patients (mortality 11%), total anomalous pulmonary vein drainage was relieved in 14 patients (mortality 28.6%). No fatal outcomes occurred in correction of an aorto-pulmonary fistula, origin of the aorta and pulmonary artery from the right ventricle, and a common ventricle. In all patients, with the exception of 4 (2 with secondary and another 2 with primary pulmonary hypertension), pressure in the pulmonary artery reduced significantly.     

Evolving surgical management for ventricular septal defect, pulmonary atresia, and major aortopulmonary collateral arteries

BACKGROUND: The purpose of this study was to evaluate the results of various surgical modalities that have been evolving for the treatment of ventricular septal defect, pulmonary atresia, and major aortopulmonary collateral arteries. METHODS: From 1993 to May 1997, 14 patients (group 1) were treated with staged unifocalization through thoracotomies and final repair by midsternotomy. From June 1997 to February 1998, 10 patients (group 2) were treated with midsternotomy, single-stage complete unifocalization, and repair. RESULTS: In group 1, 14 patients had 21 procedures (1.5 procedures per patient), of which 3 patients (21%) had final correction. There were two deaths (14%). One patient died of blocked shunt. Another patient who had aneurysmal dilation of homograft tubes that were used for unifocalization died after final repair because of low cardiac output. In group 2, 10 patients had ten surgical procedures for complete unifocalization and 9 of 10 (90%) of them achieved final correction. One patient with low cardiac output in whom we did not close the ventricular septal defect died (10%) of suprasystemic right ventricular pressure. CONCLUSION: In single-stage complete unifocalization, more patients had final correction. It reduces the number of operations and hospitalization and hence is more cost effective than multistaged procedures.     

Reconstruction of palate with radial forearm flap; a report of 3 cases

A prosthesis is the preferred method for the closure of a palatal defect following maxillectomy, with fairly satisfactory results. However, some patients suffer from symptoms due to mismatch of the prosthesis. We have reconstructed palatal defects using radial forearm flaps in 3 cases. The forearm flap is utilised for palatal reconstruction in the double-folded form. The flap is thin and pliable, there are no problems in regard to bulk and drooping of the flap, and the patients can wear the denture with minimal discomfort. We believe that reconstruction of the palate with a forearm flap is a useful procedure, especially for patients suffering from symptoms due to mismatch of the prosthesis.     

Early radical corrective surgery in congenital deformities of the lateral and posterolateral portion of the vertebrae

The article deals with the techniques of operation for semivertebra extirpation together with one-stage correction of the deformity with a contractor and anterior and posterior fixation of the spine. The operation was performed on 28 children as a result of which the spinal deformity was corrected, on the average, by 75% of the initial deformity in patients with scoliosis and by 50-66% in those with kyphoscoliosis. In 9 patients the initial deformity was corrected by more than 90%. Stable correction of the deformity was achieved in 84% of patients. The poor results (3 cases) were due to the use of a short compressing rod and erroneous choice of the material for spondylodesis. The operation is rekommended in progressing spinal deformities in young children under 3 years of age.     

Obtaining rigidity in total phalloplasty: experience with 35 patients

PURPOSE: The combination of a neourethra and erection prosthesis in a single neophallus in the female-to-male transsexual remains a challenge. The outcome reported in the literature is disappointing. We report our experience with 35 patients. MATERIALS AND METHODS: Between August 1996 and December 2001, 35 patients underwent implantation surgery. A 1-piece hydraulic Dynaflex prosthesis (American Medical Systems, Minnetonka, Minnesota) was used in 10 patients, while a 3-piece hydraulic CXM and CX (American Medical Systems) prosthesis was placed in 9 and 16, respectively. The 1-piece model was withdrawn from the market in 1997. Thereafter a 3-piece prosthesis was implanted. RESULTS: Of 10 patients in the 1-piece group prosthesis implantation was uneventful in 8. In 2 patients with technical failure the prosthesis was replaced, including 1 in whom the new prosthesis was removed due to infection and successfully replaced by a 3-piece prosthesis. To date at a mean followup of 3.5 years 9 patients have a 1-piece hydraulic prosthesis in place. In the 3-piece prosthesis group of 25 patients implantation was uneventful in 20. In 1 patient infection and partial necrosis of the neophallus developed, 2 had infection, in 1 a cylinder perforated the tip of the phallus and in 1 technical failure occurred. Of the latter 4 patients the prosthesis was replaced successfully in 2 patients, while the other 2 are on the waiting list. The patient with partial necrosis of the phallus is no longer a candidate for an erection prosthesis. To date at a mean followup of 1.8 years 23 patients have a 3-piece hydraulic prosthesis in place. A single patient in the 1-piece group has a 3-piece CX prosthesis. CONCLUSIONS: Good results were observed after implantation of the Dynaflex prosthesis in patients who underwent total phalloplasty. This model is no longer available today. For the more complex 3-piece CX and CXM prostheses implantation results are comparable to those of the 1-piece model.     

Does prenatal diagnosis influence the morbidity associated with left in situ nonfunctioning or poorly functioning renal moiety after endoscopic puncture of ureterocele?

PURPOSE: We compared long-term morbidity associated with left in situ nonfunctioning or poorly functioning renal moiety of a duplex system in children with prenatal vs postnatal diagnosis of ureterocele who underwent endoscopic puncture. MATERIALS AND METHODS: A total of 48 children underwent primary endoscopic puncture of duplex system ureterocele. Of the cases 35 (73%) were diagnosed prenatally (group 1) and 13 (27%) postnatally (group 2). Median age at time of puncture was 4 months in group 1 and 3.5 years in group 2. A total of 20 patients in group 1 (57%) and 8 in group 2 (62%) presented with intravesical ureterocele, while 15 in group 1 (43%) and 5 in group 2 (38%) had ectopic ureterocele. A total of 20 children in group 1 (57%) and 7 in group 2 (54%) had a nonfunctioning renal moiety, and 15 in group 1 (43%) and 6 in group 2 (46%) had a poorly functioning ureterocele moiety. Vesicoureteral reflux (VUR) was present in 23 children in group 1 (66%) comprising 30 renal refluxing units (RRUs), and in 12 in group 2 (92%) comprising 14 RRUs. Median followup was 9 years (range 1 to 15) for both groups. RESULTS: Preoperative urinary tract infection (UTI) was common in group 2 (92%) vs group 1 (20%). No patient in group 1 had development of UTI after puncture, while 23% of the children in group 2 presented with UTI. Four children (2 from each group) with ectopic ureterocele required secondary puncture resulting in satisfactory drainage. A total of 14 RRUs (47%) showed spontaneous resolution of VUR in group 1 compared to 3 (21%) in group 2. Four RRUs (13%) required endoscopic correction due to high grade VUR in group 1. Two RRUs (17%) were treated with endoscopic correction and 2 (17%) with ureteral reimplantation due to UTI in group 2. Only 1 patient in group 1 underwent nephrectomy due to nonfunctioning kidney, while 2 patients in group 2 required partial nephrectomy due to UTI. CONCLUSIONS: Our data reveal that prenatal diagnosis of duplex system ureterocele is associated with fewer UTIs, and early endoscopic management may decrease UTI and the need for additional surgery. Nonfunctioning or poorly functioning renal moieties left in situ following successful endoscopic decompression of ureterocele are not associated with additional morbidity and do not require partial nephrectomy in the majority of the cases.     

Soft tissue reconstruction following total knee arthroplasty: treatment possibilities and retrospective analysis of plastic surgery therapy treatment possibilities

AIM: We performed a retrospective analysis of patients with soft tissue defects following total knee arthroplasty and therapy. Furthermore, we described the possibilities of covering soft tissue defects following knee arthroplasty. METHOD: In 5 patients, soft tissue defects following knee arthroplasty were covered with medial M. gastrocneminus flaps. Localisation and size of the defect, microbiology, risk factors, and interval between arthroplasty and the occurrence of the soft tissue defect were retrospectively analysed. RESULTS: On average, defects occurred 9 weeks after prosthesis implantation. In all cases, the soft tissue defect developed in the area of the incision. In 3 cases, wound infection was diagnosed. Four patients exhibited factors associated with wound-healing failure. No prosthesis was lost. CONCLUSION: The gastrocneminus muscle flap provides good quality coverage with small donor site defect, permits early mobilisation, and allows for fast rehabilitation. The risk of flap loss is minimal. Early and adequate defect coverage can reduce both prosthesis loss and amputation rates.