Association of hiatus hernia with asplenia syndrome

During a 13-month period, 13 patients with asplenia syndrome were evaluated with MRI for cardiovascular and visceral anomalies. The MR images were reviewed for the presence of hiatus hernia which was found in three patients. One of the remaining ten patients with no MRI evidence of hiatus hernia was diagnosed as having gastro-oesophageal reflux and hiatus hernia by an oesophagogram and 24-h pH monitoring. This patient had undergone fundoplication prior to MRI. Out of the 13 patients (31%) with asplenia syndrome, 4 had hiatus hernia. It appears that among patients with the asplenia syndrome, hiatus hernia is a frequent finding. Recurrent pneumonia or bronchiolitis in patients with asplenia syndrome requires evaluation for the presence of hiatus hernia and gastro-oesophageal reflux.     

The safe use of intraoperative transesophageal echocardiography in the management of total anomalous pulmonary venous connection in newborns and infants: a case series

BACKGROUND: Intraoperative transesophageal echocardiography (TEE) is useful in evaluating the repair of lesions in patients with congenital heart disease. But the use of TEE in infants with total anomalous pulmonary venous connection (TAPVC) remains unclear. We reviewed the safety and efficacy of intraoperative TEE during TAPVC repair. METHODS: Twenty-eight consecutive 1 day to 7 month-old infants with TAPVC (14 supracardiac, six intracardiac and eight infracardiac type) had surgical repair with intraoperative TEE monitoring. RESULTS: Four patients received immediate surgical revision after primary surgery for residual anastomotic stenosis diagnosed by TEE. In addition, two unsuspected ventricular septal defects and three persistent ductus arteriosus were detected before surgery. Eight infants (29%) had hypotension and hypoxemia associated with TEE probe insertion before surgery, but this hemodynamic disturbance returned to baseline value after withdrawing the TEE probe from the esophagus. However, these eight patients had uneventful TEE probe insertion following sternotomy. The mechanism was probably because of the reduction of intrathoracic pressure when the chest was opened. CONCLUSIONS: TEE probe insertion in TAPVC patients may pose a potential risk of compression of pulmonary venous confluence resulting in hemodynamic instability. Therefore, we suggest that the use of TEE in such TAPVC patients appears to be safer after sternotomy.     

Combined external urethral bulking and artificial urinary sphincter for urethral atrophy and stress urinary incontinence

OBJECTIVE: To describe a technique of externally bulking the urethra with a soft-tissue graft before placing another artificial urinary sphincter (AUS), as when placing another AUS for recurrent male stress urinary incontinence (SUI) other manoeuvres, e.g. placing a tandem cuff or transcorporal cuff, must be used to obtain urinary continence in an atrophic urethra, and each is associated with morbidity. PATIENTS AND METHODS: From January 2003 to July 2004, five patients (mean age 74 years, range 62-84) treated by radical prostatectomy were referred for recurrent SUI after placing an AUS (four, including one with urethral erosion) or a male sling (one, with a resulting atrophic urethra). Each patient was treated with an external urethral bulking agent (Surgisis) ES, Cook Urological, Spencer, Indiana) and had an AUS placed. RESULTS: In each patient the greatest urethral circumference was <4 cm. To place a functional 4 cm cuff, the diameter of the urethra was enhanced by wrapping it with Surgisis ES. Continence was significantly improved in all patients except one 84-year-old man who had the replanted artificial sphincter removed because of erosion 14 months after surgery. CONCLUSION: In cases of severe recurrent SUI from urethral atrophy after placing an AUS, externally bulking the urethra with Surgisis ES before placing another AUS is well tolerated, and gives satisfactory results.     

The additive erectile recovery effect of brain-derived neurotrophic factor combined with vascular endothelial growth factor in a rat model of neurogenic impotence

OBJECTIVE: To test the hypothesis that combined intracavernosal injection with vascular endothelial growth factor (VEGF) with adeno-associated virus-mediated brain-derived neurotrophic factor (AAV-BDNF) synergistically facilitates the neural regeneration and erectile function after cavernosal nerve injury. MATERIALS AND METHODS: Forty Sprague-Dawley male rats were randomly divided into five equal groups: eight had a sham operation while 32 had bilateral cavernosal nerve freezing followed by an immediate intracavernosal injection with either phosphate-buffered saline (PBS), VEGF, AAV-BDNF, or AAV-BDNF + VEGF. Erectile function was assessed by cavernosal nerve electrostimulation at 3 months, and samples of the major pelvic ganglia and penile tissue were evaluated histologically. RESULTS: In this animal model of impotence from nerve injury, the recovery of erectile function was greatest in those receiving AAV-BDNF + VEGF; the mean (sd) maximal intracavernosal pressure in this group was 87.2 (20.78) cmH2O, compared with 37.3 (11.39) for VEGF alone and 49.8 (29.58) for AAV-BDNF alone. No erectile dysfunction was identified in the sham group, with a pressure of 100.7 (22.70) cmH2O, while all treatment groups significantly outperformed the PBS (control) group, at 29.3 (13.52) cmH2O. Furthermore, all animals receiving monotherapy or combined treatment had more NADPH-diaphorase-positive nerve fibres than controls but less than in the sham group. CONCLUSION: Bilateral cavernosal nerve freezing causes erectile dysfunction with accompanying neurological changes. Intracavernosal injection with either VEGF or AAV-BDNF alone enhances nerve regeneration, with combined therapy (VEGF and AAV-BDNF) promoting neural and erectile recovery additively.     

Lipofibromatous hamartoma of the median nerve with long-term follow-up

Lipofibromatous hamartoma is a rare, benign tumor that most often involves the median nerve. A 16-year-old male with lipofibromatous hamartoma of the median nerve at the wrist level is described. This patient was a child when the mass was first noted. Although there were no symptoms or signs of carpal tunnel compression, the growth of the tumor was progressing. In addition to the release of the carpal tunnel, microsurgical intraneural dissection was done to preserve the thenar motor branch. Then segmental excision of the residual sensory component with sural nerve grafting was performed. Subjectively the patient did not notice the minor motor deficit, however, the patient did experience numbness of fingertips after surgery. There were no scars or trophic ulcers on fingertips at 3 years of follow-up regardless of the inadequate sensory return. Treatment of this benign tumor is still controversial. The relevant reports in the literature are reviewed.     

Congenital radioulnar synostosis treated using a microvascular free fasio-fat flap

Congenital radioulnar synostosis, which is caused by fusion between the proximal end of the radius and ulna, is an uncommon deformity of the upper extremity. It is characterized by the forearm being fixed in some degree of pronation and patients present with a variety of functional limitations of the involved limb. This condition may lead to difficulties in daily activities, such as writing, eating, and accepting objects in an open palm. Many operative procedures have been developed to mobilize the fixed forearm but the long-term results are disappointing. Herein, we reported a 12-year-old female patient with congenital radioulnar synostosis of the bilateral forearm who was treated with separation of the bony bridge between the left radius and ulna combined with a free groin flap transfer into the separated space. The functional results are good and no recurrent ankylosis occurred during a period of 28 months postoperatively. The flap size and volume of the subcutaneous fat are the key factors for satisfactory results.     

Toward normal perfusion after radiosurgery: perfusion MR Imaging with independent component analysis of brain arteriovenous malformations

BACKGROUND AND PURPOSE: Brain perfusion is disturbed by cerebral arteriovenous malformations (AVMs). Our study was conducted to determine the radiosurgical effects on this disturbed perfusion. METHODS: MR perfusion imaging with independent component analysis was performed in five healthy subjects and 19 patients with AVM before and after radiosurgery (every 6 months up to 2 years). Perfusion map relative cerebral blood volume (rCBV), cerebral blood flow (rCBF), and mean transient time (rMTT) were assessed. Regions of interest (ROIs) on AVM target sections were defined as follows: N, AVM nidus; H, the rest of the ipsilateral hemisphere; P, immediately posterior to the nidus; A, immediately anterior to the nidus; Ar, anterior remote; Pr, posterior remote. Similar ROIs in the contralateral hemisphere (N1, H1, P1, A1, Pr1, and Ar1) served as internal references. Perfusion ratios of ROI-ROI1 were defined. Nonparameteric Mann-Whitney U tests and generalized linear models were used for statistical analysis. RESULTS: Before radiosurgery, patients' H/H1 rCBV and rCBF ratios were significantly higher than those of healthy subjects (P < .005), indicating AVM steal. Three types of perilesional perfusion disturbance were observed. From the first postradiosurgical follow-up at 6 months, N/N1 rCBV and rCBF ratios gradually decreased to 1.0 (both P < .001), whereas rMTT ratios gradually increased to 1.0 (P < .015); H/H1, A/A1, and P/P1 rCBV and rCBF ratios decreased after radiosurgery (P < .005), indicating reversal of steal toward normal perfusion. CONCLUSION: Initial high transnidal flow and perinidal perfusion disturbances were demonstrated. They gradually changed toward normal perfusion after radiosurgery. This explains, in part, the pathophysiologic factors of AVM and therapeutic effects.     

Sixty-five clinical cases of free tissue transfer using long arteriovenous fistulas or vein grafts

Traumatic limb injuries requiring free tissue transfer for coverage, often lack healthy recipient vessels adjacent to the defect. In these patients, vein grafts are required to bridge the gap of either the artery, vein or both. For the latter situation, a temporary arteriovenous fistula (AVF) can be created and allowed to mature and then divided and used as recipient artery and veins for the free flap. These cases are challenging and several variables including vein graft length, vein graft diameter, and arterial inflow affect the patency of the vessels and the final outcome of the reconstruction. Sixty-five defects were reconstructed with free tissue transfers using vein grafts of significant length (>20 cm for the arterial gap). The ipsilateral or contralateral great saphenous veins were used for vessel lengthening in all cases. Inflow arteries were either major arteries (superficial femoral, popliteal or brachial), or lesser arteries (sural, anterior or posterior tibial, thoracodorsal, or superior gluteal). The patients were divided into those that underwent AVF followed by free tissue transfer in two stages (n = 6), AVF followed by free tissue transfer in one stage (n = 28) and patients that underwent vein grafting for the arterial defect only with (n = 6) or without (n = 25) a simultaneous bypass graft for lower limb revascularization. In the two-stage AVF group, the rate of occlusion of the graft after AVF creation was 50% (3/6); re-exploration rate was 33.3% (2/6); free flap failure rate was 33.3% (2/6); and limb salvage rate was 83.3% (5/6). In the one-stage AVF group: re-exploration rate was 28.6% (8/28); free flap success rate was 89.3% (25/28); and limb salvage rate was 92.9% (26/28). In the long vein graft group for arterial defects only: re-exploration rate was 25.8% (8/31); free flap success rate was 96.8% (30/31); and limb salvage rate was 87.1% (27/31). In patients where the graft was anastomosed to a major artery the re-exploration rate and free flap failure rate were 22.4% (11/49) and 8.2% (4/49). In patients where the graft was anastomosed to a lesser artery, the re-exploration rate and free flap failure rate were 43.4% (7/16) and 12.5% (2/16). The limb salvage rate was comparable in both groups (89.8%, 44/49, versus 87.5%, 14/16). In all groups, patients undergoing re-exploration were noted to have a an arterial gap of 31.78 cm as compared with the patients that did not require re-exploration which had an arterial gap of 26.26 cm. Vein grafting for bridging vascular defects is a safe procedure when proper indications and techniques are followed. Although a longer graft length seemed to be associated with a higher re-exploration rate, there was no statistical significance. One-stage AVFs can be used with good results, however, two-stage AVFs are associated with a high graft occlusion rate, wound failure rate and limb amputation rate. In all cases, a large caliber graft such as the great saphenous vein provided a large (relatively low resistance) conduit for bridging the defect.     

Extended trapezius myocutaneous free flap for the reconstruction of a foot defect lacking adjacent recipient vessels

Foot reconstruction requires tissue that is durable and can withstand the extremes of pressure and stress. The trapezius myocutaneous flap has not been used previously as a free flap for foot reconstruction. In this report, the trapezius was used as an extended myocutaneous free flap for the reconstruction of a foot wound lacking adjacent and adequate recipient vessels. The extended trapezius flap may be one of the longest free flaps that can be harvested. The indications for the use of this flap are limited. In an extremity that lacks adequate recipient vessels adjacent to the defect, this flap can be extended such that more proximal vessels in the leg can be used as the recipient vessels without the need for vein grafts to bridge the distance. The donor-site morbidity of this flap is minimal when the superior fibers of the trapezius muscle and its innervation are preserved.     

Immediate toe transfer following index finger amputation for extensive giant cell tumor of the tendon sheath with intraosseous invasion

Giant cell tumor of the tendon sheath (GCTTS) is the second most common benign tumor of the hand. Although bony indentation from external compression by the GCTTS is frequently seen on x-ray film, the intraosseous invasion is relatively rare and is a sign for high recurrence. We present a woman with extensive GCTTS located in the left index finger at the level of distal interphalangeal joint. X-ray films revealed multiple osteolytic cystic cavities in the shaft of the middle phalanx. Amputation of the index finger at the base of the middle phalanx was performed because of extensive bony involvement and concern about possible recurrence from inadequate excision. Her left second toe was transferred to replace the amputated index finger in the same session. Follow-up examination at 15 months postoperative revealed good function and appearance of the reconstructed index.