Gilliatt-Sumner hand or true neurogenic thoracic outlet syndrome. A report on seven operated cases

Background and purpose

The clinical picture of hand atrophy related to a cervical rib or elongated C7 transverse process was well described in the modern literature by Gilliatt and Sumner; in 1970, they reported a series of nine patients whose motor status was stabilized following brachial plexus decompression. We report here seven patients suffering from thoracic outlet syndrome (TOS), who developed hand atrophy, sometimes because of diagnostic delay.

Methods

The patient's charts were analysed retrospectively.

Results

The seven patients were all female; the mean age was 43 years. The first complaints were arm pain and paresthesias lasting six months to 5 years. Three patients were treated with C56/C67 discectomy plus disc prosthesis (one patient), ulnar neurolysis at the elbow (the same patient), carpal tunnel release (one patient), and intravenous immunoglobulins (one patient) before TOS diagnosis. Hand atrophy, severe in five patients, was present at presentation. All patients underwent brachial plexus decompression by the anterior (four), posterior (two), or transaxillary (one) approach. This last approach was completed 18 months later by brachial plexus neurolysis via the anterior approach. Postoperatively, motor deficit was improved in two patients and stabilized in five patients.

Conclusions

Physicians’ unfamiliarity with TOS diagnosis or their reluctance to accept the diagnosis without electrical confirmation can lead to hand atrophy. Brachial plexus decompression at this stage usually stabilizes the deficit.     

Portomesenteric venous thrombosis associated with rectal venous malformations

Purpose

We report thrombosis of portal and mesenteric veins in patients with a pattern of rectal venous malformations (VMs) and ectatic major mesenteric veins.

Methods

Eight patients having rectal VMs with either ectatic mesenteric veins and/or evidence of portomesenteric venous thrombosis (PVT), evaluated from 1995-2009, were reviewed.

Results

Portomesenteric venous thrombosis was evident in 5 patients at presentation. Three had patent ectatic mesenteric veins, 2 with demonstrated reversal of flow, and 2 of whom went on to thrombosis during observation. Six patients developed portal hypertension. Five remain on long-term anticoagulation. After recognizing this pattern, one patient underwent preemptive proximal ligation of the inferior mesenteric vein (IMV) to enhance antegrade portal vein flow and prevent propagation or embolization of venous thrombus from the IMV to the portal vein.

Conclusion

Rectal VMs should be evaluated for associated ectatic mesenteric veins. The ectatic vein siphons flow from the portal vein down to the rectal VM, leading to stagnation of blood in the portal vein and resultant thrombosis. Primary thrombosis in the stagnant rectal VM and/or mesenteric vein can also predispose to embolization up into the portal vein. This pattern of rectal VM and ectatic mesenteric vein should be considered a risk factor for devastating PVT.     

Extrahepatic portal vein morphology in children with extrahepatic portal hypertension assessed by 3-dimensional computed tomographic portography: a new etiology of extrahepatic portal hypertension

Background/Purpose

The cause of extrahepatic portal hypertension in children has not been clarified. Our aim was to determine the morphological features of the extrahepatic portal vein in children with extrahepatic portal hypertension by 3-dimensional computed tomographic portography and to clarify the etiology of this disorder.

Materials and Methods

Six patients ranging in age from 10 to 18 years (median age, 12.8 years) who had portal hypertension presented with hematemesis. They underwent intravenous computed tomographic portography using a helical computed tomography scanner and 3-dimensional image reconstruction.

Results

The extrahepatic portal vein was visualized in all patients by 3-dimensional computed tomographic portography. None of the patients showed extrahepatic portal vein obstruction or cavernous transformation. All patients had a tortuous η-shaped extrahepatic portal vein, and a line could be drawn through the flexures of the portal vein to the hepatic hilum.

Conclusion

In children, extrahepatic portal hypertension is not caused by extrahepatic portal vein obstruction and may be of embryological origin.     

Innovative and cost-effective management of large omphalocele

Background/Purpose

The aim of this study was to develop a method of management of large omphalocele, with easily available inexpensive materials. The efficacy of using the plastic of urine collection bag and paper stapler, in creating the “silo” for the management of 3 newborns with such defects, were assessed.

Methods

All operations were done within 36 hours of birth. A silo was created with the plastic of a sterile urine collection bag, which was stapled with a paper stapler at its free margin. The omphalocele was gradually reduced every 24 to 48 hours, using the stapler, until the contents were reduced, when the abdominal wall was repaired.

Results

The mean time taken to close the abdominal defect was 34 days. All patients could be breast-fed from 48 hours after the first stage is done. Rooming in was done by day 7. None of the babies required assisted ventilation.

Conclusion

This method is simple and cost-effective, using minimally expensive, easily available materials.     

Briefing guide study: preoperative briefing and postoperative debriefing checklists in the Veterans Health Administration medical team training program

Background

The purpose of this study was to examine the outcomes of checklist-driven preoperative briefings and postoperative debriefings during the Veterans Health Administration (VHA) medical team training program.

Methods

A briefing score (1, never started; 2, started then discontinued; 3, maintained on original targeted cases; 4, expanded to other services; 5, briefing all cases, all services) was established at 10.1 ± .3 months after introduction of the checklist. Outcomes included antibiotic and deep venous thrombosis prophylaxis compliance rates before and after use of the checklist.

Results

Antibiotic (97.0% ± .1% vs 92.1% ± 1.5%; P = .01) and deep venous thrombosis (95.7% ± .8% vs 85.1% ± 4.6%; P = .05) prophylaxis compliance rates were higher after initiation of a surgical checklist.

Conclusions

Checklist-driven preoperative briefings and postoperative debriefings are associated with improvements in patient safety for surgical patients.     

Lateral semicircular canal fenestration for congenital conductive hearing loss: Solution for a dilemma

Objective

To determine functional results after lateral semicircular canal fenestration on congenital conductive hearing loss.

Study Design

Case series with chart review.

Setting

Amir-Alam otolaryngology tertiary referral center.

Subjects and Methods

Twenty patients with congenital oval window malformations who were not candidates for ossicular reconstruction underwent lateral semicircular canal fenestration. A skin graft was placed over the perforated fascial graft on the fenestrated area.

Results

The median preoperative mean air conduction (MAC) was 56.9 dB (50.0 dB median air-bone). Postoperative median MAC gain of 34.3 dB (P < 0.001) and the median air-bone gap of 18.8 dB were observed. The mean bone conduction (MBC) did not show any significant changes postoperatively (P = 0.12). No sensorineural hearing loss, tinnitus, or imbalance was observed.

Conclusion

We demonstrated hearing improvement after lateral semicircular canal fenestration. This technique can be considered as an alternative for patients with middle ear anomalies who are not candidates for ossicular reconstruction.     

Ethanol lock therapy to reduce the incidence of catheter-related bloodstream infections in home parenteral nutrition patients with intestinal failure: preliminary experience

Background

Catheter-related bloodstream infections (CRBSI) cause morbidity and mortality in patients with intestinal failure dependent on parenteral nutrition. Ethanol lock of central venous catheters may decrease CRBSI, but limited pediatric data are available.

Methods

Home parenteral nutrition patients with at least one previous CRBSI were initiated on a 70% ethanol lock protocol for a minimum of 4 hours. Infection rates (per 1000 catheter days) before and after initiation of ethanol locks were compared using a paired t test.

Results

Ten patients (4 girls; median age, 44 months [range, 31-129 months]) began ethanol lock therapy after a total of 91 CRBSIs (37 gram-positive, 30 gram-negative, and 24 fungal) with a mean of 10.2 ± 6.2 per 1000 catheter days. Patients received ethanol lock for an average of 227 ± 64 days with only 3 CRBSI occurring (CRBSI rate of 0.9 ± 1.8 per 1000 catheter days [P = .005]). Central venous catheter replacements decreased from 5.6 per 1000 days to 0.3 per 1000 days posttherapy (P = .038). Ethanol lock was discontinued in 2 of 10 patients because of catheter thrombosis.

Conclusion

Preliminary results demonstrate a significant decrease in CRBSI with a 70% ethanol lock protocol. Catheter thrombosis may be a limitation that needs to be addressed. With such a dramatic therapeutic effect, a randomized trial is feasible and should be performed.     

Early diagnostic value of plasma PCT and BG assay for CRBSI after OLT

Aim

The aim was to evaluate the role of procalcitonin (PCT) and (1-3)-β-D-glucan (BG) tests for early detection or exclusion of central venous catheter-related bloodstream infections (CRBSI) in patients after orthotopic liver transplantation (OLT).

Methods

Fifty-five patients with clinically suspected CRBSI were assessed after OLT in this prospective study. On the day of clinical suspicion of CRBSI, blood samples were obtained from central venous catheters and a peripheral vein for blood cultures and from a peripheral vein for PCT and BG tests. Plasma PCT and BG values were measured by using an immunoluminometric assay and Fungitell BG assay, respectively. No prisoners or organs from prisoners were used in this study.

Results

Twenty-five patients (45%) were diagnosed with CRBIS. Among them, 13 (52%) displayed gram-positive bacteriemia, 11 (44%) gram-negative bacteriemia, and 1 (4%) fungemia. The PCT values were higher in CRBSI than in non-CRBSI patients (P = .003). CRBSI patients did not show significant increases in plasma BG values compared with non-CRBSI subjects (P = .051). PCT and BG area under receiver operating characteristic curves were 0.840 and 0.486, respectively. Sensitivity, specificity, and positive and negative predictive values of a PCT of ≥3.1 ng/mL for the diagnosis of CRBSI were 0.72, 0.87, 0.82, and 0.79, respectively. The figures for a BG of ≥83 pg/mL were 0.32, 0.90, 0.73, and 0.61, respectively. Among the 24 patients with bacteria infections, PCT was higher in patients with gram-negative than those with gram-positive bacterial infections (P = .022).

Conclusion

We concluded that the PCT assay may be a useful rapid diagnostic adjunct for the diagnosis of suspected CRBSI in OLT patients.     

Thoracic outlet syndrome

Thoracic Outlet Syndromes (TOS) consists of a group of distinct pathologies arising from compression or impingement of structures at the thoracic outlet. The structures at risk are, from anterior to posterior, subclavian vein (venous — VTOS), subclavian artery (arterial — ATOS) and brachial plexus (neurogenic — NTOS). NTOS is the most common presentation, usually caused by compression of the brachial plexus at the scalene triangle or pectoralis minor space. Neurogenic compression syndromes at the carpal and cubital tunnels should be excluded. Management of NTOS is usually conservative, employing physiotherapy and postural exercises, but pain or muscle wasting may be indications for surgery. VTOS is caused by compression of the subclavian vein at the costoclavicular junction, resulting in venous thrombosis (Paget—Schroetter syndrome) often as a result of exercise in fit young muscular people or musicians. Positional swelling of the upper limb without thrombosis is termed McCleery's syndrome. In acute thrombosis, clot lysis, first rib excision and venoplasty may be indicated. ATOS occurs due to compression of the subclavian artery at the scalene triangle, often in association with an anomalous bony structure, such as cervical rib, causing post-stenotic aneurysmal dilation of the artery, thrombosis and distal embolization. Acute upper limb ischaemia necessitates urgent cervical rib excision and arterial reconstruction.     

Computed tomography before transfer to a level I pediatric trauma center risks duplication with associated increased radiation exposure

Introduction

Community hospitals commonly obtain computed tomographic (CT) imaging of pediatric trauma patients before triaging to a level I pediatric trauma center (PTC). This practice potentially increases radiation exposure when imaging must be duplicated after transfer.

Methods

A retrospective review of our level 1 PTC registry from January 1, 2004, to December 31, 2006, was conducted. Level I and II trauma patients were grouped based on whether they had undergone outside CT examination (head and/or abdomen) at a referring hospital (group 1) or received initial CT examination at our institution (group 2). Subgroups were analyzed based on whether duplicate CT examination was required at our PTC (Fischer's Exact test).

Results

A duplicate CT scan (within 4 hours of transfer) was required in 91% (30/33) of group 1 transfer patients, whereas no group 2 patient required a duplicate scan (0/55; P < .0001). There was no significant difference within the groups for weight, age, or intensive care unit length of stay.

Conclusion

A significant number of pediatric trauma patients who receive CT scans at referring hospitals before transfer to our level I PTC require duplicate scans of the same anatomical field(s) after transfer, exposing them to increase potential clinical risk and cost.     

A novel approach for the simultaneous repair of large midline incisional and parastomal hernias with biological mesh and retrorectus reconstruction

Introduction

Patients with concomitant large midline incisional and parastomal hernias present many unique challenges to the reconstructive surgeon.

Methods

We describe a novel approach of simultaneously repairing the midline incisional and parastomal defect, while prophylactically reinforcing the relocated stoma site with a retrorectus biological graft.

Results

During the study period, 9 men and 3 women with a mean age of 65 years, body mass index (BMI) 34 kg/m², and American Society of Anesthesiologists score (ASA) 3.1 underwent repair. Hernia defects averaged 338 cm². Seven patients had a myofascial advancement flap. Mean operative time was 277 minutes. Postoperative complications occurred in 4 patients (33%) and included superficial surgical site infection, transient renal failure, and deep venous thrombosis; in addition, 1 patient died suddenly on postoperative day 3. After a mean follow-up of 14 months, 2 patients have asymptomatic hernia recurrence.

Conclusions

The use of various advanced abdominal wall reconstructive techniques may offer an acceptable approach to repairing these challenging defects.     

Local wound exploration remains a valuable triage tool for the evaluation of anterior abdominal stab wounds

Background

Recent guidelines do not support local wound exploration (LWE) or diagnostic peritoneal lavage (DPL) in the evaluation of patients with anterior abdominal stab wounds (AASWs), favoring computed tomography scanning or serial examinations. In patients without immediate indications for laparotomy, we hypothesized that LWE/DPL would identify patients requiring surgery while limiting unnecessary hospital admissions.

Methods

Patients sustaining penetrating trauma at our level I trauma center over a 3-year period were reviewed.

Results

During the study period, 139 patients with AASW followed our LWE/DPL algorithm. Fifty-six patients had LWE without fascial penetration: 46 were discharged immediately, 10 required admission. Fifty-eight patients had fascial penetration on LWE but negative DPL: 37 were observed for less than 24 hours, 19 were observed for more than 24 hours, and 2 patients developed peritonitis requiring exploration. Twenty-five patients had positive LWE/DPL: 13 had therapeutic laparotomy, 12 had nontherapeutic laparotomy.

Conclusions

Only 11% of patients with AASWs without overt indication for laparotomy require surgical care. LWE remains a valid method to exclude intra-abdominal injury and to eliminate hospitalization in more than one third of AASW patients.     

Pierre Robin sequence: appearances and 25 years of experience with an innovative treatment protocol

Purpose

The aim of the study was to evaluate the largest number of Pierre Robin sequence (PRS) cases to date and its treatment outcome.

Design

This is a retrospective study.

Settings

The study was conducted in a tertiary care university hospital.

Patients

One hundred eighty-eight patients with PRS have been subjected to analysis, defined by the clinical triad of glossoptosis, retro/micrognathia, and cleft or agenesis of the palate, for incidence, risk factors, associated syndromes, other concomitant abnormalities, airway and feeding difficulties, and outcome regarding the neonatal and perinatal surgical and nonsurgical management.

Results

The incidence of PRS was 6.02%. Risk factors were found in 41.5% of mothers with children with PRS. Eleven other syndromes/appearances were associated. Feeding difficulties were seen in 51.8% of the patients. Glossopexy (6.9%) and tracheotomy (2.13%) for airway management were performed very seldom. The suction and drinking plate as well as the surgical treatment protocol may be considered to be the reasons for reduced airway (up to 91.0%), feeding problems (up to 79.9%), and mortality rate (2.1%).

Conclusion

The Pierre Robin sequence, as seen as a heterogeneous group, presents with variation of the cleft palate defects with glossoptosis and concomitant micrognathic mandible. The surgical management and certain prepalatal intervention as nonsurgical management with an innovative treatment strategy were evaluated.     

Total oxidant status and oxidative stress are increased in infants with necrotizing enterocolitis

Background

Oxidative stress has been implicated in the pathogenesis of necrotizing enterocolitis (NEC). In this study, we compared the global oxidant/antioxidant status by measuring total antioxidant capacity (TAC), total oxidant status (TOS), and oxidative stress index (OSI) in preterm infants with NEC and with control preterms.

Methods

Forty-one preterm neonates with NEC (stage 1 [group 1; n = 23] and stages 2 and 3 [group 2; n = 18]) and age-matched 36 healthy preterm controls (group 3) were included in this study. Blood samples were obtained both at the time of NEC diagnosis and 72 hours after for the evaluation of TAC and TOS. Serum levels of TAC, TOS, and OSI in patients with NEC were compared with controls.

Results

Demographic characteristics were comparable in all 3 groups. Preterm neonates in group 2 (with stages 2 and 3 NEC) had the highest TOS levels and OSI (P < .001 vs both groups 1 and 3). There was no difference in TAC levels among the groups (P = .26).

Conclusions

Our findings demonstrated that although TAC levels were similar in all 3 groups, oxidant stress mechanisms were activated in preterm neonates with definite NEC (stages 2 and 3 NEC). Premature neonates with increased levels of TOS and OSI were associated with severity of NEC.     

The catheter is stuck: complications experienced during removal of a totally implantable venous access device. A single-center study in 200 children

Background

Totally implantable venous access devices (TIVAD) facilitate repeat intravenous therapy for children. Many children recover and the device may be removed. Although removal should be a simple procedure via a single incision, in our experience, this has not been the case.

Methods

Two hundred consecutive cases of removal of TIVAD from September 2000 to January 2004 at Sophia Children's Hospital, Rotterdam, were reviewed.

Results

Average patient age was 5.9 years. The commonest indication for placement was administration of chemotherapy (88%); commonest indication for removal was remission of disease (70%). The median duration in situ of the catheter was 29 months (range, 0.4-91 months). Complications with removal of the polyurethane catheter of the TIVAD were experienced in 16% of cases. To enable removal, a second incision was required in 28 patients, venotomy in 5; the catheter could not be removed in 3. For all complicated removals the catheter had been in situ for longer than 20 months.

Conclusions

Long-term implantation of TIVAD with polyurethane catheter appears unsuitable owing to a high incidence of complication at time of removal.     

Complications of recombinant activated human coagulation factor VII

Background

Recombinant factor VIIa (rFVIIa) frequently is used for treatment of life-threatening hemorrhage in trauma.

Methods

A retrospective review of injured patients receiving rFVIIa at an American College of Surgeons-verified Level 1 trauma center was performed. Controls were matched for age, sex, Injury Severity Score, and traumatic brain injury. Thrombotic complications in patients administered rFVIIa, including deep venous thrombosis (DVT), pulmonary embolus, acute myocardial infarction, ischemic stroke, mesenteric ischemia, arterial thromboembolism, and death, were determined.

Results

Thirty-six patients were given rFVIIa, of whom 5 (13.8%) had thrombotic complications. Indications for rFVIIa were life-threatening intracranial bleeding in the presence of pre-injury anticoagulation or hemorrhage. The incidences of DVT (n = 4) and acute myocardial infarction (n = 1) were noted. In the control group, there were fewer thrombotic complications (DVT, 1; pulmonary embolus, 1). The mortality rate (52.8%) was higher in patients receiving rFVIIa compared with the control group (22.2%; P = .014). Pre-injury anticoagulation was common in the treatment group.

Conclusions

Pre-injury anticoagulation is frequently the indication for rFVIIa administration. Thrombotic complications occur with rFVIIa administration. The mortality rate of injured patients who receive rFVIIa is high.     

Surgical treatment in Paget's disease of the breast

Background

The aim of this study was to evaluate the outcomes of surgical treatment of Paget's disease of the breast, with special emphasis on magnetic resonance imaging (MRI) and sentinel node biopsy (SNB).

Methods

The study included 58 consecutive patients with Paget's disease treated from 1995 to 2006.

Results

Twenty-five patients had ductal carcinoma in situ, and 31 had invasive carcinoma. MRI was performed in 14 patients, with positive findings in 7 patients, 5 of whom had negative findings on conventional imaging. The overall mastectomy rate was 76%. Eighteen patients underwent SNB, and 26 patients underwent full or partial axillary clearance. Fourteen patients had no axillary surgery. One patient had local recurrence after breast conservation, and another had axillary recurrence after negative results on SNB. Six patients had distant metastases. Four patients died of breast cancer.

Conclusions

Paget's disease is frequently associated with peripheral or multicentric cancer. MRI may be helpful when considering breast conservation or omitting axillary nodal staging.     

Thoracic outlet syndrome: Fact or fancy? A review of 409 consecutive patients who underwent operation

Objectives

From an analysis of patients operated on by a single surgeon between 1973 and 1994 for thoracic outlet syndrome (TOS), to determine what findings are helpful in substantiating this diagnosis and what are the results of decompressive thoracic outlet surgery in the management of TOS.

Design

A chart review with emphasis on signs, symptoms and results. A preoperative questionnaire was presented to all patients operated on since 1989. Postoperative follow-up was by interview or telephone.

Setting

A 560-bed university-affiliated tertiary-care teaching hospital.

Patients

There were 409 adults; 83% were women and the average age was 36 years. In 95% of patients physiotherapy had been unsuccessful. Patients presented with neurologic type (368 [(90%]), arterial impingement (29 [7%]) and venous obstructive symptoms (12 [3%]). The series included 44 patients with bilateral symptoms and 26 patients with cervical ribs. Some form of litigation or compensation was associated with 177 patients.

Interventions

Transaxillary first rib resection in 380 (93%) patients and supraclavicular thoracic outlet decompression in 29 (7%). In 244 patients there was a follow-up of at least 2 years. These patients constituted the follow-up group.

Results

Preoperatively, in the 368 patients who had neurologic TOS, 99% displayed supraclavicular brachial plexus tenderness and 98% exacerbation of symptoms with arms in the abduction external rotated position. There were eight complications of surgical intervention (1.9%). In the follow-up group, there were no deaths, no subclavian/axillary artery or vein damage and no brachial plexus injury. Seventy-eight percent of the patients with neurologic TOS in this group improved postoperatively; 21% had complete relief, 32% had good relief and 25% had fair relief. Twenty-two percent showed no improvement.

Conclusions

Signs and symptoms helpful in making the diagnosis of neurologic TOS are supraclavicular tenderness on palpation and exacerbation of symptoms with the arms in the abducted external rotated position. The surgical procedures are safe. Patients with TOS refractory to medical management can benefit from thoracic outlet decompression.     

Incidence, management, and results of vascular complications after liver transplantation

Introduction

Vascular complications show an 8%-15% incidence after liver transplantation and represent an important cause of mortality. An aggressive policy is necessary for an early diagnosis and treatment.

Patients and methods

From 2001 to 2009, we performed 240 liver transplantations in 232 patients. We employed Doppler ultrasonography on days 1 and 4 as well as before hospital discharge and always try a radiological approach.

Results

The incidence of vascular complications was 7.2% (n = 18) including arterial (n = 12, 4.8%) of early thrombosis (n = 4), late thrombosis (n = 4), and stenosis (n = 4) or portal (n = 3; 1.2%) of thrombosis (n = 2) or stenosis (n = 1); or caval complications (n = 3, 1.2%). Radiologic therapy was effective in 1 patient with arterial stenosis, in the 3 patients with portal complications, and in 2 patients with caval complications. All patients with early thrombosis and 2/4 with late thrombosis required retransplantation. Surgical treatment was effective in 1 patient with late thrombosis, 3 with stenosis, and 2 with caval complications. The overall mortality rate was 16.6%; 2 patients with arterial complications and 1 with a caval complications.

Conclusion

Vascular complications, mainly artery complications, represent serious problem after liver transplantation, which often requires retransplantation. With an aggressive policy of diagnosis and treatment, we can decrease the mortality rate from these adverse events.