Growth hormone deficiency,aortic dilation,and neurocognitive issues in Feingold syndrome 2

We report three patients with Feingold 2 syndrome with the novel features of growth hormone deficiency associated with adenohypophyseal compression, aortic dilation, phalangeal joint contractures, memory, and sleep problems in addition to the typical features of microcephaly, brachymesophalangy, toe syndactyly, short stature, and cardiac anomalies. Microdeletions of chromosome 13q that include the MIR17HG gene were found in all three. One of the patients was treated successfully with growth hormone. In addition to expanding the phenotype of Feingold 2 syndrome, we suggest management of patients with Feingold 2 syndrome include echocardiography at the time of diagnosis in all patients and consideration of evaluation for growth hormone deficiency in patients with short stature.     

Heparinoids: structure, biological activities and therapeutic applications.

Heparin is an important polyanionic drug having a wide variety of different biological activities. Substantial research effort has focused on the preparation of improved heparins and heparin analogues that might exhibit higher specificity and decreased side effects. These heparin analogues or heparinoids include sulfated polysaccharides from plant and animal origin, synthetic derivatives of polysaccharides, and acidic oligosaccharides and their small synthetic analogues. The structure, biological activities and therapeutic potential of these heparinoids are discussed.     

Successful repair of a large aortic abscess causing apical displacement of the anterior mitral valve

Anatomic continuity between the anterior mitral leaflet and the aortic root may predispose those patients with aortic root pathology to functional changes of the mitral valve without any involvement of this valve. A 34-year-old man presented with aortic valve endocarditis. Transthoracic echocardiograpy showed severe aortic regurgitation with a large aortic root abscess. The anterior leaflet of the mitral valve was displaced towards the apex of the heart causing moderate mitral regurgitation. The patient underwent aortic valve replacement with reconstruction of the aortic annulus and ventriculoaortic continuity. This procedure alone restored the mitral valve structure and function without any need for intervention on the mitral valve. Aortic abscess is a serious complication of aortic valve endocarditis and may alter the function of other structures of the heart, especially the mitral valve. Restoration of aortic wall integrity and left ventricular – aortic continuity usually restores the mitral valve structure and function if the valve is unaffected by the infection. A decision on the mitral valve should be made following correction of the aortic pathology.     

Anatomical variations of donor portal vein in right lobe living donor liver transplantation: the safe use of variant portal veins

In right lobe (RL) living donor liver transplantation (LDLT), portal vein (PV) variations are of immense clinical significance. In this study, we describe in detail our PV reconstruction techniques in RL grafts with variant PV anatomy and evaluate the impact of accompanying biliary variations on the recipient outcomes. In a total of 386 RL LDLTs performed between July 2004 and July 2012, the clinical data on 52 (13%) transplants using RL grafts with variant PV anatomy were retrospectively analyzed. Portal vein anatomy was classified as type 2 in 20 patients, type 3 in 24 patients, and type 4 in eight patients. The PV reconstruction techniques utilized included back‐wall plasty (n = 21), back‐wall plasty with saphenous vein graft interposition (n = 6), saphenous vein graft interposition (n = 5), cryopreserved iliac vein Y‐graft interposition (n = 6), and quiltplasty (n = 3). There was no donor mortality. In a median follow‐up of 29 months, none of the recipients had vascular complications. Anomalous PV anatomy was associated with a high (54%) incidence of biliary variations; however, these variations did not result in increased biliary complication rate. Overall, the 1‐ and 3‐year patient survival rates of recipients were 91% and 81%, respectively. Vascular and biliary variations in RL grafts render LDLT technically more challenging. By employing appropriate reconstruction techniques, it is possible to successfully use RL grafts with PV variations without endangering recipient and donor safety.     

Facial defects restored with extraoral implant-supported prostheses

A facial defect results in cosmetic, functional, and psychologic problems and acts as a difficult and challenging procedure for the maxillofacial surgeon and the prosthodontist. Retention of a facial prosthesis is an important key in the patient's satisfaction and acceptance. The introduction of extraoral implant applications have been accepted as an advanced retention method. The purpose of this article is to report the results from our clinical experience with patients wearing facial prostheses anchored to extraoral ITI implants (Straumann, AG, Basel, Switzerland).     

Use of Stapling Devices for Safe Cholecystectomy in Acute Cholecystitis

Many techniques are described for the ligation of a difficult cystic duct (CD). The aim of this study is to assess the effectiveness and safety of stapling of a difficult CD in acute cholecystitis using Endo-GIA. From January 2008 to June 2012, 1441 patients with cholelithiasis underwent laparoscopic cholecystectomy (LC) at the Department of General Surgery, Haydarpasa Numune Education and Research Hospital. Of these, 19 (0.62%) were identified as having a difficult CD and were ligated using an Endo-GIA stapler. All patients were successfully treated with a laparoscopic approach. The length of hospital stay was 3.4 days. There were umbilical wound infections in 4 patients (21%). The length of follow-up ranged from 1.0 to 50.4 months. In conclusion, Endo-GIA is a safe and easy treatment method for patients with a dilated and difficult CD. The cystic artery should be isolated and ligated if possible before firing the Endo-GIA stapler. If isolation and stapling are not possible, fibrin sealant can be applied to avoid bleeding. The vascular Endo-GIA can be applied in a large CD, but for acute cholecystitis with an edematous CD, the Endo-GIA roticulator 4.8 or 3.5 stapler is preferred.Key words: Cholecystectomy, Endo-GIA, Acute cholecystitis, StaplerAfter the introduction of laparoscopic cholecystectomy (LC) in 1987,¹ LC replaced open cholecystectomy as the gold standard for the treatment of cholelithiasis in international guidelines.² LC was initially considered to be contraindicated for acute gallbladder inflammation, but it is currently a common procedure for acute cholecystitis.Some of the difficult situations a surgeon is likely to face during the performance of a laparoscopic cholecystectomy include anatomic anomalies such as a sessile gallbladder or short cystic duct and pathologic entities such as an empyema, Mirizzi syndrome, or a frozen Calot''s triangle secondary to infection and fibrosis.³It is suggested that laparoscopic surgery should be carried out within 72 hours from the onset of the symptoms because after that time there are higher rates of conversion to open procedures, increased risks of complications, and longer operative times.^4–6 The generally accepted procedure in patients whose symptoms started 72 hours before admission is to “cool down” the patient with appropriate medical therapy and to perform LC after a period of 6 to 12 weeks.^7,8 This approach aims to avoid a potentially more difficult cholecystectomy during an emergency admission and to avoid the difficulties of access to an emergency room.^9,10 However, more than 20% of patients may fail to respond to conservative treatment and require an urgent and rather more difficult cholecystectomy, and a further 25% of patients will require readmission with a severe acute complication of cholelithiasis while awaiting a cholecystectomy.^11,12 The scar formation, distortion, and organized adhesions around the gallbladder occurring secondary to the chronic inflammation in Calot''s triangle make the dissection difficult. The cystic duct (CD) is sometimes edematous, fibrous, or enlarged owing to inflammation and adhesions in acute cholecystitis and may be difficult to manage. Several methods were proposed for ligating the CD, including titanium or absorbable endoclip, endoloop, tie, ultrasonic or bipolar sealer, and the Endo-GIA stapler (Covidien, Mansfield, Massachusetts).^13–19This study proposes an effective, safe, and easy procedure for the stapling of dilated or difficult CD using the Endo-GIA.     

An unusual presentation of perforated appendicitis in epigastric region

INTRODUCTION

Atypical presentations of appendix have been reported including backache, left lower quadrant pain and groin pain from a strangulated femoral hernia containing the appendix. We report a case presenting an epigastric pain that was diagnosed after computed tomography as a perforated appendicitis on intestinal malrotation.

PRESENTATION OF CASE

A 27-year-old man was admitted with a three-day history of epigastric pain. Physical examination revealed tenderness and defense on palpation of epigastric region. There was a left subcostal incision with the history of diaphragmatic hernia repair when the patient was 3 days old. He had an intestinal malrotation with the cecum fixed at the epigastric region and the inflamed appendix extending beside the left lobe of liver.

DISCUSSION

While appendicitis is the most common abdominal disease requiring surgical intervention seen in the emergency room setting, intestinal malrotation is relatively uncommon. When patients with asymptomatic undiagnosed gastrointestinal malrotation clinically present with abdominal pain, accurate diagnosis and definitive therapy may be delayed, possibly increasing the risk of morbidity and mortality.

CONCLUSION

Atypical presentations of acute appendicitis should be kept in mind in patients with abdominal pain in emergency room especially in patients with previous childhood operation for diaphragmatic hernia.     

Results of the probe technique for transatrial repair of tetralogy of Fallot

OBJECTIVE: Total correction of classical tetralogy of Fallot (TOF) by transatrial approach has become a standard procedure in the goal to minimize structural damage to the pulmonary pump. The most critical point in transatrial repair of TOF is infundibular dissection. Right atrial approach provides better surgical exposure for parietal extension of the infundibular septum when compared to the right ventricular approach. However it is not always easy to determine the localization and amount of muscle bundles to be resected and this surgical maneuver requires experience. METHODS: Nineteen patients who had repair of isolated TOF using this technique from 1993 to 2001 were reviewed. The mean age of patients were 5 +/- 2 years. Transatrial-transpulmonary approach were performed for all patients. To make easier the infundibular muscle bundles resection and to determine the localization and amount of muscle bundle to be resected, we placed a Hegar dilator into the right ventricle through pulmonary arteriotomy. The muscle bundles between the dilator and the anterior leaflet annulus of the tricuspid valve were totally excised until the intraventricular part of the dilator and pulmonary annulus became completely visible. The area between the Hegar dilator and the margins of the ventricular septal defect (VSD) was left untouched. None of the patients had transannular patch. To improve exposure, tricuspid valve detachment was performed in 11 patients. All patients were followed-up in our clinic every 6 months using echocardiography. RESULTS: There were no early or late deaths, and no reoperation for residual VSD or residual right ventricle (RV) outflow obstruction. All patients were in NYHA Class I. RV on the echocardiography was spared late dilatation and had a good late functional status. Eighteen patients had no or mild pulmonary regurgitation. One patient who had undergone tricuspid anterior leaflet detachment showed mild tricuspid insufficiency. CONCLUSION: On the basis of hemodynamic outcomes, this procedure for elective repair of TOF in selected cases shows excellent early and mid-term results.     

Pedicled LIMA graft to the posterior coronary artery: which side to be anastomosed,dorsal or ventral?

It has not clearly been defined in the literature which side of the pedicled LIMA graft should be anastomosed to the posterior coronary arteries. Using the ventral side of pedicled LIMA graft causes an 180 degree torsion of the pedicle. Sometimes this torsion may cause reduction or cessation of LIMA flow as we have seen in two of our patients. In this paper we point out that using the dorsal side of the pedicled LIMA graft for anastomosis to the posterior coronary arteries is helpful.