Frank-Martin Haecker

Near-fatal bleeding after transmyocardial ventricle lesion during removal of the pectus bar after the Nuss procedure.

a rib segment to an intact portion of the same or adjacent ribs. Our patient’s injuries precluded other described techniques owing to the lack of intact structures to anchor stabilizing components. Our novel use of Nuss bars enabled us to create a viable superstructure to suspend the chest wall and allow full re-expansion of the lung. This technique will be useful in patients whose severity of injury precludes stabilization by previously described methods. We corresponded with Dr Donald Nuss for his thoughts. He wrote:

Pectus excavatum repair according to Nuss: is it safe to place a retrosternal bar by a transpleural approach, under thoracoscopic vision?

We present a new case of cardiac perforation during retrosternal dissection beginning a Nuss procedure for pectus excavatum repair in an 18-year-old boy. The true incidence of life-threatening complications, such as heart injuries during Nuss bar placement for pectus excavatum repair, as well as cardiac lacerations during removal of the bar, remains unknown. Many papers suggest measures to prevent these complications: approaching the retrosternal space through an additional subxiphoid short incision, lifting the sternum during bar placement, or placing the bar extrapleuraly. Nuss procedure is gaining more and more popularity due to its apparent simplicity; however, its operational complications should be well known and discussed to be avoided later on.

Minimally Invasive Repair of Pectus Excavatum (MIRPE) - The Basel Experience

PURPOSE Minimally invasive repair of pectus excavatum (MIRPE) was first reported in 1998 by D. Nuss. This technique has gained wide acceptance during the last 4-5 years. In the meantime, some modifications of the technique have been introduced by different authors. Our retrospective study reports our own experience over the last 36 months and modifications introduced due to a number of complications. METHODS From 3/2000 to 3/2003, 22 patients underwent MIRPE. Patients median age was 15.5 years (10.7 to 20.3 years). Standardised preoperative evaluation included 3D computerised tomography (CT) scan, pulmonary function tests, cardiac evaluation with electrocardiogram and echocardiography, and photo documentation. Indications for operation included at least two of the following: Haller CT index > 3.2, restrictive lung disease, cardiac compression, progression of the deformity and severe psychological alterations. RESULTS In 22 patients (2 girls, 20 boys) undergoing MIRPE procedure, a single bar was used in 21 patients and two bars in one boy. Lateral stabilisers were fixed with non resorbable sutures on both sides. Overall, postoperative complications occurred in six patients (27.3%). In two patients (9.1%) a redo-procedure was necessary due to bar displacement. An additional median skin incision was performed in two patients to elevate the sternum. Pneumothorax or hematothorax in two patients resulted in routine use of a chest tube on both sides. Long-term favourable results were noted in all patients. CONCLUSIONS The MIRPE procedure is an effective method with elegant cosmetic results. Modifications of the original method help to decrease the complication rate and to accelerate acquirement of expertise.

Intraoperative Use of the Vacuum Bell for Elevating the Sternum During the Nuss Procedure

OBJECTIVE To evaluate the routine use of the vacuum bell for elevating the sternum during minimally invasive repair of pectus excavatum (MIRPE) (the Nuss procedure). SUBJECTS AND METHODS This was a retrospective evaluation of a prospective database including all patients who underwent MIRPE at our institution between 2005 and 2010. Data included the patients demographic characteristics, age at surgery, gender, Haller index, duration of surgery, and intraoperative complications. RESULTS Fifty patients from 9 to 28 years old (average, 14.95 years) were observed, including 39 males and 11 females. The preoperative Haller index was between 3.25 and 7.4 (average, 5.05). Mean duration of surgery was 58 minutes (range, 45-92 minutes). The use of the vacuum bell led to a clear elevation of the sternum as confirmed by thoracoscopy. Advancement of the pectus introducer and placement of the pectus bar were safe, successful, and uneventful in all patients. No cardial and/or pericardial lesion was noted as well as no lesion of the mammary vessels. CONCLUSIONS The intraoperative use of the vacuum bell during the MIRPE is safe and effective as it facilitates the retrosternal dissection and the insertion of the pectus bar. If available, we recommend the routine use of this device for MIRPE.

Laparoscopy-assisted single-port appendectomy in children: is the postoperative infectious complication rate different?

AIM In childhood, laparoscopy-assisted single-port appendectomy (SPA), including the advantages of open and laparoscopic surgery, is not widely used. However, there is debate whether the retrieval of the infected appendix via the umbilicus results in a higher infection rate compared with other laparoscopic or open techniques. The aim of the study was to determine the postoperative infection rate and possible risk factors for infection after SPA in children. METHODS For this retrospective study, case notes of all children (n = 262) who underwent SPA between August 2005 and December 2008 were reviewed. Those children in whom the preoperative ultrasonography revealed suspected perforation were excluded from SPA and subsequently underwent open surgery. SPA was performed using a 12-mm trocar with one 5-mm working channel, introduced through a sub-umbilical incision. After grasping the appendix with atraumatic forceps, the appendix was exteriorized through the umbilicus and dissected outside the abdominal cavity as in open surgery. Preoperatively, each patient received one dose of Metronidazole and Cefuroxime, and the umbilicus was cleaned in particular. RESULTS Of the 262 children who underwent SPA, 146 were boys (55.7%) and 116 girls (44.3%). Median age at operation was 11.4 years (range, 1.1-15.9). Six obese (with a body mass index greater than the 95th percentile) children (2.3%) developed intra-abdominal abscess after perforated appendicitis that was treated with a course of antibiotics. One child required revisional surgery and drainage. The median length of antibiotic treatment was 3 days (range, 0-15). CONCLUSION In our institution, SPA is the method of choice for appendectomy in children with acute appendicitis, in whom preoperative ultrasound does not reveal signs of perforation. The infection rate (2.7%) after SPA is not increased compared with other laparoscopic or open techniques. Overweight (body mass index greater than the 95th percentile) and perforated appendicitis seem to increase the risk of postoperative infectious complications.

Renal dysplasia in children with posterior urethral valves: a primary or secondary malformation?

Abstract Routine prenatal ultrasound examination of the urogenital tract is of importance in patients with posterior urethral valves (PUV), because the renal function and long-term prognosis of these patients depend on early diagnosis and subsequent therapy. Opinion is divided as to whether the often-observed association of PUV with renal dysplasia represents a primary malformation or a secondary pathology caused by recurrent infections. These aspects should have an influence on therapeutic consequences and optimal timing of therapy in order to preserve long-term renal function. The histology of kidney specimens from 13 children with PUV who underwent nephrectomy was reviewed to attempt to differentiate between primary dysplastic malformations and secondary pathologies. Clinical data were analyzed and compared with the histologic findings. The average age at nephrectomy was 29 months (range 3–158 months). Approximately 80% of the specimens showed primary dysplastic malformations (mesenchymal or fetal cartilage tissue or dysplastic glomeruli and tubuli) in the presence of well-developed renal parenchyma. All specimens showed secondary pathologies such as renal-cortical atrophy, interstitial fibrosis, and interstitial-nephritis atrophy. The histologic evidence of well-differentiated renal parenchyma in concurrence with dysplastic parenchyma makes infravesical obstruction as the only cause of renal alterations in patients with PUV questionable. This coincides with the fact that organogenesis of the kidney is terminated at the 12th gestational week and secondary renal damage is irreversible at the 20th gestational week, but prenatal urinary diversion of the upper urinary tract is feasible in the 20th gestational week at the earliest. These facts must be taken into account when considering intrauterine urinary diversion.

Neurogenic appendicopathy: clinical, macroscopic, and histopathological presentation in pediatric patients.

BACKGROUND The proliferation of nerve fibers in the appendix, in association with an increase of the neuropeptides such as vasoactive intestinal peptide and substance P characterizes neurogenic appendicopathy (NA), which may mimic the symptoms of acute appendicitis (AA). It may be difficult to differentiate this little known distinct pathological entity clinically from AA. The aim of this study is to describe the epidemiology, clinical signs, and histological description of NA in pediatric patients. PATIENTS AND METHODS After institutional review board approval, all appendiceal specimens from children who underwent appendectomy between February 2006 and December 2008 were analyzed. Analysis included age, gender, clinical symptoms, blood tests, intraoperative macroscopic assessment of the appendix, and the histopathological type of NA. NA was diagnosed by hematoxylin-eosin staining and /or S-100 immunochemistry and then classified into different histopathological groups. RESULTS Of the 385 appendix specimens examined, 29 (7.5%) met the histopathological criteria of NA. The median duration of abdominal pain was 2 days (range, 1 to 7). The surgeon characterized the appendix as follows: not inflamed in 5 (17.2%), acute in 17 (58.6%), phlegmonous in 4 (13.8%), and perforated in 3 patients (10.4%). Histopathological diagnosis of NA classified the specimen as axial neuroma in 2 (7%), mucosal in 12 (41%), and submucosal in 15 cases (52%). CONCLUSION NA is a relatively common diagnosis in children. NA must be considered in patients with recurrent right lower quadrant pain or signs of AA, even if intraoperative findings are normal. For these patients, we recommend appendectomy and consideration of a subsequent histopathological workup.

Vacuum bell therapy

BACKGROUND For specific therapy to correct pectus excavatum (PE), conservative treatment with the vacuum bell (VB) was introduced more than 10 years ago in addition to surgical repair. Preliminary results using the VB were encouraging. We report on our 13-year experience with the VB treatment including the intraoperative use during the Nuss procedure and present some technical innovations. METHODS A VB with a patient-activated hand pump is used to create a vacuum at the anterior chest wall. Three different sizes of vacuum bells, as well as a model fitted for young women, exist. The appropriate size is selected according to the individual patients age and ventral surface. The device should be used at home for a minimum of 30 minutes (twice a day), and may be used up to a maximum of several hours daily. The intensity of the applied negative pressure can be evaluated with an integrated pressure gauge during follow-up visits. A prototype of an electronic model enables us to measure the correlation between the applied negative pressure and the elevation of the anterior chest wall. RESULTS Since 2003, approx. 450 patients between 2 to 61 years of age started the VB therapy. Age and gender specific differences, depth of PE, symmetry or asymmetry, and concomitant malformations such as scoliosis and/or kyphosis influence the clinical course and success of VB therapy. According to our experience, we see three different groups of patients. Immediate elevation of the sternum was confirmed thoracoscopically during the Nuss procedure in every patient. CONCLUSIONS The VB therapy has been established as an alternative therapeutic option in selected patients suffering from PE. The initial results up to now are encouraging, but long-term results comprising more than 15 years are so far lacking, and further evaluation and follow-up studies are necessary.

Non-surgical Treatment for Pectus Excavatum and Carinatum

Pectus excavatum (PE) and carinatum (PC) are characterized by an abnormal overgrowth of sternal and costal cartilages, which result in a depression or protrusion of the sternum and costal cartilages, respectively. Both chest wall malformations are cosmetic and functional pathologies. Whereas PE is commonly associated to cardiopulmonary dysfunction, PC causes deformation of the entire thoracic cage. PE is generally corrected operatively. In contrast, due to inherent risks of a major surgery, only severe cases of PC are operated. One of the authors (FMH) will describe his 12 years experience with vacuum bells to treat PE patients conservatively. The use of vacuum bells allow significant lift of the ribs and sternum, until definitive correction of cartilage growth takes place. When employed during minimally invasive repair of PE (MIRPE), vacuum bells can also be used as a tool to enhance retrosternal dissection, advancement of the pectus introducer and insertion and flipping of the pectus bar/s. The other author (MMF) will describe his 13 years experience with the FMF® Dynamic Compressor System to treat patients with PC conservatively. When considering results, there should be little doubt that no patient would be selected as a candidate for surgery before trying a non-operative approach. Further evaluation and follow-up studies are still necessary for both conservative approaches, though.

Laparoscopic-Assisted Single-Port Appendectomy in Children: It Is a Safe and Cost-Effective Alternative to Conventional Laparoscopic Techniques?

Aim. Laparoscopic-assisted single-port appendectomy (SPA), although combining the advantages of open and conventional laparoscopic surgery, is still not widely used in childhood. The aim of this study was to evaluate the safety and the cost effectiveness of SPA in children. Methods. After institutional review board approval, we retrospectively evaluated 262 children who underwent SPA. The appendix was dissected outside the abdominal cavity as in open surgery. For stump closure, we used two 3/0 vicryl RB-1 sutures. Results. We identified 146 boys (55.7%) and 116 girls (44.3%). Median age at operation was 11.4 years (range, 1.1–15.9). Closure of the appendiceal stump using two sutures (cost: USD 15) was successful in all patients. Neither a stapler (cost: USD 276) nor endoloops (cost: USD 89) were used. During a follow-up of up to 69 months (range, 30–69), six obese children (2.3%, body mass index >95th percentile) developed an intra-abdominal abscess after perforated appendicitis. No insufficiency of the appendiceal stump was observed by ultrasound. Five of them were treated successfully by antibiotics, one child required drainage. Conclusion. The SPA technique with conventional extracorporal closure of the appendiceal stump is safe and cost effective. In our unit, SPA is the standard procedure for appendectomy in children.