Long-term haematological response and maintained immunological function after laparoscopic subtotal splenectomy in patients with hereditary spherocytosis.

INTRODUCTION: Subtotal or total splenectomy are recommended in severe and should be considered in intermediate forms of hereditary spherocytosis (HS). Data on laparoscopic subtotal splenectomy (LSTS) in HS patients are sparse. METHODS: Thirty three patients with HS (median age 10.7 years (yrs), range 1.8-15.5) underwent LSTS. Baseline and follow-up investigation included haematological parameters, microscopic analysis of pitted erythrocytes (pitE), and B-cell subpopulations assessed by flow cytometry. Results were compared to those of non-splenectomised HS patients, HS patients after total splenectomy (TS), and healthy individuals. RESULTS: After LSTS, haemoglobin levels were normalised in all patients. During median long-term follow-up of 3.9 yrs (range 1.1-14.9), only four patients presented mild anaemia. Despite re-growing of the remnant spleen none of the patients required a second surgical intervention. As compared to TS, PitE in LSTS patients were significantly lower and indicated normal to only moderately decreased spleen function. Relative but not absolute IgM memory B-cell counts were reduced in both LSTS and TS patients. CONCLUSIONS: LSTS is effective for the treatment of patients with HS. A small remnant spleen is sufficient to provide adequate phagocytic function and to induce a pool of IgM memory B-cells.

Impact of surgical correction of pectus excavatum on cardiac function: insights on the right ventricle. A cardiovascular magnetic resonance study†.

OBJECTIVES: Pectus excavatum (PE) is often regarded as a cosmetic disease, while its effect on cardiac function is under debate. Data regarding cardiac function before and after surgical correction of PE are limited. We aimed to assess the impact of surgical correction of PE on cardiac function by cardiovascular magnetic resonance (CMR). METHODS: CMR at 1.5 T was performed in 38 patients (mean age 21 ± 8.3; 31 men) before and after surgical correction to evaluate thoracic morphology, indices and its relation to three-dimensional left and right ventricular cardiac function. RESULTS: Surgery was successful in all patients as shown by the Haller Index ratio of maximum transverse diameter of the chest wall and minimum sternovertebral distance [pre: 9.64 (95% CI 8.18-11.11) vs post: 3.0 (2.84-3.16), P < 0.0001]. Right ventricular ejection fraction (RVEF) was reduced before surgery and improved significantly at the 1-year follow-up [pre: 45.7% (43.9-47.4%) vs 48.3% (46.9-49.5%), P = 0.0004]. Left ventricular ejection fraction was normal before surgery, but showed a further improvement after 1 year [pre: 61.0% (59.3-62.7%) vs 62.7% (61.3-64.2%), P = 0.0165]. Cardiac compression and the asymmetry index changed directly after surgery and were stable at the 1-year follow-up [3.93 (3.53-4.33) vs 2.08 (1.98-2.19) and 2.36 (2.12-2.59) vs 1.38 (1.33-1.44), respectively; P < 0.0001 for both]. None of the obtained thoracic indices were predictors of the improvement of cardiac function. A reduced preoperative RVEF was predictive of RVEF improvement. CONCLUSIONS: PE is associated with reduced RVEF, which improves after surgical correction. CMR has the capability of offering additional information prior to surgical correction.

Lessons learned from lethal cardiac injury by nuss repair of pectus excavatum in a 16-year-old.

This report investigates cardiac injury and arrest during a Nuss repair of severe pectus excavatum in a 16-year-old boy in 2006. The injuries of the right atrial auricle and the right ventricle were sutured, and the patient was resuscitated. Ultimately he died on the 11th day of progressive malignant cerebral edema and respiratory distress syndrome despite cerebral decompression and hypothermia. Typical morphologic features of cardiac injuries are demonstrated, and strategies to avoid inadvertent organ injury in pectus operations are discussed.

Re-evaluation of histological findings of nonparasitic splenic cysts.

OBJECTIVE: The pathogenesis of nonparasitic splenic cysts (NPSCs) has not been clarified completely. The aim of this multinational and multicentre retrospective study was to further elucidate the origin of NPSCs. METHODS: From 1980 to 2006, 50 children and adolescents were surgically treated for NPSC at six paediatric surgical centres in four European countries. The initial histology report of 35 NPSCs, 22 epidermoid cysts, 11 pseudocysts or post-traumatic cysts and two mesothelial cysts was available. Additional re-evaluation, including immunohistochemistry, to detect cytokeratin, carcino-embrionic antigen and mesothelioma antibody in the inner surface of the cysts was carried out. Special attention was given to the possibility of preceding trauma to the splenic area and whether it played a role in the genesis of NPSC. RESULTS: The pathological re-evaluation showed 30 epidermoid cysts, four mesothelial cysts and one pseudocyst. Immunohistology revealed eight epidermoid and two mesothelial linings of the cysts in those 11 patients in whom pseudocyst was diagnosed originally. No pseudocyst was documented in those patients who had a history of previous blunt abdominal trauma but was not proved by ultrasound and computed tomography scan. CONCLUSION: In contrast with the prevailing belief, it has been demonstrated that NPSCs are congenital in origin, and there is no clinically proven evidence that trauma does play a role in their genesis.

New Berlin-Buch "reversed Nuss," endoscopic pectus carinatum repair using eight-hole stabilizers, submuscular CO2, and presternal Nuss bar compression: first results in 35 patients.

INTRODUCTION: Since 2001 we minimized access (2.9-4.7 cm) for universally applicable endoscopic hybrid carinatum technique with two transsternal Willital bars in 173 endoscopic hybrid (EH) patients with very satisfactory results. In 2008-2009, endoscopic Nuss bar compression with endoscopic repair of costal flaring applied a new eight-hole stabilizer, which allows the use in pectus carinatum (PC) beyond adolescence including redos and combined deformities. This prospective study of 35 "endoscopic Berlin-Buch reversed Nuss" repairs intends to establish indications for this improved technique. MATERIALS AND METHODS: In February 2008 to February 2010, we used endoscopic Nuss bar compression by applying a bilateral new eight-hole stabilizer fixed to the bar without screws or wires, which allows unprecedented versatility and the use in pectus carinatum beyond adolescence. Thirty-five patients aged 17.05 ± 10.2 years (range: 11.3-33.1 years) were recorded prospectively and followed at 3 monthly intervals. We implanted a standard Nuss bar (11-14') into an endoscopically dissected submuscular presternal pocket correcting PC by sternal pressure. The bars were put under tension by traction via bilateral eight-hole stabilizers and three pericostal wire sutures on each side. Bars were removed after 2 years. RESULTS: All 35 "reversed Nuss" pectus carinatum repairs, including 2 redos after Ravitch, were successful, with no conversion. So far there was no local or general complication and no seroma or bar dislocation. Thirty-one patients judged their result as excellent and 4 as good. CONCLUSIONS: Although this is a very early experience, "reversed Nuss" is safe and effective and new technical improvements have expanded the range of applicability to older patients and suitable redos.

[Case Report: Failed epidural puncture for insertion of a catheter]. / Kasuistik: Perioperative Schmerztherapie - Fehllage eines thorakalen Periduralkatheters nach Punktion in Seitenlage.

We report on a failed epidural puncture for insertion of a catheter during chest wall correction by the minimally invasive procedure according to Nuss in a 16-year-old boy. After insertion of the catheter without any problem and establishment of a symmetrical thoracic analgesia and initiation of general anaesthesia, the catheter was surprisingly observed in the thoracic cavity upon insertion of the endoscopic camera. The catheter was then withdrawn under vision and the operation continued without any further incidents.

Laparoscopic approach to ovarian mass in children and adolescents: already a standard in therapy.

INTRODUCTION: Functional cysts, ovarian torsion, and benign neoplasms are the most common ovarian masses among young adolescents. The laparoscopic approach to giant ovarian cysts in the pediatric population maybe difficult due the limited working space and the high risk of spillage. In this paper, we evaluate the role of laparoscopic surgery in the treatment of adnexal disease occurring in young girls. MATERIALS AND METHODS: With the approval of the institutional review board, a retrospective chart review(2007-2003) of patients with adnexal disease was conducted. RESULTS: Overall, 12 patients were evaluated with preoperative imaging, sonography, and magnetic resonance imaging (MRI) scan and laboratory values. None resulted in malignant histology. All resections of ovarian cysts were performed laparoscopically. The outcome was uneventful in all patients. CONCLUSIONS: Treatment is indicated if the diagnosis is in question, the cyst persists, in the case of ovarian torsion,or if the patient is symptomatic. Laparoscopy is becoming the favored approach by most pediatric surgeons for the treatment of ovarian cysts. All surgical procedures for ovarian cysts should spare functional ovary as much as is technically possible. Simple cysts can be fenestrated, but complex or functional cysts should be excised, with the preservation of the remaining ovary by careful dissection. The laparoscopic approach for adnexal masses can be performed in an acceptable manner, with comparable results to an open approach, plus the cosmetic advantages of minimally invasive surgery, which is an important aspect for the treated patients.

New endoscopic minimal access pectus carinatum repair using subpectoral carbon dioxide.

PURPOSE: This study establishes a minimal access hybrid technique for pectus carinatum repair. Based on 132 conventional repairs (1984-2000) and our own endoscopic technique for correcting prominent costal arches, the conventional carinatum repair was adapted to an endoscopic-assisted technique. DESCRIPTION: Inserting two submuscular trocars and inflating CO2, the entire ventral thoracic wall was dissected endoscopically detaching pectoral muscles from ribs and sternum. February 2001 to February 2004, we repaired 37 patients (32 male) of 16.8 +/- 4.3 years (12 to 36 years). Endoscopic-assisted rib resection and axial reanastomosis, transsternal struts, and sternotomies were performed semi-open from a 2.9 to 4.7 cm incision. EVALUATION: All were completed minimally invasively, one seroma was managed conservatively. Thirty-three patients rated their result as excellent, 4 as good with a follow-up of 29.1 +/- 9.5 months (range, 18 to 55 months). Twenty-one struts were removed with no recurrence. CONCLUSIONS: Minimal access pectus carinatum repair is safe, effective, and offers high comfort for the patient. The results are at least as good as conventional repairs, but hospital stays could be halved. Encouraging results of this early experience warrant further evaluation by other centers.

Thoracoscopic diaphragmatic eventration repair in children: about 10 cases.

BACKGROUND AND AIM: Recent reports in literature have emphasized the clinical perception of reduced pain, postoperative morbidity, and dysfunction associated with thoracoscopic approach compared with standard thoracotomy. The authors describe a thoracoscopic approach and technical details for diaphragmatic eventration repair in children. PATIENTS AND METHODS: Ten patients, 4 girls and 6 boys, 1 teenager (14 years old) and 9 children (age range, 6-41 months; average, 17 months), were operated for a diaphragmatic eventration in 3 different pediatric surgery teams, according to the same technique. Symptoms were recurrent infection (7 cases), dyspnea on exertion (2 cases), and a rib deformity (1 case). An elective thoracoscopy was performed, patient in a lateral decubitus. A low carbon dioxide insufflation allowed a lung collapse. Reduction of the eventration was made progressively when folding and plicating the diaphragm. Plication of the diaphragm was done with an interrupted suture (6 cases) or a running suture (4 cases). The procedure finished either with an exsufflation (4 cases) or a drain (6 cases). RESULTS: A conversion was necessary in 2 cases: 1 insufflation was not tolerated and 1 diaphragm, higher than the fifth space, reduced too much the operative field. Patients recovered between 2 and 4 days. Dyspnea disappeared immediately. Mean follow-up of 16 months could assess the clinical improvement in every patient. DISCUSSION: Thoracoscopic conditions are quite different between a diaphragmatic hernia repair previously reported and an eventration. Concerning diaphragmatic hernias, reduction is easy, giving a large operative space for suturing the diaphragm. Concerning diaphragmatic eventrations, the lack of space remains important at the beginning of the procedure despite the insufflation into the pleural cavity. The operative ports must be high enough in the chest to allow a good mobility of the instruments. Chest drainage seems to be unnecessary. CONCLUSION: Diaphragmatic eventration repair by thoracoscopy is feasible, safe, and efficient in children. Above all, it avoids a thoracotomy. It improves the immediate postoperative results with a good respiratory function.

Extrapleural, submuscular bars placed by bilateral thoracoscopy--a new improvement in modified Nuss funnel chest repair.

BACKGROUND: Thoracoscopic Nuss funnel chest repair still has a significant complication rate. Bar dislocation, pneumothorax, pleural effusions, and pericarditis seem to be caused mechanical irritation by the bar. We intended to reduce these problems by further technical modification of the Nuss technique. METHODS: Of 157 prospectively followed modified Nuss repairs, the last 57 patients had the bars placed in an extrapleural position and fixed by 10 to 14 pericostal sutures under bilateral thoracoscopy. RESULTS: Entirely, extrapleural bar position was feasible in 53 of 57 patients. Four patients had minor holes over one of the bars, predominantly on the left side of the thorax. Pleural effusions, pneumothorax, and pain were greatly reduced, so that we discontinued the so far routine use of bilateral pleural drainages. CONCLUSIONS: Extrapleural bar position is feasible in more than 90% of modified Nuss repairs. It reduces pleural secretion and pain, and seems to reduce pneumothorax, pulmonary bar adhesions, and pericardial effusions. The technique is easy and safe, and reduced the incidence of most complications in this early experience of 57 adolescent patients, although no sportive restrictions were imposed at all.

Abdominal inflammatory masses mimicking neoplasia in children-experience of two centers.

Despite progress in modern imaging, some inflammatory masses are difficult to distinguish clinically from neoplastic processes. In such cases the pathology report has a great distinctive value, but even then the final diagnosis may be difficult to reach. Eight patients with abdominal tumors of inflammatory origin were treated in two institutions, the Department of Pediatric Surgery of the Medical University of Gdansk, Poland, and Helios Center of Pediatric Surgery in Berlin, Germany, during the last 10 years. Four tumors were located in the pelvis, two in the liver, and two in the colonic mesentery. Five of them were inflammatory pseudotumors (two subclassified as inflammatory fibrosarcoma), one had nonspecific inflammatory changes, one was diagnosed as idiopathic retroperitoneal fibrosis, and one was diagnosed as bacillary angiomatosis. All patients underwent surgical tumor biopsy, excisional in four and incisional in four. All but two children underwent macroscopically complete tumor excision (four primarily, two secondarily). In one case the tumor resolved with antibiotherapy. Surgery in retroperitoneal masses was often extensive and associated with significant complications because of invasive tumor growth. In conclusion, intraabdominal inflammatory lesions may closely mimic neoplasia in children. Clinical doubts result in repeated biopsies, and for this reason excisional biopsy should be preferred. In some cases, when excisional biopsy is not feasible due to invasive growth of the tumor, delayed complete mass excision should follow, despite occasional significant morbidity. The etiology and exact nature of inflammatory pseudotumors are still obscure, and it is unknown whether they represent inflammatory lesions or true neoplasia.

The technique of laparoscopic retroperitoneal splenopexy for symptomatic wandering spleen in childhood.

BACKGROUND: Wandering spleen is an uncommon diagnosis, difficult to prove by standard investigations. The authors report a new method for laparoscopic splenopexy in children using a balloon-dilated retroperitoneal pouch. METHODS: From 3 accesses, the spleen is mobilized and displaced into a retroperitoneal pouch dilated to the double splenic volume. The pouch is dilated by a self-made balloon via a further intercostal access and narrowed by sutures incorporating the cranial and caudal edge of the gastrosplenic ligament. RESULTS: The peritoneal pouch contracts around the retroperitoneal spleen resulting in a firm fixation of the organ. This technique was successful in a 9-year-old girl with a 5-year history of severe recurrent abdominal pain. CONCLUSIONS: Laparoscopic retroperitoneal pouch splenopexy is a safe and effective procedure for symptomatic wandering spleen precluding the use of foreign materials in this age group.

Laparoscopic antireflux procedures in the management of gastroesophageal reflux following esophageal atresia repair.

OBJECTIVES: The validity of open fundoplication after esophageal atresia (EA) repair is still disputed. The authors have retrospectively evaluated the results achieved in their centers using laparoscopic antireflux procedures (LARP) in children operated for EA at birth. METHODS: From 1998 to 2002, 350 children underwent LARP. Of these, 21 (6%) underwent EA repair at birth. Our study focused only on the management of these 21 patients; 5 of them (23.8%) were neurologically impaired children (NIC). All underwent LARP, 9 patients according to Nissen, 9 according to Thal, and 3 according to Toupet. The 5 NIC with feeding problems underwent concomitant g-tube placement during the same procedure. RESULTS: All the procedures were completed in laparoscopy, without intraoperative complications. The mean operative time was 65 minutes (range 45-140). We had no mortality in our series. Hospital stay varied from 2 to 9 days (median 3 days). At a maximum follow-up of 6 years, all patients were evaluated with a 24-hours pH-metry and barium swallow. The 16 neurologically normal children were free of symptoms at the last follow-up; five of them (31.2%) had mild dysphagia, which disappeared spontaneously within 3-6 months. One girl experienced an important episode of aspiration 2 years after the LARP, although there was no evidence of reflux at the follow-up examinations. As for the 5 NIC, one patient eats only through a g-tube, the other 4 undergo mixed feeding (g-tube and mouth); none have signs of GER, but two of them still present respiratory symptoms, and one has delayed gastric emptying. CONCLUSIONS: In our experience laparoscopic antireflux surgery is an appropriate treatment of GER in children operated for EA at birth, independently of the antireflux mechanism adopted; the 31.2% rate of short-term dysphagia presenting as residual respiratory symptoms may be due to a primary dysmotility of the esophagus consequent to the esophageal atresia.

Gastroschisis: a 15-year, single-center experience.

70 cases of gastroschisis (GS) were surgically treated at the Pediatric Surgical University Clinic, Münster, from 1984 through 1998. The defect occurred more frequently in males (44) than females (26). The average birth weight was 2,383 g and mean gestational age 36.8 weeks. 9 infants (12.9%) were delivered vaginally and the rest (87.1%) by cesarean section; 34 of the 61 (55.7%) cesarean sections were done solely for prenatal ultrasonic identification of the abdominal-wall defect. 10 infants (14.3%) underwent primary closure; in 19 (27.1%) primary closure of the skin was possible, however, a single solvent-dried dura (SDD) graft was required for fascial enlargement. The remaining 41 infants (58.6%) had extensive defects and required two grafts for optimal closure. 22 patients (31.4%) had associated anomalies, the most common being bowel atresias and undescended testis. 14 (20%) required secondary laparotomies because of bowel-associated complications and 1 (1.4%) for a urinary-bladder perforation. 11 patients (15.7%) had non-bowel-associated complications. The average postoperative tracheal intubation time was 3.9 days and the average hospital stay was 75.6 days. The overall mortality was 2.8%. No major complications associated with SDD implants were encountered; only 4 patients (5.7%) had minor complications such as local inflamation and infection and were managed conservatively. The present data support the employment of SDD implants as acceptable biomaterial for the repair of large GS defects.

Thoracoscopic aortopericardiosternopexy for severe tracheomalacia in toddlers.

BACKGROUND/PURPOSE: Aortosternopexy from a left anterolateral thoracotomy is the procedure of choice in severe tracheomalacia. The authors report an alternative technique of modified thoracoscopic aortopericardiosternopexy. METHODS: Thoracoscopy under mild CO2 insufflation (insufflation pressures 4 to 6 mm Hg) provides excellent access without selective intubation. The importance of visualizing the phrenic nerve, mobilization of the thymus without disrupting its vascular supply, and intraoperative bronchoscopy is stressed. The technique of passing the needle through the sternum and back is shown. In long segment tracheomalacia, not only the ascending aorta, but also the innominate artery and base of the pericardium are fixed to the sternum, and the effect is monitored by intraoperative bronchoscopy. RESULTS: This technique was dramatically successful in a 4-year-old boy with long segment tracheomalacia and as a redo procedure in a 2-year-old girl after failed open aortopexy. CONCLUSION: Thoracoscopic aortopexy seems to be as effective as open aortopexy.

Submuscular bar, multiple pericostal bar fixation, bilateral thoracoscopy: A modified Nuss repair in adolescents.

BACKGROUND/PURPOSE: Thoracoscopic Nuss repair of funnel chests is used increasingly, but has a high bar dislocation rate. The authors intended to reduce this by technical modifications of the original Nuss technique. METHODS: In 40 patients from 12.3 to 42.1 years of age (mean, 17.6 +/- 5.8) the bars were placed directly on the ribs in a submuscular position and fixed by a minimum of 14 absorbable figure of 8 sutures around the bar and the underlying rib placed under bilateral thoracoscopy. Two stabilizers were used in all patients, the bar was introduced from the left in severe cases, and a second bar was implanted in most beyond 16 years of age. All patients underwent follow-up to date in a prospective observation study. RESULTS: There was no bar or stabilizer dislocation, no prolonged pain or neuralgia, but one traumatic seroma, one pleural, and one pericardial effusion. One bar was easily removed after 13 months. CONCLUSIONS: Submuscular position provides a far better bar fixation and soft tissue coverage of Nuss implants. The technique is technically more demanding but safe and has reduced the incidence of bar dislocation to zero in this early experience of 40 adolescent patients, although no sportive restrictions were imposed on the patients at all.

Ultrasonic shear coagulation of main hilar vessels: A 4-year experience of 23 pediatric laparoscopic splenectomies without staples.

BACKGROUND: Ultrasonic shears (LCS) are used increasingly for laparoscopic splenectomy. So far however, all investigators use vascular staplers or clips for section of the main splenic artery and vein. METHODS: After several trials the authors started to use the ultrasonic triple welding technique in open surgery to occlude major vessels of 5 to 8 mm by 10-mm LCS. In June 1997 the authors introduced triple welding into laparoscopic splenectomy to mobilize the complete spleen by LCS. RESULTS: There was no hemorrhage in 23 laparoscopic splenectomies performed exclusively by LCS and no complications except 1 port site hernia. CONCLUSIONS: Laparoscopic splenectomy entirely by reusable LCS without clips and stapler is a safe, simple, and inexpensive technique. Moreover, the policy of "leaving nothing back" is an attractive strategy in endoscopic pediatric surgery.