Alexander Rokitansky, R.J. Hahn, R. Stanek
Department of Pediatric Surgery, Donauspital , Vienna, Austria.
We report our initial experience with the minimally invasive method of surgical reconstruction of pectus excavatum using a recently developed pectus bar (PSI-Implantat/Hofer Company/Austria). The new pectus bar is equipped with an integrated, firmly connected stabilizer at one end. In contrast to conventional implants, an additional stabilizer need not be inserted and fixed. At our department 120 pectus excavatum patients have been operated on by the minimally invasive method of reconstruction (Nuss technique). From November 2005 to the present time, we have used the newly designed bar for reconstruction of a pectus excavatum in 10 patients.
The following advantages of the new method deserve mention:
1. The one-piece implant avoids dislocation of the stabilizer plates. The procedure is also superior to the increasingly popular conventional implant that employs only one stabilizer plate.
2. The three perforations in the stabilizer facilitate surgical handling and permit better fixation in the tissue.
3. If necessary the stabilizer can be adjusted to the desired chest wall curvature.
4. In contrast to the conventional implant, the ends of the bar can also be bent and adjusted to the curvature of the body.
5. When using two pectus excavatum implants, unpleasant contact with metal, which is a characteristic of conventional stabilizer plates, is avoided with the new stabilizer.
In summary, the development of the new one-piece pectus bar is a significant advancement in the treatment of pectus excavatum.
Spyros Spyrakos, Michael Soutis, Dimitrios Antoniou, George Christopoulos-Geroulanos
2nd Surgical Department, Aghia Sophia Children’s Hospital, Athens, Greece.
Oesophageal strictures following caustic ingestion in children are a serious problem requiring long term treatment and in severe cases even esophageal replacement. Our experience in the management of caustic injuries is presented.
Material and Methods : During the period 2000-2005, 20 patients were admitted because of injection of caustic substances. All the patients were submitted to endoscopy. Follow up oesophagoscopy revealed a normal oesophagus in 12 patients. Eight patients developed strictures and were submitted to dilatations by balloon and/or Savary dilators. Antireflux treatment was prescribed to all patients. Cortisone was administered to all patients with 2nd and 3rd degree burns. Initially dilatations were performed every 2 weeks, the intervals being prolonged according to the response to the dilatations. Patients who could not be treated every 2 weeks as well as patients who did not follow antireflux therapy were resistant to the dilatations. Twenty-four hour pH-metry of patients who did not require further dilatations showed no reflux.
The duration of the treatment varied according to the severity of the burn.
Conclusions : A two week interval between the dilatations is required for gradual improvement of the stenosis. Antireflux treatment is very important and noncompliance of the patient must be kept in mind in cases of refractory esophagitis. Esophageal stenosis following resolution of oesophagitis responds well with dilatation at 3 months intervals.
Marcos Bettolli, Steven Rubin
Department of General Pediatric surgery, Children’s Hospital of Eastern Ontario, Ottawa, Canada
Background: Pediatric surgical reports of the results of laparoscopic large paraesophageal hernia (LPEH) repair are few. LPEH repair in adults is associated with significant intraoperative and postoperative morbidity. Ten years of satisfactory laparoscopic fundoplications encouraged us to repair PEH in children laparoscopically. We report our recent initial experience.
Methods: Since 2005 four children aged 3-17 years underwent laparoscopic LPEH repair with fundoplication. Closure of the hiatal defect included crural sutures and prosthetic material, either expanded polytetrafluoroethylene mesh or a small intestinal submucosal patch. Two children with cerebral palsy (CP) had gastrostomies.
Results: The operative time was 300 – 600 min, with neither conversions nor surgical/ anesthetic complications. Four patients were discharged within 3 days. Superior mesenteric artery syndrome in a severely malnourished 17-year-old with CP, delayed his discharge until the sixth postoperative day. In all patients, postoperative UGI x-ray showed no recurrence of the LPEH and an intact fundoplication.
Conclusion: Laparoscopic LPEH repair in children is uncommon and technically demanding. Prosthetic material was necessary to accomplish tension-free hiatal closure. We consider familiarity with laparoscopic fundoplication useful for LPEH repair. Our experience with these four patients suggests that a multicenter prospective study of laparoscopic LPEH repair in children is indicated
Sandra Duncan, Sarah Conway, Richard Turnock, Colin Baillie, Simon Kenny
Department of Surgery, Royal Liverpool Children’s Hospital (Alder Hey) , Liverpool, United Kingdom.
To document the incidence of intussusception in children admitted to hospital with Henoch-Schonlein Purpura (HSP) and assess the role of surgery. We hypothesized that not all children with ultrasound proven small bowel intussusception and HSP need operative intervention.
The care records of children discharged with a diagnosis of HSP (ICD 69.0) between June 1997 and 2005 from a secondary and tertiary paediatric centre were examined. Demographic data, number of admissions, radiological investigations, findings, surgical management and clinical course, were collated.
160 children had 203 inpatient episodes; (range 1-6 admissions per child.) Average age 7 years (range 7 years (range 0.5-16.2 years.) 90 (56%) were male. 48 (30%) children had abdominal ultrasound examination for clinical suspicion of intussusception. 7 ultrasounds were positive. Spontaneous reduction was seen in 3 children during examination. 2 children with radiological evidence of intussusception required surgical intervention because of physiological instability.
Intussusception was diagnosed radiologically or intraoperatively in 5 /160 (3.1%)children with HSP.
HSP is a relatively common condition and abdominal pain suggestive of intussusception is a manifestation in a third of children requiring hospital admission. The likelihood of surgical pathology is low; although it is likely that transient intussusception is occurring in a significant proportion of children with abdominal pain. This study has shown that in cases of HSP related intussusception a non-operative management strategy can be adopted with close observation. Laparotomy should be reserved for children whose symptoms and signs are worsening or failing to improve.
María José Moya Jiménez, José Antonio Cabo, Israel Fernández Pineda, María del Carmen Macías, Rocío Granero Cendón, Juan Carlos de Agustín, Manuel López-Alonso
Paediatric Surgery, Hospital Infantil Virgen del Rocío, Sevilla, España.
BACKGROUND. The relationship between gastroesophageal reflux (GER) and hiatal hernia (HH) is still not clearly understood in children. With the introduction of new diagnostic methods we can now deeply explore the correlation of GER with the anatomical alterations observed in HH.
AIM. Evaluate the influence of the HH on the RGE in children.
METHODS. In the last 5 years 1501 paediatric GER patients have been studied at our Motility Unit. 47 of these (3.1%) have been diagnosed as having HH; Eleven were excluded. This group was compared with 36 control patients with GER and without HH, who were selected with such criteria of exclusion and through a random numbers table.
RESULTS: The populations were statistically comparable through weight, height and age to the date of study. The total number of reflux in the control population was of 40.5 (16 – 64.5) (medium, interquartile rank), 38.5 (25.5 – 69.5) in patients with HH (p = 0.53). Concerning reflux index (% pH < 4) it was of 4.6% and 6.7% respectively (p = 0.15). Either there was statistically no significant difference (p = 0,2) between population control and patients with HH as far as bolus clearance time nor to the reflux number greater to 5 minutes (p = 0.18).
CONCLUSION: We did not find statistically significant difference between the values obtained by 24 h pH-monitoring in both groups (HH and GER without HH), which lead us the idea that the anatomical alterations are not determining factors in the aetiology of the GER.
Marianna De Marco1, Ciro Esposito2, Francesca Alicchio1, Giuseppe Ascione1, Emanuela Giordano1, Alessandro Settimi1
1-Pediatric Surgery, University "Federico II" , Naples, Italy.
2-Dept. Pediatric Surgery University "Magna Grecia" Catanzaro, Italy
Background: Feeding problems and gastroesophageal reflux (GER) are major problems in neurologically impaired children. Many patients are managed with a simple gastrostomy, with or without fundoplication. Some others with a feeding jejunostomy.
Methods: Between 2002 and 2006, 28 patients(age range, 5-14 years) have been operated for feeding problems in our Istitution.
At preoperative work-up all the patients had an important malnutrition and 10 of them presented a gastroesophageal reflux. Four patients without GER already received abdominal surgery.
14 patients received a PEG(Percutaneous endoscopic gastrostomy); 10 a LAJ (laparoscopic assisted gastrostomy); 4 a LAG (laparoscopic assisted gastrostomy).
Results: Surgery lasted 40 minutes on average (25-55 minutes). There were no perioperative complications; hospital stay was 3-4 days for all patients. At follow-up, all patients had a significant weight gain, with high parental satisfaction. One patient died (LAJ group) one year after the procedure, for unknown causes. One patient (LAJ group) received a redo surgery for a malfunctioning of the jejunostomy. All the others patients are doing well, without complications or problems and their parents are extremely satisfied with the improved quality of life of their children.
Conclusions: In our experience PEG represents the first choice in patients with feeding problems without GER. In patients with a history of previous abdominal surgery we prefer to adopt laparoscopic assisted gastrostomy. In case of feeding problems and GER we prefer to perform a LAJ to avoid the risk to increase the reflux. All these procedures seem to solves the patients’ feeding problems with a great parents’ satisfaction.
Jesús Broto, Jose Maria Gil Vernet, Marino Asensio, Gloria Royo
Pediatric Surgery, Valle de Hebrón Hospital, Barcelona, España.
Aesophageal stenosis , due to its important implications on the patients’ quality of life, poses a serious, and often times difficult to resolve problem . Our group has tried over the last thirty years to employ conservative solutions in the damaged aesophagus, showing that a complete cure is possible in most cases, although the way to achieve this can often be dramatically long. To shorten the process, we employ Mitomycin C in the most severe cases.
Material and Methods
We present our experience employing topical Mitomicina C over the last 18 months in nine selected patients, six of them affected with caustic stenosis and three with intervention secuelae of aesophageal atresia, describing the method employed .The application time is five minutes and the dose is 0.4 mg/ml, applying up to 5ml.
Upon completing the application , a cleansing of the product is performed using the same fibroscope.
We analysed the four cases that received at least five doses. These were three children of two, seven and eight years, and a 25 year-old woman. In the children the dilatations interval passed from 4-5 weeks to 8-11, respectively.The woman has passed from 12 to15 weeks. The children have been in the dilatation program between 6 months and 1 year and the woman over 18 years.
Although the time period that we have applied Mitomicina C is still short, the symptom-free interval after dilatations is proving to be highly promising.
Key words: Mitomycin C, aesophageal stenosis.
Olga Suárez, Ane Andrés, Satur Barrena, Jose Luis Encinas, Ana Luis, Carmen Soto-Bauregard, Mercedes Díaz, Zoraida Ros, Juan Carlos López Gutiérrez
Cirugía Pediátrica, Hospital Infantil La Paz, Madrid, España.
Introduction: Aplasia cutis is a congenital absence of skin most commonly affecting the scalp. The lesions are non inflamatory and well demarcated. Most defects are small and superficial, and commonly presents as a solitary defect. Approximately 20% of cases involve absence of the skull. Such defects expose the brain and sagital sinus, with concomitnat risk of fatal hemorrage, infection, or both. Majority of these scalp defects occurs sporadically, but family cases have been reported. Multiple etiologies have been suggested for aplasia cutis: genetic causes , syndromes and teratogens.
Materials : A retrospective study of children with Aplasia Cutis Congenita who received treatment between 1995 and 2005 was undertaken. We checked location, moment of the surgery, type of surgery and aesthetic results.
Results: Between 1985-2005, we treated 36 patients with Aplasia Cutis. 33 patients have affecting the scalp, 4 of them involve absence of the skull, two slight and two severe. Only in 3 cases the trunk was involve. 3 patients had Adams-Oliver and one Cutis Marmorata Telangiectasica syndrome.
Fifteen patients were operated in neonatal period with direct closure or advancement or rotational flaps, and in 17 cases the late treatment included use of tissue expanders to cover definitely the defect. One of the patients died for bleeding of the sagital sinus while was waiting for the secondary closure of the wound, and other patient required complex skull reconstruction to achieve a complete coverage.
Conclusions: we believe that early surgery prevents vital risks, reduces local complications and makes easier the reconstruction.
Pediatric Surgery, Al Ain University, Al Ain, United Arab Emirates.
Haemangiomas (H) and vascular malformations (VM) are common benign congenital diseases causing a variety of manifestations, from purely cosmetic to life threatening. Their etiology, natural history, complications and the appropriate treatment is still controversial.
Material and Method:
Since 1995 a prospective series of 304 cases of H-VM (216 in RSA and 78 in UAE) is presented. Cases were classified according to Mulliken’s classification (1.Haemangiomas; 2.Vascular malformations: low flow (venous) and high flow arterio-venous fistulae (A-V F); 3.Lymphatic malformations; 4. Complex malformations). The initial treatment has been Intralesional Bleomycin Infiltration (IBI). Details of the technique and the long term results are briefly presented. A mean of 5 IBI sessions are needed (1 - 18) for majority of patients at 2-6 week intervals. The protocol for high flow (A-V F) consists of primary embolization, followed by IBI (8 cases) is also discussed.
On overall series 42% achieved complete remission and 38% significant improvement (on pain, swelling, cosmesis). The only serious complications noticed (4% of cases) are ulcerations and flagellate pigmentation.
The treatments utilized for H-VM is reviewed. Surgery as primary treatment is contraindicated. At present IBI proves to be the most effective option. The author also presents the preliminary result of a study (20 pts) of the blood and urine Bleomycin level after IBI which is very low, making the risk of pulmonary complication negligible.
Laura Burgos, Jose Luis Encinas, Ana Lourdes Luis, Leopoldo Martinez, Juan Antonio Tovar
Pediatric Surgery, Hospital La Paz, Madrid, España.
Aim: Perineal canal is a rare condition which consists in a midline perineal traject communicating the vulva with the anal crypts. It has been advocated that local inflammation could account for this condition but others believe that it is one of the rarest forms of anorectal malformation. The purpose of the present case report is to provide additional evidence of this malformative origin.
Method: We report a female in whom there was only a single urogenital orifice at birth and a normal anus. Upon endoscopic assessment of the urogenital sinus, a perineal canal was found allowing for the cystoscope to be passed from the vulva into the anal canal. A double vaginal confluence into the canal was also found. After diverting colostomy, the canal was opened and its posterior part excised to reconstruct the perineal skin exposing the vaginal openings into a rather normal vulva. Twelve years later, after dilating one of the vaginas which was partially obstructed, we took down the common wall with a stapling-cutting device.
Results: At thirteen, the patient has a normal looking vulva and a vagina of normal calibre and she has normal menses.
Conclusion: This case adds evidence supporting that perineal canal belongs to the spectrum of anorectal malformations with duplication of Müllerian structures.
Leonardo Siplovich, Bassem Kawar
Pediatric Surgery, Ha’emek Medical Center, Afula, Israel.
Total Colonic Aganglionosis is an infrequent variant of Hirschsprung’s disease which accounts for less than 10%of the cases and carries a high morbidity and mortality rate.
The signs and symptoms are different and sometimes more severe than those seen in typical Hirschsprung’s disease because the affected aperistaltic segment is too long to expel the fecal mass.
Advances in the understanding of the genetic basis,the clinical radiological and histological picture, contemporary neonatal intensive unit care, the use of total parenteral nutrition and the advent of current operative techniques, has dropped the mortality to less than 25%.
Our experience with 10 patients during the last 10 years showed that an adequate diversion of the normal bowel is mandatory.
In cases were the diagnosis of total colonic aganglionosis is not clear at the time of surgery , a stoma is performed at the level of normal ganglionated bowel and the aganglionic segment should be resected to prevent episodes of severe enterocolitis and sepsis.
In those cases with a correct diagnosis of the disease a modified Duhamel pullthrough was the procedure of choice at any age.
With this policy our survival rate is of 90%
Jai Mahajan, Krishan Narasimhan, Kattargadda Rao
Pediatric Surgery, Postgraduate IOnstitute of Medical Education and Research Chandigarh, Chandigarh, India.
Background- The most commonly used technique for transanal pull-through is long endorectal dissection leaving a long muscular cuff, which is usually split posteriorly. We present our initial experience following transanal Swenson’s operation for rectosigmoid disease.
Methodology- Seventeen patients underwent single stage transanal Swenson’s procedure. Initial diagnosis was based on clinical features and barium enema examination. Technique of the procedure involved incision of full thickness the rectal wall posteriorly at the dentate line and then incising the whole circumference of rectal wall. The remainder of rectal mobilization is done by working strictly on the surface of the rectal wall akin to mobilization in anorectal malformation. Fourteen patients were males. Age of the patients ranged from 2 months to 8 years (Mean 19.5 months). Two patients required abdominal mobilization of colon. There were no intraoperative complications. The average hospital stay was 4-5 days. None developed anastomotic dehiscence. Two patients had episodes of postoperative entetrocolitis and two patients who required abdominal mobilization developed stricture of the anastomosis. Follow up period ranged from 6 months to 25months (Mean 15.4months). Postoperative soiling was observed in all the patients that lasted from 2 to 6 weeks (Mean 3.4 weeks). None of the patients have voiding disturbances clinically and post void residual urine on ultrasound examination was normal in all the patients. Initial increased frequency of bowel movements has settled to 1-3 stools per day.
Conclusions- Single stage transanal Swenson’s pull through is a safe procedure and avoids problems of long muscular cuff.
Mircia-Aurel Ardelean, Jan Bauer, Christa Schimke, Barbara Ludwikowski, Christian Heil, Guenther Schimpl
Clinic of Paediatric Surgery, Paracelsus Medical University, Salzburg, Austria.
To estimate the bowel function in patients (pts) with anorectal malformations (ARM) reoperated in our department.
Material and Methods
Forty children (25 boys and 15 girls) and one adult with previously ARM repairs underwent surgery. Initially anomaly was low in 9 pts, intermediate and high in 32 pts.
Sacral anomalies were found in 23 children.
Different degrees of anal stenosis, misslocated rectum and neoanus, dilatation of the caudal colon or failure to diagnose the malformation at the time of primary operation were found in the 39 pts with incontinence. Two pts presented with complete stenosis of the neoanus.
Surgical therapy consisted on posterior sagittal anorectoplasty (PSARP) in 22 pts, posterior sagittal anorectovaginourethroplasty in 4, PSARP and total urogenital sinus mobilisation in 3 pts, PSARP and antegrade continent enema procedure (ACE) in 2, posterior sagittal anorectovaginoplasty in 1, ACE in 6 pts, sigmoid resection in 1, anaoplasty in 1, and definitive colostomy in 1.
Follow-up examinations (3 – 15 years postoperative) had 39 pts.
Eighteen patients are continent without constipation. Social continent are 20 pts: they need antegrade or retrograde enemas and some of them diet or medications to be clean. One pt had definitive colostomy.
Surgical therapy is the best alternative for most pts with faecal incontinence previously operated on anorectal malformation. Posterior sagittal anorectoplasty should be used as secondary procedure except for pts with correct placed rectum and without stenosis. In these last pts ACE, sigmoid resection or anoplasty may improve the faecal continence.
Philippe Montupet1, Frederic Gauthier1, Annabel Paye-Jaouen1, Sophie Branchereau1, Christophe Chardot1, Brigitte Bader-Meunier2, Gil Tchernia2
1-Pediatric surgery, Bicêtre Universitary Hospital, Le Kremlin Bicêtre, France.
2-Department of pediatrics, Bicêtre hospital
Two previous studies assessed the beneficial effect of subtotal splenectomy (STS) in children with hereditary spherocytosis (HS). This retrospective study is focused on indications, surgical aspects and long term follow-up.
From 1985 to 2005, 75 children underwent STS for HS at a mean age of 7 years. Indications were severe anemia in 60 (1 to 12 transfusions), chronic anemia and fatigue in 15. Cholelithiasis was present in 28. A short transverse laparotomy, preceded by video-dissection in 3 cases was used. STS preserved the lower pole in 73 patients. Remnant fixation was performed 39 times, associated cholecystectomy 29, drainage systematically. Preoperative vaccinations and antibioprophylactic regimen were the same as for a total splenectomy.
Two hemorrhages required a second look, without splenectomy.Three splenic remnants (4%) were lost early after surgery. The 72 other patients kept a functioning spleen (assessed by 51Cr scinti-scan and pitted erythrocytes count) and maintained for years a mean hemoglobin level of 12g/dl, in spite of the splenic remnant regrowth. However 7 patients needed completion of splenectomy, 2 to 8 years after STS, because of reccurent anemia. In 4 cases, a secondary splenopexy was performed to treat a wandering spleen. Cholecystectomy for delayed cholelithiasis concerned 8 children. No overwhelming infection occured.
Our twenty years experience confirms that open STS is a safe and valid therapy for severe forms of HS. It requires to be careful of vascular dissection and transsection in order to preserve vascular supply of the splenic remnant. Fixation and drainage are recommended.
2nd Surgical Department, Aghia Sophia Children’s Hospital, Athens, Greece.
Congenital splenic cysts are rare clinical entities. Diagnosis is usually incidental unless symptoms occur due to complications requiring treatment. Interval partial splenectomy following percutaneous drainage of the cysts has been suggested for the treatment of complicated splenic cysts. Two cases of complicated congenital splenic cysts submitted to one stage partial splenectomy following their clinical manifestation are presented.
Material and Methods
During the period 1996-2005 six children were admitted for the surgical management of congenital splenic cysts. In two complications had ensued leading to operation. The other four were discovered incidentally. One of the former two was infected by Salmonella Paratyphi. The second presented with abrupt enlargement of the cyst mimicking acute splenomegaly. Abdominal ultrasongraphy revealed the existence of all the cysts leading to either urgent or interval operation. Content aspiration was followed by removal of the cystic wall using the Ligasure regis Vessel System. The splenic artery ligation precceded partial splenectomy in the patient with the infected splenic cyst.
Postoperative course was uneventful and both patients were discharged on the 4th postoperative day. Scintigraphy of the spleen at six months, as well as measurement of the complement and immunoglobulins showed normal splenic function.
The management of complicated congenital cysts does not require total splenecomy. The use of Ligasure regis Vessel System for their management is safe and decreases significantly the duration of hospitalization.
Alastair Millar, Girish Gupte, Sue Beath, Khalid Sharif, Darius Mirza, Sara Clarke, David Mayer, Carla Lloyd, Jean de Ville, Deirdre Kelly
Hepatobiliary Surgery,Hepatology and Transplantation, Birmingham Children, Birmiingham, West Midlands.
Introduction:There is a need for effective management of intestinal failure (IF) and intestinal failure associated liver disease (IFALD) including transplantation.
Aim: To review the trend in referrals and progress made in transplant management of children with IF and IFALD.
Subjects and Methods: 226 patients with IF and/or IFALD were referred for assessment for transplantation between 1992 and 2005. 91 were recommended for transplant (28 died on waiting list and 4 improved). Diagnoses were Atresia (5), Gastroschisis (33); Intestinal aganglionosis (9); Microvillus inclusion disease (6); Necrotising enterocolitis (11); Pseudo-obstruction (14); Mid gut volvulus (2); Others (11). 57 were transplanted of whom 17 were isolated liver (ILTx) for IFALD, 2 as a bridge for later intestinal transplant (ITx) and 15 for short bowel with the expectation of eventual full adaptation. 41 were ITx (9* isolated small bowel (ISBTx) and 32 combined liver and small bowel (CSBLTx); 15 whole liver and intestine and 17 with size reduction of liver and/or bowel. The median age at transplant was 3.4yrs (range 6mnths to 16yrs).
Results: Overall 29 are alive. 27 are on full enteral feeds with one immediately post transplant (8/10 ILTx, 3/3 ISBTx & 16/16 CSBLTx). Actuarial survival with up to 7 year follow-up is 64% for ILTx, and ~50% for intestinal transplants.
Conclusions: Recently there has been an increase in referrals and transplants for IF. Survival in good health on full enteral feeds can now be achieved in the majority of those transplanted.
* One child had a re-transplant
Anna Poupalou, Pantelis Nikolaides, Michalis Sklavos, Georgios Petousis, Spyros Kosteletos, Poli Maniati, Eleni Kosmidi, Emmanouhl Androulakakis
Peadiatric Surgery, Athens, Greece.
AIM: The aim of our study is to show that the combination of chemiotherapy and surgical treatmnent is very efficient in the treatment of hepatoblastoma, a potenzially letal tumor.
MATERIAL AND METHODS: We involved three children in our study: a 2 years old boy, an one years old girl and a boy of 50 days. All of them had enormous tumor visualized by abdominal Ultrasound, CT or MRI. Levels of a-fetoprotein were very high at the moment of diagnosis. All children were treated first with chemiotherapy in base of international protocolls until the tumor became resectable. Right or left hepatectomy was the surgical treatment. All children had chemiotherapy after surgery.
RESULTS: The combinated treatment was succesfull in all cases. A- Fetoprotein is important in the monitoring of the patients. All children are healthy 2 years, 3 years and 10 months later.
CONCLUSION: the combination of chemiotherapy and surgery can save patients with hepatoblastoma.
S. Guindos1, J. Bueno2, V. Martínez-Ibáñez1, J. Sánchez de Toledo3, C. Sabado3, J. Balsells4, A. Escartín5, C. Margarit5
1-Pediatric Surgery, Hospital Vall d´Hebrón, Barcelona, España.
2-Pediatric Surgery Department and Liver Transplantation Unit
3-Pediatric Oncology Department
4-Pediatric Intensive Care Unit
5-Liver Transplantation Unit
INTRODUCTION: Liver neoplasies are 1% of pediatric cancer. Liver Transplantation (LT) is the unique rescue therapy in those liver cancer without response to Chemotherapy (Ch) and surgically unapproachable.
AIM: Retrospective analysis of our experience in LT and liver malignancies.
MATERIAL AND METHODS: In 166 pediatric LT, hepatic malignancies were found in 8 (4’8%). Mean age at transplantation was 4’5 years (range 7 months-11 years). There were 3 Hepatoblastoma (HB) and 5 HC. One of the HB also presented with portal vein thrombosis and 2 lung methastasis (M). In 4 cases (group A) LT was indicated after stablishment of irresecability and all of them received previous Ch. In the other cases (group B) malignancies were found as an incidental finding in the analysis of the explanted liver; none of them were treated with previous Ch. LT indications in these cases were: 2 thyrosinemia, 1 autoimmune hepatitis and 1 billiary atresia. There were 4 whole organ LT and 4 partial.
RESULTS: 6 of 8 (75%) are alive with an average follow up of 6’3 years (range 5 days-17 years). In group B, one child need retransplantation after untractable chronic rejection. Other patient died 5 days after LT due to primary graft disfunction.In group A, the patient with HC died 16 months after LT because of brain M. 9 months after LT he need lung M resection surgery.
CONCLUSIONS:LT in unresectable liver malignancies can be an effective rescue treatment, as it is in chronic hepatopathies and HC.
Santiago Guindos, Javier Bueno, Alfredo Escartin, Carlos Margarit, Vicenç Martínez-Ibáñez
Pediatric Liver Transplantation Unit, Hospital Vall d´Hebrón, Barcelona, España.
INTRODUCTION: Hepatic hemodynamic changes during liver transplantation (OLTX) in children have not yet been studied and their clinical significance is unknown.
MATERIAL AND METHODS: Portal vein flow (PVF) and hepatic arteria flow (HAF) (ml/min) were measured intraoperatively in 58 children with ultrasonic flow probes during OLTX. Flows were adapted to recipients weight. Initially, flows of native liver were measured after hilar dissection, and allograft flows immediately after graft reperfusion and one hour later. Type of grafts included: whole liver (n=28) and left-lateral segment (n=30).
RESULTS: There were no differences between mean HAF and PVF in native liver. After graft reperfusion, HAF remain similar however PVF dramatically increases (P=0.00). One hour later, AHF increases (P=0.00) while PVF remains stable. No flow differences were seen between whole and partial grafts. In biliary atresia the native PVF is poorer and graft HAF higher if compared to others diseases. There were 3(5%) arterial thrombosis and 8(14%) portal vein thrombosis which occurred in the first 72h (n =6) and at 15 days (n=2). In portal vein thrombosis group PVF was lower in both, native liver and graft than in no thrombosis group (P< 0.05). No graft loss occurred due to PV thrombosis.
CONCLUSION: In children, PVF and HAF measurements during OLTX may predict high risk patients for portal vein thombosis. In cirrhosis PVF and HAF are similar, and after transplantation return to a physiological situation. Patients with biliary atresia can tolerate early portal vein thrombosis due to the high graft HAF.
Agustín Cañizo1, Ana Laín1, Alberto Parente1, María Fanjul1, Julio Cerdá1, Elena Cela3, Paloma Galarón2, Felipe Calvo3, Juan Vázquez1
1-Servicio Cirugía Pediátrica, Hospital Infantil Gregorio Marañón, Madrid, España.
2-Servicio Pediatría. Hospital Infantil Gregorio Marañón
3-Servicio Oncología Radioterápica. HGU Gregorio Marañón. Madrid. España
INTRODUCTION: Intraoperative radiotherapy with displacement of adjacent radiosensitive organs out of the treatment field allows a better local control of tumors without extensive surgical resection and reduce the radiation dose.
BACKGROUND/PURPOSE: To report our experience with the intraoperative radiotherapy (IORT) use for the treatment of advanced stages of pediatric malignancy.
MATERIAL AND METHODS: Eighteen pediatric patients aged from 1 month to 16 years, seven with an advanced stage neuroblastoma (stage III, IV), five with Ewing Sarcoma, one Rhabdomyosarcoma (stage III), one Sarcoma Sinovial (Stage II) , one released dysgerminoma and three fibromatosis treated with IORT were reviewed retrospectively. All of them underwent tumoral excision surgery with single IORT doses of 500-1250 cGy, energy of 4-18Mev and application size from 5 to 15 cm. . Eight patients also benefited from external beam irradiation (EBRT) (dose 2000-5000 cGy). The mid follow up was 50 months.
RESULTS: Ten patients (4 neuroblastoma, 3 Ewing sarcoma, , 1 dysgerminoma and 2 fibromatosis) are alive and disease free.
One Neuroblastoma and the sinovial sarcoma had local control but diseminated disease. There were no IORT associated complication.
CONCLUSIONS: Intraoperative radiotherapy is a safety procedure which appears to enhance local control with supervivence improvement. IORT merits future exploration by prospective study
Rocío Granero Cendón, Iñigo Tuduri, María José Moya, Rocío Granero, Israel Fernández-Pineda, Rosa Cabello, Juan Carlos de Agustín
Paediatric Surgery, Hospital Infantil Virgen del Rocío, Seville, España.
Pulmonary blastoma is a recently described entity, showing blastemal, sarcomatous and epithelial elements. It accounts for 0.5% of all primary malignant lung tumours. It is associated with other infantile embryonic tumours and congenital cystic adenomatoid malformation.
To study the epidemiology, clinical presentation and treatment of blastomas in a tertiary centre.
MATERIAL AND METHODS
We review the medical records of all patients diagnosed of blastomas since 1974 to 2006. Demographical variables and clinical data are reviewed.
Diagnostics methods and treatment results are also analysed.
From nine primary pulmonary tumours 4 were true blastomas. Mean age was 3 years at the time of diagnosis. All presented with fever and respiratory distress (cough, dyspnoea).The patients started to be symptomatic by 5.7 months of life. Diagnosis was done by thoracoscopy (1), and by pre or intraoperative biopsy (3). We identified 3 type III and 1 type II cases.
There was no metastastatic disease. Extended surgical resection was performed in all cases along with pre and postoperative chemotherapy with VAC (vincristine, actinomicyn, cyclophosphamide). Survival in our series was 50%.
- Pulmonary blastoma is a rare tumour that simulates many other benign
respiratory conditions but do not respond to standard treatment protocols.
- Early diagnosis by thoracoscopy allows preoperative cytoreductive QT.
- It is a very chemo-sensitive tumour, but a generally accepted protocol is lacking. They are also very radiosensitive tumours, but more studies are needed.
Ingo Königs (1), Marion Wilmsmeyer (2), Katrin Zahn (1), Dorothee Friedrich (2), Ahmed Hadidi (1), Karl-Ludwig Waag (1), Ingo Jester (1)
1-Department of Pediatric Surgery, University Hospital Mannheim, Mannheim, Germany.
2-Department of Pediatrics, University Hospital Mannheim, Germany.
The management of necrotizing enterocolitis (NEC) is still controversial. Especially in very low birth weight infants (<1000g) in bad general condition there are encouraging results regarding the mortality of peritoneal drainage reported as exclusive therapy in comparison with primary resection of affected bowel with enterostomy or primary anastomosis. Aim of our study was the evaluation of our standard management which is enterostomy without primary bowel resection.
From 2000 to 2005 38 neonates were treated with NEC. The clinical course was examined retrospectively with particular attention to mortality, time of enteral feeding, early complications, number of operations and stay in hospital. The results are discussed with data in the current literature.
30 children (80%) had to be treated operatively. 18 children were less than 1000g at birth (mean 675g) and 12 more than 1000g (mean 1875g). In all a double barrel enterostomy was performed shortly proximal to the affected area without primary resection and without further preparation to avoid mobilization of toxic substances and to limit the operating time and stress. The rules followed in general surgery in these conditions were abandoned on purpose. As a main result the overall-mortality was 13%.
The operative treatment of NEC with primary enterostomy without resection is a good option. The mortality with this treatment is lower than in other series (18-61%). Avoiding the resection of bowel the extent of the primary operation in the acute period is reduced dramatically and also the risk of a short bowel syndrome is minimized.
Alon Yulevich1, Ilan Erez2, Jorge Mogilner3, Yechiel Sweed1
1-Department of Pediatric Surgery, Western Galilee Hospital – Nahariya, Nahariya, Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel.
2-Department of Pediatric Surgery, Meir Medical Center, Kfar Saba, Sackler Faculty of Medicine, Tel-Aviv University, Tel-Aviv, Israel.
3-Department of Pediatric Surgery, Bnei Zion Medical Center, Haifa, Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel.
Objective: Presenting the long survival of preterms suffering pan-necrotizing enterocolitis.
Method: Revising patients’ files from 1.01.2000 to 31.5.2006, at 3 medical centers.
Results: Five patients were found from October 2003 who survived longer than 3 weeks. Gestational age was between 30-34 weeks. Birth weight range was 930-2105g. Symptoms appeared at ages 4-18 days. All patients underwent explorative laparotomy and extensive necrotic bowel from the duodenum to the colon was found. In all patients the abdomen was either closed as is or necrotic intestine was resected followed by a second look laparotomy within 48 hours. The same conclusion was reached for all the preterm patients that nothing can be done and the baby will shortly die.
However, all patients survived and died only 3-9 weeks post the surgery, either from liver failure or sepsis. One patient was operated again 3 weeks after the first laparotomy because of the parents’ wish to ensure that nothing more could be done. Indeed, only disintegrating intestine was revealed.
Conclusion: The occurrence of preterm surviving post pan-necrotizing enterocolitis is an ethical and surgical challenge for pediatric surgeons and seems not to be anecdotal anymore. The rising incidence is probably due to better treatment in the neonatal intensive care units. It is very difficult – if at all possible – to discontinue any part of the full treatment these babies already receive. The infants are too small and young to be candidates for intestinal transplantation.
Jai Mahajan, Money Gupta, Kattargadda rao
Pediatric Surgery, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
AIM- To study the effect of pleural wrap in preventing anastomotic dehiscence in patients with esophageal atresia (EA).
METHODS AND RESULTS- Thirty consecutive patients of EA and lower pouch fistula undergoing primary repair were studied randomly in two groups. Group-I patients underwent conventional surgery for EA whereas in Group-II patients with longer gap length, an additional cover of the parietal pleura was provided around the anastomosis.
Group-I included 16 patients. Associated anomalies were present in 9 (56percent) and 5 (55 percent) patients had cardiac anomalies. Mean gap length was 1.62cm (range 0-3 cms). Lower pouch mobilization was performed in 4 (25percent) patients. Eleven patients required ventilatory support varying from 1-23 days (mean 4.7 days). Anastomotic leak occurred in 4 (25percent) patients, out of which 3 had a gap length of 2.5-3 cms. Two patients with major congenital heart disease died.
Fourteen patients in Group-II had associated anomalies in 6 patients and 5 (83 percent) of them had cardiac defects. Mean gap length was 3.2cms (2-4.5cm). Lower pouch mobilization was required in 10 (71percent) patients and 11 patients were on ventilator postoperatively for mean of 11.8 days (3days to 1month). Three (21 percent) patients had anastomotic leak and two of them died.
CONCLUSIONS- Major cardiac defects are more commonly associated with longer gap EA patients. The leak rate for group-II patients was lower; hence, the pleural wrap prevented anastomotic leakage in some patients with wide gap length.
Ingo Königs (1), Thomas Schaible (2), Ulrike Subotic (1), Steffan Loff (1), Hartmut Wirth (1), Ahmed Hadidi (1), Karl-Ludwig Waag (1)
1-Department of Pediatric Surgery, University Hospital Mannheim, Mannheim, Germany.
2-Department of Pediatrics, University Hospital, Mannheim, Germany
The role of ECMO on Congenital Diaphragmatic Hernia (CDH) survival is still controversial. In this study we examined the use of ECMO and its effect on CDH survival in Mannheim, Germany over the last 15 years (1991-2005).
280 patients with diaphragmatic hernias were treated in our institutions. Due to different management protocols and changes in neonatal intensive care medicine this period was divided into 3 periods of 5 years. From 1991 to 1995, the unit received 38 patients with CDH, 19 requiring ECMO. 1996-2000 58 patients were admitted in the unit and ECMO was utilized in 29 patients. 2000-2005 184 babies with diaphragmatic hernias were treated, 88 patients with ECMO.
The number of CDH referred to the unit was nearly increased five times (38→184). The number of patients requiring ECMO was more or less the same (~50%). The number of patients who died after ECMO was reduced form 66% to 36%. The overall mortality in CDH was reduced from 50% (91-95) to 20% (01-05). Most of the patients who died in the last 5 years were in a bad general condition and died before CDH-closure.
A strong association exists between employment of ECMO and improved survival in CDH. The study also shows the importance of referral to a specialised unit in order to develop experience and improve survival. The study showed that although 50% of patients with CDH required ECMO during the different time periods, the mortality and morbidity was reduced effectively.
Ane Andrés1, Jose Luis Encinas1, Federica Pederiva1, Ana Lourdes Luis1, Beatriz Herrero2, Alfonso Fernandez1, Ramon Policart1, Jose Miguel Mariño1, Luis Lassaletta1, Juan Antonio Tovar1
1-Cirugía Pediátrica, Hospital Infantil La Paz, Madrid, España.
2-Obstetricia y Ginecología Hospital La Paz. Madrid.
BACKGROUND: The possibility of postnatal regression, the hidden mortality and the timing of postnatal management of congenital cystic adenomatoid malformation (CCAM) are still often issues. We review our experience aiming at proposing attitudes.
PATIENTS AND RESULTS: In the last 11 years we treated 17 patients with prenatal diagnosis of CCAM. Five had an abortion after diagnosis prior the 20th week of gestation. Two women miscarriaged spontaneously between athe 20 and 32th week with anasarca and ascitis. Two newborns died before 24h of life with hydrops fetalis. There was 1 case of CCAM type III diagnosed at the 20th week of gestation that was born without problems and nowadays has no symtoms. Fourteen patients, 7 with and 7 without prenatal diagnosis, were operated. Median age at the surgical procedure was 8 months (range 1 week-30 months). Lobectomy was made in 13 cases and segmentectomy in the remaining one. After a follow-up period of 4.3 years (range 1-9.5)the only complication was a slight pectus excavatum deformity.
CONCLUSIONS: More than 50% of children with CCAM die before coming to operating room. Prenatal diagnosis may detect this malformation in 86% of the cases. The lesions can be limited or dissapear. When hydrops fetalis with severe cardiac displacement and ascitis is detected, fetal surgery may be indicated. Children who were operated during the postnatal period have and excellent prognosis.
Israel Fernández-Pineda, Rosa Cabello-Laureano, María José Moya-Jiménez, Rocío Granero-Cendón, Íñigo Tuduri-Limousin, Carmen García-Vallés, Ana Millán Lopez, Juan Carlos de Agustín-Asensio
Paediatric Surgery, Hospital Infantil Virgen del Rocío, Sevilla, España.
Background. Long term studies of patients treated of Congenital Cystic adenomatoid malformation (CCAM) are lacking. We present our 30 years experience and long term results in a paediatric population.
Material and methods. The medical records of all surgically treated CCAM patients since 1976 to 2006 were reviewed. Surgical results and follow up were studied through personal interview and telephone calls.
Results. Twenty-four patients (16 males and 8 females) were studied. Mean age: 5.4 years old (1day to 9 years). Respiratory distress was the most relevant symptom in infants and neonates, but chronic coughing and recurrent infections in the older. Chest X-ray was suggestive, but CT scan was more specific of CCAM. Lobectomy was done in all cases. Stockes CCAM type 1 (n = 14), type 2 (n = 8) and type 3 (n = 2). Associated anomalies were: Broncogenic cyst, anomalous systemic artery, Double aortic arch, and massive chylothorax. Complications were: one pneumothorax and one postoperative pneumonia. One type 3 patient died of massive chylothorax.
In the long term follow up (6 months to 30 years) all except one patient are alive with good lung function and no malignancy was detected so far.
Conclusions. CCAM is a rare but clinically relevant entity. Lobectomy was determinant to obtain excellent results avoiding later recurrences. Low mortality, and neither malignancy nor any other significant complications were observed in these series. Quality of life is good in all. We recommend early treatment in life to obtain full survival and good long term results.
Juan Carlos de Agustin-Asensio2, Ruben Greco-Martinez2, Manuel Garrido-Morales1
1-Paediatric Surgery, Hospital Infantil Univeristario Virgen del Rocio, Seville, Spain.
2-Paediatric Cardiothoracic Surgery, Hospital Infantil Universitario Gregorio Marañón, Madrid, Spain
INTRODUCTION: The purpose of thoracoscopy is to perform surgical techniques through a minimal access surgery (MAS), improving visualisation and hastening postoperative recovering. However in some cases thoracoscopy is impractical or adds unacceptable risks over standard approaches.
OBJECTIVES: We explore alternatives of MAS others to thoracoscopy and to define the limits between them.
MATERIAL AND METHODS: We review the experience of two tertiary centres in cardiothoracic surgery. Three MAS techniques were evaluated: Bianchi-Atkins axilar thoracotomy (n=7), diaphragmatic plication (n=4) and lung biopsies (n=17).
RESULTS: Five neonates and 2 infants (from 25 weeks of gestation (12 days of life) to 5 months) were underwent axilar thoracotomy to treat Persistence Ductus Arteriosus and Pulmonary Banding. Four infants underwent diaphragmatic plication through a 3 cm. minithoracotomy and 5 mm optical thoracoscopic assistance. Two infants with poor clinical condition underwent lung biopsies through minithoracotomies. Their data were compared to 15 thoracoscopic lung biopsies. There were no complications. Advantages obtained were: excellent visualization, low cost (standard nonthoracoscopic surgical material was used), short operating time, inconspicuous scars, and analgesia. Disadvantages: a vertical working field must be getting used to.
CONCLUSIONS: MAS other to thoracoscopy exists, and obtains the same objectives than pure thoracoscopic techniques. It is indicated in the very premature babies and in the very poor condition ones that do not support thoracoscopic management. They can also be done at the intensive care units.
Marcos Bettolli, Carl Christian Jackson, Brian Sweeney, Steven Rubin
General Pediatric Surgery, Children’s Hospital of Eastern Ontario, Ottawa, Canada.
Background: Whereas congenital Morgagni (anterior) diaphragmatic hernias account for 2-6 % of diaphragmatic defects, acquired hernias, especially intrapericardiodiaphragmatic hernias are rare. These hernias are most often the result of blunt or penetrating trauma. Symptoms are often absent for prolonged periods. Incarceration of peritoneal content including omentum, transverse colon, stomach, small intestine and liver may present as cardiac tamponade, an acute abdomen or intestinal obstruction. Urgent repair is indicated.
Methods: Two patients ages 7 and 12 months were diagnosed with IPDH. Both had undergone cardiac surgery including the placement of subxiphoid pericardial drain tubes. Postoperative chest X-ray at two months showed an IPDH in one child. In the second patient at eleven months post cardiac surgery during laparoscopic assisted PEG, incarcerated omentum was noticed in an IPDH.
Results: Both patients had uneventful laparoscopic repair of the hernias, with out recurrence
Conclusion: This is the first report of IPDH in childhood. Placement of subxiphoid pericardial drains may result in IPDH. A high index of suspicion is necessary to avoid missing this potentially serious and possibly undiagnosed complication after cardiac surgery.
Ane Andrés, Ana Lourdes LUIS, Jose Luis Encinas, Laura Burgos, Olga Suárez, M. José Martínez_Urrutia, Enrique Jaureguízar, Luis Lassaletta, Juan Antonio Tovar
Pediatric Surgery, Hospital La Paz, Madrid, España.
We report on a set of female twins in whom prenatal diagnosis of omphalopagia with both bladders empty was made. They were delivered by C-section. At birth they were joined by a bridge that involved a omphalocele and an exstrophied bladder. They had no anus and a single urogenital orifice.The only digestive orifice was located in the middle of the exstrophied bladder.
The twins were separated in the first day of life. Both small bowels converged in a single terminal ileum which opened into a single colon. Each twin had a urogenital sinus with mullerian duplication and 2 kidneys that were fused in one of them.
The intestinal plate was separated from the bladder, the omphalocele was closed completing the separation and colostomies were fashioned using the proximal half of the colon with the corresponding ileum in one and the distal part of the colon anastomosed to the ileum in the other one.
One year later we performed urogenital separation and vaginal and colonic pullthroughs. At the age of 5 years the patients are continent for feces with only rare accidents. One is clean for urine with intermittent bladder catheterization and the other one is able to void spontaneously without continence problems. The vaginas are patent. They are healthy and happy although the road until this stage has been difficult for the girls and their parents.
Separation of conjoined twins involve all fields of pediatric surgery and is very demanding in terms of surgical skills, long-term functional management and psychological support.
Santiago Guindos (1), Jose Luis Peiró (1), Josep Lloret (1), Claudia Marhuenda (1), Mario Gómez (2), JF De Luis (2), Félix Castillo (3), Elena Carreras (4), Nuria Torán (5), Vicenç Martínez-Ibañez (1)
1-Pediatric Surgery Department, Hospital Vall d’Hebron, Barcelona, España.
2-Hospital Universitario N. S. Candelaria. Santa Cruz de Tenerife. Spain.
3-Neonatology division. Hospital Vall d’Hebrón. Barcelona. Spain.
4-Fetal Medicine Unit. Hospital Vall d’Hebrón. Barcelona. Spain.
5-Pathology Division. Hospital Vall d’Hebrón. Barcelona. Spain.
Gastroschisis (GS) permits bowel exposure to amniotic fluid (AF). Intestinal damage is related to the chemical action of AF producing thickened intestinal wall and atresias at term. Surgical intestinal reubication may be difficult. Patients require a long hospital stay and total parenteral nutrition (TPN) owing to intestinal hypoperistalsis. Our aim is to evaluate the benefits of a preterm delivery.
METHODS: Fourteen patients with GS have been treated since 1998. Seven cases of prenatally-diagnosed GS have been treated following a new strategy since July 2002. A preterm c-section was programmed at 34 weeks of gestational age (GA). After birth, bowel reduction was performed under general anesthesia. This preterm group (PT) was compared the past 7 cases at term (AT). Macroscopic appearance, atresia existence, surgical technique, neonatal outcome, TPN and hospital stay were analyzed.
RESULTS: All cases AT (mean GA: 36.3 weeks) presented bowel inflammation. Only 3/7 cases allowed the intestine to be housed in a primary closure after laparotomy and 4/7 required staged silo repair. Oral feeding was started at 27.5 days. Mean TPN was 42.3 days and mean hospital stay 71.2 days. After elective c-section delivery (mean GA: 34.8 weeks) bowel loops presented normal appearance. No prematurity-related complications were observed. Easy bowel reduction was performed. Oral feeding was started at 6 days. Mean TPN was 14.7 days and mean hospital stay 26.6 days.
CONCLUSIONS: Preterm delivery in GS avoids intestinal damage, prevents “peel” and intestinal atresia, renders surgical reduction easier, reduces hypoperistalsis, need for TPN and hospital stay. Multidisciplinary coordination is required.
Jose L. Peiró, Félix Castillo, Elena Carreras, Josep Lloret, Claudia Marhuenda, Goya Enriquez, Nuria Torán, Vicenç Martinez-Ibañez
Fetal Surgery program. Hospital Vall d’Hebron. Barcelona. Spain.
Background: Several congenital malformations as giant cervical fetal masses, laryngeal atresia or therapeutic tracheal occlusion in congenital diaphragmatic hernia (CDH) can obstruct the high airway, causing vital risk in the newborn for asphyxia neonatorum. The EXIT (EX-utero Intrapartum Treatment) procedure offers the surgeon enough time during the childbirth to assure the airway of the fetus. We present the fetal cases that, after the prenatal diagnosis of suspected airway obstruction, could have benefited of this procedure.
Methods: Thirteen fetal patients with foreseeable neonatal airway obstruction submitted to EXIT have been reviewed. An EXIT procedure is carried out supporting the uterine relaxation, to guarantee the placentary flow and the fetal oxygenation.
Results: Thirteen fetal cases (2 teratomas, oropharyngeal and cervical, 1 cervical lymphangioma and 10 CDH ) were prenatally evaluated. An EXIT procedure was planned in all of them after predict an airway compromise. In the 10 CDH cases, removal of a tracheal balloon were performed. A nasotracheal intubation, fetal tracheostomy and surgical partial resection was required in the cervical mass cases. Time spent in the procedure was a mean of 21 minutes. All cases could be ventiled after birth with the airway secured.
Conclusions: The intrapartum surgery with placentary support offers the possibility of controlling a fetus during the birth, when this one still does not depend on his breathing. Prenatal diagnosis allows to anticipate us and to assure the airway by EXIT procedure, and this way to avoid hurried neonatal actions that put in vital risk the newborn child.
Anna Poupalou, Michalis Sklavos, Pantelis Nikolaides, Loukas Prezas, Eleni Kosmidou, Nikolaos Anastasiou, Emmanouhl Androulakakis
Peadiatric Surgery, A.&P. Kyriakou Childrens Hospital, Athens, Greece.
INTRODUCTION: The aspergollosis in immunodepressed children is a potentially letal illness. The farmaceutical treatment only is not allways sufficient. V.A.T.S is gaining place in the treatment of these patients.
AIM: The aim of our study is to show the efficacy and utility of the v.a.t.s. in the escission of the polmonar aspergilloma.
MATERIAL AND METHODS: Three immunodepressed children (4, 8 and 16 years old)underwent thoracoscopy for diagnostic and terapeutical reasons. The sfenoid escission of the polmonar aspergilloma was succesfull in all cases. All patients had farmaceutical treatment before and after thoracoscopy.
RESULTS AND CONCLUSIONS: The combination of farmaceutical treatment and thoracoscopy can saved the life of 3 immunodepressed children
Gabriela Guillen1, Claudia Marhuenda1, Santiago Guindos1, Josep Lloret1, Pilar Bastida2, Vicenç Martínez-Ibáñez1
1-Pediatric Surgery, Vall d´Hebron Hospital, Barcelona, España.
2-Paediatric Haematology, Vall d´Hebron Hospital, Barcelona, España.
Invasive pulmonary aspergillosis (IPA) reaches high mortality rates (30-90%) in inmunocompromised patients. There is short experience in paediatric patients with the combination of antifungal drugs and surgery.
OBJECTIVES: To compare the survival of paedatric inmunocompromised patients with IPA that followed or not surgical resection of the lesions. To review the different surgical techniques applied.
PATIENTS AND METHODS: All the cases of IPA found in the register of our center since 1995 until 2005, were reviewed. We compared the results between operated and non operated patients, as well as analyzed factors associated to a better prognose.
RESULTS: Sixteen patients were included, between 1 and 17 years old. The most frequent underlying diseases were acute leukemias (mieloblastic 50% and lymphoblastic 18%). Seven patients underwent surgery (7 atypical resections and 1 lobectomy). Nine patients received only medical treatment. Ten patients died, 2 operated and 8 non-operated; in 5 of them the death cause was attributed to IPA. The mean survival from diagnose is 420 days in operated and 35.8 in non-operated. The operated patients remained asympthomatic and Aspergillus-free. There were only 2 surgical complications. After surgery, seven children received further cytotoxic treatment and four received bone marrow trasplantations. The only statistically significative variable regarding survival was surgery.
CONCLUSIONS: The antifungal treatment must be started aggressively, and if there are focused lesions that do not response to medical treatment, we recommend surgical resection.
The removal of the lesions offers a better survival for the inmunocompromised child, as solves the infection and allows future treatments.
Anna Poupalou, Georgios Petousis, Michalis Sklavos, Pantelis Nilolaides, Spyros Kosteletos, Poli Maniati, Aristeidis Prapas, Theodoros Dionisis, Vasiliki Gkoulioni, Emmanouhl Androulakakis
Peadiatric Surgery, A.&P. Kyriakou Childrens Hospital, Athens, Greece.
INTRODUCTION: Parapneumonic effusions constitute a rather common coexisting presentation of microbial pneumonia, whereas empyemas are considered as complication of the disease.
AIM: The aim of our study was to prove the necessity and utility of thoracic paracentesis or the positioning of a drainage tube and/ or surgical treatment ( thoracoscory or thoracotomy).
MATERIALS AND METHODS: We included in our study 25 children with complicated parapneumonic effusion caused by pneumonia. All children received antibiotics and underwent surgical treatment. The ultrasonografy was very important in the valuation of the effusion.Of our patients 3 had only paracentesis, 15 needed a drainage tube, 5 underwent video assisted toracic surgery and 3 of them had thoracotomy. The choice of the treatment was based on the dimension of the parapleural effusion, its type,the duration of the illness, the toxicity of the microbs, the state and age of the little patient.
RESULTS: Antibiotics and surgical treatment salved little patients.
Concepció Barceló1, Claudia Marhuenda1, José Luis Peiró1, Pere Soler2, Josep Lloret1, Viçens Martínez-Ibáñez1
1-Pediatric Surgery , Hospital Vall d’Hebron, Barcelona, España.
2-Pediatric Infectious Disease. Vall d’Hebron Hospital. Barcelona. España
Objectives: Loculated parapneumonic empyema (PPE) is nowadays a frequent complication of acute bacterial pneumonia in children. There is no consensus regarding the ideal treatment of PPE.The aim of this study was to compare the effectiveness of two different treatments: video-assisted thoracoscopy (VATS) and drainage plus urokinase (DU).
Methods: Prospective non-randomized study of all the patients admitted from January 2001 to March 2006 at a single institution with a parapneumonic empyema. Loculated empyema was diagnosed by chest sonography and patients were treated with DU or VATS depending on the surgeon’s preference. Compared variables were: total hospital stay, days of tube thoracostomy, postoperative fever and postoperative stay. Data were compared using one way analysis of variance.
Results: Sixty four patients (4 months to 14 years) were included in the study. Thirty seven (58%) were treated with VATS and 27 (42%), with DF. No statistically significant differences (p>0.05) were found between VATS and DU for hospital stay (11.6 vs 13.56), duration of tube thoracostomy (4.62 vs 6.44), postoperative fever (3.57 vs 5.63) or postoperative stay (9.7 vs 12.3). One patient of group VATS needed a second drainage procedure and one, a third. In the group DU, four patients needed a second procedure and 1, a third.
Conclusions: According to our experience, the results of VATS and DU are similar for the treatment of loculated empyema. Nevertheless, it seems to be a higher rate of failure, with the need of more procedures in the DU group. The lack of randomization is the main limitation of this study, and it could exist a bias in treatment selection, with a tendency to perform VATS in the most complicated cases.
Katrin Zahn1, Ingo Koenigs1, Ulrike Subotic1, Steffan Loff1, Karl-Ludwig Waag1, Stuart Hosie2
1-Kinderchirurgie , Universitätsklinikum Mannheim, Mannheim, Germany.
2-Kinderchirurgie, Krankenhaus München Schwabing, München, Germany
The necessity of bladder augmentation in paediatric patients is a dilemma since it might be the only solution to prevent renal insufficiency but is also prone to longterm complications. We report on a girl, who suffered from neurogenic bladder following surgical reconstruction of a high anorectal malformation performed elsewhere. Over the years bladder capacity decreased to 50 ml and vesicoureteral reflux with recurrent urinary tract infections and urinary incontinence caused persistent problems. Sonography at the age of 12 years showed a large cyst in the lower abdomen. A MRI-scan was performed for further diagnostic work-up. It revealed a duplex uterus and vagina with one being enlarged by hematocolpos due to vaginal atresia. After ipsilateral salpingo-hysterectomy an augmentation cystoplasty using the dilated vagina (vaginocystoplasty) was performed without complications. The augmented bladder has a very good capacity of 360 ml. Until now we have not observed any electrolyte-derangements or acid-base-imbalance. With clean intermittent self-catheterization the patient is continent and able to live an almost normal life. Follow-up is nine months so far. To our knowledge this procedure has not been reported for paediatric patients in the literature before. In this particular complex malformation this technique was very useful to perform a bladder augmentation without the early complications of an enteroplasty. Longterm-follow-up is essential to detect eventual complications later.
Mircia-Aurel Ardelean, Christa Schimke, Joerg Schnoell, Guenther Schimpl
Clinic of Paediatric Sugery, Paracelsus Medical University, Salzburg, Austria.
To evaluate the continence and the urinary tract function in patients (pts) with cloaca.
We did surgery in 32 girls with cloaca, 3 months to 16 years old (07/90-06/04).
Group A. In 12 pts urethral and vaginal reconstructions and the pull-through of rectum were done together at primary operation. 5 pts had VUR, 3 neurogenic bladder (NB), 2 solitary kidneys. Bladder augmentation n=1 and VUR-correction n=2 were done as secondary operations.
Group B. Other 20 pts had elsewhere surgery onto cloaca. They presented with urogenital sinus (UGS) n=14, urethral pathologies n=4, reconstructed functional urethra n=2. Other urinary pathologies: VUR n=13 pts, NB n=7, solitary kidneys n=6, megaureter n=4. Only 2 pts were continent. Urethral reconstruction n=14, bladder augmentation/substitution n=5, vesico-cutaneostomie n=5, VUR-correction n=5 were performed.
Group A. There are no pts with renal insufficiency.
All pts are dry spontaneously n=7, or by catheterisation via urethra n=5.
Group B. Reflux-nephropathy leading to renal insufficiency was seen in 3 pts: one presented with transplanted kidney, another one was transplanted after re-do operation for cloaca (she died 6 month later). The 3rd is waiting for transplantation.
There are 7 continent, 10 social continent, and 2 incontinent pts.
The outcome of urinary tract function is better when urethral and vaginal repairs and the pull-through of the rectum are done together (group A). To prevent the damage of the urinary tract long-term follow-up is essential. Some of these patients need secondary operations to achieve urinary control or to correct reflux.
Alexander Rokitansky, M. Löbl, E. Frigo
Dept. of Ped. Surgery, SMZOst Danubehospital, Vienna, Austria.
Hypospadia is one of the most common deformities of the urogenitalsystem. A great diversity of procedures to correct hypospadias are suggested.
In our department we use for the correction of the so called “distal” hypospadia mainly two different methods, the Snodgras urethroplasty and the Y-V glanuloplasty modified Mathieu technique.
We investigated retrospectively for both methods ( Snodgras group n= 40 and Mathieu group n= 60) the outcome regarding the complication, the cosmetic satisfaction and the voiding and compared the results. The mean age in both groups was comparable and is located in between the international recommended correction time of 12 – 18 month.
The mean observation time was 29 month for the Snodgras urethroplasty group respectively 16 month for the Y-V glanuloplasty modified Mathieu.
We find an good cosmetic look, an adequate voiding and a comparable outcome regarding the complication rate. As we do, in contrast to the “Snodgras” group, not use any stents or cystofix in the “Mathieu” group, the crucial element is, in our opinion, an adequate pain relief during the first days so that one get no voiding problems.
All over, we think that the patient benefits from the Y-V glanuloplasty modified Mathieu technique as for this methods we use no stents and the hospital stay is much shorter than in the other group.
Dominique Bourdelat1, Clarisse Mazzola1, Cyprien Zare1, Mohamed Ahmad1, Anne Marrel2
1-Pediatric surgery, C.H.U. POINTE A PITRE GUADELOUPE, POINTE A PITRE , Guadeloupe.
2-Depart.Of Cytopathology C.H.U. POINTE A PITRE
The clinical and pathological features of one case of juvenile granulosa cell tumor occuring in a 14 months old black girl are studied.A precocious puberty and an abdominal mass were noted with a vaginal bleeding. Clinical examination showed pubic hair and developing breasts. Abdominal ultrasongraphy and CT Scan showed a 53 X 33 mm mass in the right ovary. C.E.A., Alphafoetoprotein, Beta HCG levels were within the normal ranges. Serum 17 Beta Estradiol was 320ng/l (normal value for age :5-15). Oophorectomy without salpingectomy was the only treatment. No adjuvant therapy was delivered. The tumor weighted 52g and measured 6cm in diameter with solid and nodular areas.The post operative course was unremarkable. Histology showed different cells with abundant vacuoted cytoplasm. Many mitotic figures were present. Only a few cases in infants have been reported in the literature so it is necessary to improve our knowledge of this uncommon tumor.
Ciro Esposito2, Marianna De Marco1, Concetta De Luca1, Salvatore Iacobelli1, Aurelie Chiappinelli1, Alessandro Settimi1
1-Pediatric Surgery, University "Federico II" , Naples, Italy
2-Dept. Pediatric Surgery "University Magna Grecia" Catanzaro, Italy
Aims: Massive resection of small bowel is a trigger for remodelling of the remnant intestine. We tested the hypotesis that the adaptation changes have a segmental pattern. We also investigated the mechanisms of intestinal adaptation in proximal to distal intestinal segments.
Methods: Growing 210-225 grams Sprague_Dawley rats (n=29) were divided in 3 groups: the resected group, which had 75% of the jejunoileum removed, the sham-resected control group and the not-operated control group. Intestinal length and wet weight were evaluated at 15 days post-resection. Histological and morphological parameters in proximal to distal remaining intestinal segments were compared in the 3 groups.
Results: Small intestinal length increased by 48% compared to sham-resected rats.An increase in wet weight was observed only in the ileum. Significant increases in villus height, crypt depth and mucosal thickness were evident in the distal ileum of the resected rats, while only slight modifications were observed in the jejunum. Stomach, duodenum and colon didn’t show modifications.A reduced number of villi with increased base diameter and height was observed in the ileum but not in other segments.Overall, the absorptive surface increased by 23% in jejunum and 55% in ileum compared to sham-operated rats. The increase of mucosal layer was due to hyperplasia in jejunum, but to hypertrophy in ileum.
Conclusions: The intestine reacts to massive resection with a differential segment-related pattern:the ileum and the jejunum with a distinct restructuring of mucosal architecture; the stomach, duodenum and colon are not morphologically involved.Sparing ileal segments during intestinal resection may improve the long-term chance of gaining intestinal function.
M. Roll, M. Chmelnik, S. Holland-Cunz, K.-L. Waag
Pediatric Surgery, Heidelberg University, , Germany.
Aim: In contrast to adult surgery the neonatal small intestine confronts the pediatric surgeon with variable diameters of the gut. Therefore anastomoses are usually performed by hand with interrupted sutures. The present study demonstrates alternative techniques of small bowel anastomosis.
Method: An anastomosis in the distal ileum of Sprague Dawley rats was performed either with a single clip applicator (Anastoclip®, n=21), a patch of collagen coated with fibrinogen and thrombin coagulation factors (Tacho Sil®, n=11) or with a common hand-sutured anastomosis (PDS 6-0, n=16). Rats were sacrificed at day 3 or 14 after laparotomy. The bowel anastomosis was evaluated concerning leakage, adhesions and stenosis (x-ray examination). Tension tests were performed to measure the stability. Histological examinations were performed with HE staining.
Results: The clipped anastomosis is feasible and faster to perform than the conventional hand-sutured and the glued anastomosis (5 vs. 19 vs. 11min). There were less stenosis and less adhesions in the group of the clipped anastomosis. Furthermore there were differences in the mechanical stability, with higher tension forces needed for rupturing the clipped anastomosis (clipped=2,33N vs. sutured=1,78N). The glued anastomosis showed significantly less mechanical stability and a higher insufficiency rate compared to the other methods. Histological examinations did not show significant differences between the three groups.
Conclusion: In the presented animal model the clipped intestinal non perforating anastomosis shows significant advantages compared to the common hand-sutured anastomosis. The results of the glued anastomosis were less promising.
Thomas Huckstadt, Steffen Richter, Christine Matthes, Till Ploenes, Felix Schier
Pediatric Surgery, University Medical Center Mainz, Mainz, Germany.
Objective: Gas embolism is a complication of laparoscopy. Cardiopulmonary effects under laparoscopic conditions during and after carbondioxide embolism in healthy and pulmonary compromised piglets were evaluated.
Methods: Two groups of 13 piglets, mean body weight 6.9kg and 6.7kg, were anesthesised. Pulmonary lavage (30ml/kgBW) was performed only in the second group. Lavage was stopped when partial arterial oxygen pressure was less than 200mmHg. After induction of capnoperitoneum (10 mmHg) normocarbia was maintained (paCO2 35-40 mmHg). Carbondioxide was injected into the V. cava inferior for 30 minutes with a flow of 0.5 ml/kgBW/min, followed by subsequent desufflation. Fifteen minutes later, ventilation was continued on normocarbia. Heart rate, mean arterial pressure, mean central venous pressure, mean pulmonary arterial pressure, oxygenation index and pH were registrated. The parameters were examined at the beginning, at the end and 60 minutes after CO2 injection. (animal right committee approved study 1.5 177-07/051-6)
Results: In group 1 twelve and in group 2 ten animals survived. In both groups, there was an increase in oxygenation index and arterial pulmonary pressure during embolism (0.9 / 2.1 , 8.5 / 19.4 and 14 / 28 , 23 /39 mmHg respectively). pH decreased (7.41 / 7.22 and 7.39 / 7.17). Other parameters remained in physiological range.
Nine animals of group 1 and seven animals of group 2 returned to baseline levels (+/- 10% deviation).
Conclusion: Both groups showed similar cardiopulmonary reactions. Despite primary deterioration, good recovery was achieved in both groups. Cardiopulmonary effects caused by CO2 invasion of the venous system are reversible.
Steffen Richter, Thomas Huckstadt, Christine Matthes, Till Ploenes, Felix Schier
Pediatric Surgery, University Medical Center Mainz, Mainz, Germany.
Laparoscopy involves increasement of intraabdominal pressure. Pulmonary diseases are frequent in the newborn period. This investigation was initiated to quantify changes during increasing intraabdominal pressures up to 24 mmHg in healthy and pulmonary compromised piglets.
Twentysix piglets (body weight 4.8-7.9 kg, median 6.8 kg) were anesthesised. Bronchopulmonary lavage was performed randomised in thirteen piglets until partial arterial oxygen pressure was less than 200 mmHg. The investigation was started on normocarbia. After registration of baseline values pneumoperitoneum was induced. The intraabdominal pressure was raised in 6 mmHg steps up to 24 mmHg, followed by desufflation. Each pressure level was maintained for 20 minutes. Ventilation was adjusted to normocarbia by elevating the airway pressure. Tidal volume, mean blood pressure of carotic artery and pulmonary artery were registrated. Oxygenation index was calculated.
(animal right committee approved study 1.5 177-07/051-6)
Systemic and pulmonary arterial blood pressure increased significantly after the first elevation of intraabdominal pressure (healthy piglets 52 / 63 mmHg and 13 / 17 mmHg, compromised piglets 48 / 53 mmHg and 21 / 27 mmHg). Oxygenation index increased at higher intraabdominal pressure levels (18 mmHg). Tidal volume remained nearly unchanged after adaptation of the airway pressure. All parameters reached baseline levels after desufflation.
Elevated intraabdominal pressures during laparoscopy are tolerated by healthy and pulmonary compromised newborn piglets. Impairment of vital parameters has proven to be reversible after reestablishment of physiological conditions.
Christine Matthes, Thomas Huckstadt, Steffen Richter, Till Ploenes, Felix Schier
Pediatric Surgery, University Medical Center Mainz, Mainz, Germany.
Laparoscopy has become a routine method in newborn surgery. Operation conditions require airway pressure elevation to maintain adequate ventilation. Quantification of changes in pulmonary and cardiac parameters without ventilatory adaptation is still lacking.
Eleven piglets (body weight 4.27-7.3 kg) were anesthesized. The investigation was started on normocarbia. The intraabdominal pressure (IAP) was raised in 6 mmHg steps up to 24 mmHg, followed by desufflation. Each pressure level was maintained for 20 minutes. Tidal volume, endtidal carbon dioxide pressure, heart rate, mean blood pressure of carotic and pulmonary artery and arterial oxygenation were registrated. Compliance, resistance and respiratory time constants of the respiratory system were calculated. These parameters were examined at each pressure level and 30minutes after desufflation.
(animal right committee approved study 1.5 177-07/051-6)
Tidal volume decreased continuously with 1 mL/kgBW by IAP elevation of 6 mmHg. Compliance decreased from 1.1 to 0.41 mL/cmH2O kg (IAP 0 / 24 mmHg respectively). Time constants decreased (0.15 to 0.08 s), caused by unchanged resistance. Arterial and endtidal carbondioxide increased (45 to 69 and 38 to 58 mmHg). Arterial oxygen pressure decreased after the first step but remained within physiological range. A decrease in mean arterial pressure occured immediately after the first IAP elevation. Further elevation of IAP showed only minimal changes. Heart rate and pulmonary arterial pressure didn’t change significantly. After desufflation all parameters returned to baseline levels.
Quantification of cardiopulmonary changes during laparoscopy is possible. Attention must be paid to decreasing time constants to avoid atelectasis.
T-1 LONG-TERMS RESULTS OF ESOPHAGEAL REPLACEMENT IN CHILDREN