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OBJECTIVES: Classical Hirschsprung disease (HD) is defined by the absence of ganglion cells in the rectosigmoid colon. The diagnosis is made from rectal biopsy, which reveals the aganglionosis and the presence of cholinergic hyperinnervation. However, depending on the method of rectal biopsy, the quality of the specimens and the related diagnostic accuracy varies substantially. To facilitate and objectify the diagnosis of HD, we investigated whether software-based identification of cholinergic hyperinnervation in digitalized histopathology slides is suitable for distinguishing healthy individuals from affected individuals. METHODS: N = 190 samples of 112 patients who underwent open surgical rectal biopsy at our pediatric surgery center between 2009 and 2019 were included in this study. Acetylcholinesterase (AChE) stained slides of these samples were collected and digitalized via slide scanning and analyzed using two digital imaging software programs (HALO, QuPath). The AChE-positive staining area in the mucosal layers of the intestinal wall was determined. In the next step machine learning was employed to identify patterns of cholinergic hyperinnervation. RESULTS: The area of AChE-positive staining was greater in HD patients compared to healthy individuals (p < 0.0001). Artificial intelligence-based assessment of parasympathetic hyperinnervation identified Hirschsprung disease with a high precision (area under the curve [AUC] 0.96). The accuracy of the prediction model increased when nonrectal samples were excluded (AUC 0.993). CONCLUSIONS: Software-assisted machine-learning analysis of AChE staining is suitable to improve the diagnostic accuracy of Hirschsprung disease.
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PURPOSE: Abdominal wall closure in patients with giant omphalocele (GOC) and complicated gastroschisis (GS) remains to be a surgical challenge. To facilitate an early complete abdominal wall closure, we investigated the combination of a staged closure technique with continuous traction to the abdominal wall using a newly designed vertical traction device for newborns. METHODS: Four tertiary pediatric surgery departments participated in the study between 04/2022 and 11/2023. In case primary organ reduction and abdominal wall closure were not amenable, patients underwent a traction-assisted abdominal wall closure applying fasciotens®Pediatric. Outcome parameters were time to closure, surgical complications, infections, and hernia formation. RESULTS: Ten patients with GOC and 6 patients with GS were included. Complete fascial closure was achieved after a median time of 7 days (range 4-22) in GOC and 5 days (range 4-11) in GS. There were two cases of tear-outs of traction sutures and one skin suture line dehiscence after fascial closure. No surgical site infection or signs of abdominal compartment syndrome were seen. No ventral or umbilical hernia occurred after a median follow-up of 12 months (range 4-22). CONCLUSION: Traction-assisted staged closure using fasciotens®Pediatric enabled an early tension-less fascial closure in GOC and GS in the newborn period.
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Parede Abdominal , Técnicas de Fechamento de Ferimentos Abdominais , Gastrosquise , Hérnia Umbilical , Tração , Humanos , Hérnia Umbilical/cirurgia , Gastrosquise/cirurgia , Masculino , Estudos Prospectivos , Tração/métodos , Tração/instrumentação , Feminino , Recém-Nascido , Parede Abdominal/cirurgia , Técnicas de Fechamento de Ferimentos Abdominais/instrumentação , Lactente , Resultado do TratamentoRESUMO
INTRODUCTION: Trauma is a major problem which has a significant health, social, and economic impact. Particularly, pediatric trauma carries substantial mortality and morbidity. This is a great concern for subspecialized general and pediatric surgeons. Therefore, a global initiative for pediatric trauma care is warranted and should be initiated. AIM: The international association "Global Initiative for Children's Surgery" (GICS) would like to propose and organize a children's trauma care (CTC) initiative. This initiative should comprehensively address pediatric trauma management globally, especially in low- and middle-income countries (LMICs). The initiative seeks to achieve a structured cooperation and collaboration with respective sister organizations and local stakeholders. METHODS: The initiative will address these relevant aspects: 1. first aid; 2. prehospital primary trauma care; 3. hospital primary trauma care; 4. advanced care (ATLS); 5. diagnostic facilities; 6. operation room (OR) equipment; 7. specialized surgical services; 8. rehabilitation; 9. registry, research, and auditing; 10. specialization in pediatric trauma; 11. capacity and confidence building in pediatric trauma; 12. PREVENTION: The GICS CTC provided activities have been recorded and evaluated in a structured manner. This statement paper is based on data of a narrative review as well as expert opinions. RESULTS: The Trauma Working Group of GICS provided specialized trauma prevention leaflets available for translation to different languages. A one-day children's primary trauma course has been designed to be delivered at the physical GICS meetings. Exercising advocacy, the group addressed several meetings on prevention of pediatric trauma, which included the 75th United Nations General Assembly (UNGA) (2020), GICS IVth meeting in Johannesburg (2020), Norwich (UK) Joint SPRINT Symposium on Pediatric Surgery for Pediatricians (2021), the second online Pan African Pediatric Surgical Association (PAPSA) meeting (2021), the seventh World Congress of the World Federation of Associations of Pediatric Surgeons (WOFAPS) in Prague (2022), and GICS pediatric trauma webinar (2023). Additionally, the working group participated in the preparations of a pediatric trauma module for the World Health Organization (WHO) and published several related studies. The contents of the selected articles added relevant information to the categories stated above. CONCLUSIONS: The CTC initiative of GICS is proposed as a mean to address pediatric trauma comprehensively through a process of collaboration and advocacy with existing organizations to achieve awareness, health education, prevention, health, and training. Further, it will support the provision of suitable facilities to health institutions. The establishment of a specialization in pediatric trauma is encouraged. GICS CTC initiative aims to improve pediatric trauma care in LMICs by developing injury prevention strategies; optimizing the use of locally available resources; obtaining commitment by LMICs governments; improvement in all fields of hospital care; improvements in infrastructure, education and training, and attention to data registry and research.
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INTRODUCTION: Our purpose was to assess the state of training, clinical practice, and barriers to use point-of-care ultrasound (POCUS) in pediatric surgery in Europe. METHODS: An electronic survey was disseminated among European pediatric surgeons utilizing the European Pediatric Surgery Association network and other existing networks. RESULTS: There were 186 respondents from 27 European countries and 7 non-European countries. In most countries (86.6%; N = 161), the initial ultrasound for acute admissions is performed by radiologists, with 1 to 6 hours turnover in 62.9% (N = 117) of urgent cases. Ultrasound by pediatric surgeons (point-of-care ultrasound/POCUS) is performed by 48.4% (N = 90) of respondents, with 29% (N = 54) using it at least once per week. The most common indications for POCUS include abdominal focused abdominal sonography in trauma (53.8%; N = 100), diagnosis of appendicitis (41.9%; N = 78), and intussusception (44.6%; N = 84). In malrotation-volvulus, 28.5% (N = 53) used ultrasound for its diagnosis, while 27.5% (N = 51) would not see an indication here. Training in POCUS occurred informally for 55.4% (N = 103) of participants, while 31.2% (N = 58) attended formal training courses. Almost all respondents wanted to attain further POCUS training (89.3%; N = 166), only 7% (N = 13) did not think this would be useful. For 73.1% (N = 136), POCUS is not currently part of the pediatric surgery training curriculum in their country. Perceived barriers to POCUS use include a lack of training opportunities (26.3% [N = 49]) and a paucity of portable ultrasound machines (17.8% [N = 33]). CONCLUSION: There is a wide spectrum of POCUS use in pediatric surgery across Europe. For those surgeons who practice POCUS, it is most used for the diagnosis of abdominal conditions. There are differing views among clinicians concerning the most useful applications of POCUS. The extent to which ultrasound is taught during pediatric surgery training differs substantially across European curricula.
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Sistemas Automatizados de Assistência Junto ao Leito , Especialidades Cirúrgicas , Criança , Humanos , Ultrassonografia , Currículo , Inquéritos e QuestionáriosRESUMO
BACKGROUND: Appendectomy in children is performed either lapa - roscopically (LA) or by open surgery (OA). We studied whether, and how, the outcome is affected by the technique used and by the intraoperative conversion of LA to OA. METHODS: We analyzed routine data from children and adolescents in three age groups (1-5 years, 6-12 years, and 13-17 years) who were insured by the AOK statutory health insurance carrier in Germany and who underwent appendectomy in the period 2017-2019. General surgical complications and reoperations within 90 days were assessed with relevant indicators. Associations between the surgical technique and these indicators were studied with logistic regression. RESULTS: Of the 21 541 patients included in the study, general surgical complications were observed in 2.1% and reoperations in 1.8% overall. Broken down by age group, the corresponding figures were 5.4% and 4.4% (age 1 to 5), 2.5% and 1.8% (age 6 to 12), and 1.5% and 1.6% (age 13 to 17). The main risk factors for complications and reoperations were acute complicated appendicitis and conversion from LA to OA. Regression analysis revealed similar outcomes with OA compared to LA in the 1-to-5 age group, (odds ratios and 95% confidence intervals: 1.1 [0.6; 2.1] for general surgical complications and 1.5 [0.8; 2.7] for reoperations), but worse outcomes with OA in the other two age groups (age 6 to 12: 1.9 [1.2; 2.9] and 2.1 [1.5; 2.9]; age 13 to 17: 1.7 [1.0; 2.9] and 2.2 [1.4; 3.6]). When conversions were assigned to the LA group, the odds ratio (OA compared to LA) for reoperation across all age groups was 3.5 [2.8; 4.4] in patients with acute uncomplicated appendicitis and 4.2 [3.4; 5.3] in patients with complicated appendicitis. Complicated appendicitis also increased the rate of general surgical complications and the length of stay in hospital. CONCLUSION: Among children in the two older age groups, LA was followed by fewer general surgical complications and reoperations than OA. These differences were less pronounced when conversions were counted as belonging to the LA group. Children aged 1-5 appear to benefit the least from the lapa - roscopic technique.
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Apendicite , Laparoscopia , Adolescente , Criança , Humanos , Idoso , Lactente , Pré-Escolar , Apendicectomia/efeitos adversos , Apendicite/epidemiologia , Apendicite/cirurgia , Reoperação , Alemanha/epidemiologiaRESUMO
BACKGROUND: Because 8-10% of children in the emergency room present with acute abdominal pain, a systematic work-up is essential to rule out acute abdomen. OBJECTIVES: This article highlights the etiology, symptoms, diagnostic workup, and treatment of acute abdomen in children. MATERIALS AND METHODS: Review of the current literature. RESULTS: Abdominal inflammation, ischemia, bowel and ureteral obstruction, or abdominal bleeding are causes of acute abdomen. Extra-abdominal diseases such as otitis media in toddlers or testicular torsion in adolescent boys can also lead to symptoms of acute abdomen. Abdominal pain, (bilious) vomiting, abdominal guarding, constipation, blood-tinged stools, abdominal bruise marks, and poor condition of the patient with symptoms such as tachycardia, tachypnea, and hypotonia up to shock are leading symptoms of acute abdomen. In some cases, emergent abdominal surgery is needed to treat the cause of the acute abdomen. However, in patients with pediatric inflammatory multisystem syndrome temporarily associated with SARS-CoV2 infection (PIMS-TS), a new disease causing an acute abdomen, surgical treatment is rarely needed. CONCLUSIONS: Acute abdomen can lead to nonreversible loss of an abdominal organ, such as bowel or ovary, or develop into acute deterioration of the patient's condition up to the state of shock. Therefore, a complete history and thorough physical examination are needed to timely diagnose acute abdomen and initiate specific therapy.
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Abdome Agudo , COVID-19 , Masculino , Feminino , Adolescente , Humanos , Abdome Agudo/diagnóstico , Abdome Agudo/etiologia , Abdome Agudo/cirurgia , Dor Abdominal/complicações , Dor Abdominal/diagnóstico , Abdome , COVID-19/complicaçõesRESUMO
Interstitial cells of Cajal (ICCs) are pacemaker cells of gastrointestinal motility that generate and transmit electrical slow waves to smooth muscle cells in the gut wall, thus inducing phasic contractions and coordinated peristalsis. Traditionally, tyrosine-protein kinase Kit (c-kit), also known as CD117 or mast/stem cell growth factor receptor, has been used as the primary marker of ICCs in pathology specimens. More recently, the Ca2+-activated chloride channel, anoctamin-1, has been introduced as a more specific marker of ICCs. Over the years, various gastrointestinal motility disorders have been described in infants and young children in which symptoms of functional bowel obstruction arise from ICC-related neuromuscular dysfunction of the colon and rectum. The current article provides a comprehensive overview of the embryonic origin, distribution, and functions of ICCs, while also illustrating the absence or deficiency of ICCs in pediatric patients with Hirschsprung disease intestinal neuronal dysplasia, isolated hypoganglionosis, internal anal sphincter achalasia, and congenital smooth muscle cell disorders such as megacystis microcolon intestinal hypoperistalsis syndrome.
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Doença de Hirschsprung , Células Intersticiais de Cajal , Lactente , Criança , Humanos , Pré-Escolar , Células Intersticiais de Cajal/metabolismo , Relevância Clínica , Doença de Hirschsprung/metabolismo , Motilidade Gastrointestinal/fisiologia , Canal Anal/metabolismo , Proteínas Proto-Oncogênicas c-kit/metabolismoRESUMO
BACKGROUND: Hirschsprung disease (HD) is an aganglionosis of variable length starting at the rectosigmoid colon with surgery as sole therapeutic option. The length of the resected bowel segment is a crucial information for the treating surgeons and influences the prognosis of the patient. It is often artificially altered due to post operative tissue shrinkage. The objective of this study is to quantify the extent tissue shrinkage of HD specimens. MATERIAL AND METHODS: Colorectal HD specimens were measured at the time of surgery and at the time of cut-up, either fresh or after formalin fixation and statistically analyzed. RESULTS: Sixteen colorectal specimens were included. Following formalin fixation the specimen length decreased by 22.7% (P < .001). Without formalin fixation the specimens shrank by an average of 24.9% (P = .05). There was no significant difference in the extent of tissue shrinkage with or without formalin fixation (P = .76). CONCLUSION: This study showed that there is significant tissue shrinkage in HD specimens. The 2 different cohorts revealed that tissue shrinkage is mostly caused by tissue retraction/alteration after organ removal but also to a lesser extent by fixation with formalin. Surgeons and (neuro-)pathologists should be aware of the sizeable shrinking artifact to avoid unnecessary confusion.
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Neoplasias Colorretais , Doença de Hirschsprung , Cirurgiões , Criança , Humanos , Doença de Hirschsprung/diagnóstico , Doença de Hirschsprung/cirurgia , Doença de Hirschsprung/patologia , Reto/patologia , Formaldeído , Neoplasias Colorretais/patologiaRESUMO
INTRODUCTION: Colonic atresia (CA) is a very rare disease. Two entities of CA can be differentiated: primary CA and CA with abdominal wall defects (AWD). This study aimed to investigate the differences between these two entities, especially for long-term outcomes. MATERIALS AND METHODS: Data from the major health insurance company (AOK) were analyzed. Sixty-two patients with ICD-10 codes Q42.1-2 and Q42.8-9 (atresia of the colon and rectum) who underwent surgery within 10 days after their first admission between 2007 and 2016 were obtained. RESULTS: Twenty-nine patients had an AWD, and 33 patients had primary colonic atresia (PCA). Significant differences between patients with PCA and AWD were found regarding prematurity but not for other concomitant malformations. Ostomy was the initial therapy of choice for 87.9% (29/33) of patients with PCA and 65.5% (19/29) with AWD. Central venous access was significantly more often in patients with AWD. Overall, patients with CA have an excellent short-term outcome. The 1-year mortality was 3% (1/33) in PCA and 6.9% (2/29) in AWD. Delayed management did not lead to higher mortality. Higher number of admission days and higher hospital costs were related to gastrostomy and short bowel in the long term. CONCLUSION: The long-term outcome of CA is related to short bowel and need for initial gastrostomy but not related to AWD, prematurity, or associated anomalies.
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Parede Abdominal , Atresia Intestinal , Humanos , Parede Abdominal/cirurgia , Estudos Retrospectivos , Colo , Atresia Intestinal/cirurgiaRESUMO
The biology of cancer stem cells (CSCs) of pediatric cancers, such as hepatoblastoma, is sparsely explored. This is mainly due to the very immature nature of these tumors, which complicates the distinction of CSCs from the other tumor cells. Previously, we identified a CSC population in hepatoblastoma cell lines expressing the CSC markers CD34 and CD90, cell surface Vimentin (csVimentin) and binding of OV-6. In this study, we detected the co-expression of the immune escape factor PD-L1 in the CSC population, whereas the other tumor cells remained negative. FACS data revealed that non-CSCs give rise to CSCs, reflecting plasticity of CSCs and non-CSCs in hepatoblastoma as seen in other tumors. When we treated cells with cisplatin and decitabine, a new CD34+/lowOV-6lowCD90+ population emerged that lacked csVimentin and PD-L1 expression. Expression analyses showed that this new CSC subset shared similar pluripotency and EMT features with the already-known CSCs. FACS results further revealed that this subset is also generated from non-CSCs. In conclusion, we showed that hepatoblastoma CSCs express PD-L1 and that the biology of hepatoblastoma CSCs is of a plastic nature. Chemotherapeutic treatment leads to another CSC subset, which is highly chemoresistant and could be responsible for a poor prognosis after postoperative chemotherapy.
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Significant progress has been made in the management of Wilms tumor (WT) in recent years, mostly as a result of collaborative efforts and the implementation of protocol-driven, multimodal therapy. This article offers a comprehensive overview of current multidisciplinary treatment strategies for WT, whilst also addressing recent technical innovations including nephron-sparing surgery (NSS) and minimally invasive approaches. In addition, surgical concepts for the treatment of metastatic disease, advances in tumor imaging technology and potentially prognostic biomarkers will be discussed. Current evidence suggests that, in experienced hands and selected cases, laparoscopic radical nephrectomy and laparoscopic-assisted partial nephrectomy for WT may offer the same outcome as the traditional open approach. While NSS is the standard procedure for bilateral WT, NSS has evolved as an alternative technique in patients with smaller unilateral WT and in cases with imminent renal failure. Metastatic disease of the lung or liver that is associated with WT is preferably treated with a three-drug chemotherapy and local radiation therapy. However, surgical sampling of lung nodules may be advisable in persistent nodules before whole lung irradiation is commenced. Several tumor markers such as loss of heterozygosity of chromosomes 1p/16q, 11p15 and gain of function at 1q are associated with an increased risk of recurrence or a decreased risk of overall survival in patients with WT. In summary, complete resection with tumor-free margins remains the primary surgical aim in WT, while NSS and minimally invasive approaches are only suitable in a subset of patients with smaller WT and low-risk disease. In the future, advances in tumor imaging technology may assist the surgeon in defining surgical resection margins and additional biomarkers may emerge as targets for development of new diagnostic tests and potential therapies.
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BACKGROUND: Duodenal obstruction is a rare cause of congenital bowel obstruction. Prenatal ultrasound could be suggestive of duodenal atresia if polyhydramnios and the double bubble sign are visible. Prenatal diagnosis should prompt respective prenatal care, including surgery. The aim of this study was to investigate the rate and importance of prenatally diagnosed duodenal obstruction, comparing incomplete and complete duodenal obstruction. METHODS: A retrospective, single-center study was performed using data from patients operated on for duodenal obstruction between 2004 and 2019. Prenatal ultrasound findings were obtained from maternal logbooks and directly from the investigating obstetricians. Postnatal data were obtained from electronic charts, including imaging, operative notes and follow-up. RESULTS: A total of 33/64 parents of respective patients agreed to provide information on prenatal diagnostics. In total, 11/15 patients with complete duodenal obstruction and 0/18 patients with incomplete duodenal obstruction showed typical prenatal features. Prenatal diagnosis prompted immediate surgical treatment after birth. CONCLUSION: Prenatal diagnosis of congenital duodenal obstruction is only achievable in cases of complete congenital duodenal obstruction by sonographic detection of the pathognomonic double bubble sign. Patients with incomplete duodenal obstruction showed no sign of duodenal obstruction on prenatal scans and thus were diagnosed and treated later.
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Esophageal atresia (EA) is a rare congenital disease which is usually not of the detected prenatally. Due to the lack of prenatal diagnosis, some newborns with EA are born outside of specialized centers. Nevertheless, centralized care of EA has been proposed, even if a clear volume-outcome association in EA management remains unconfirmed. Furthermore, whether outcomes differ between outborn and inborn patients with EA has not been systematically investigated. Therefore, this single-center, retrospective study aimed to investigate EA management and outcomes with a special focus on inborn versus outborn patients. The following data were extracted from the medical records of infants with EA from 2009 to 2019: EA type, associated anomalies, complications, and long-term outcome. Patients were allocated into inborn and outborn groups. Altogether, 57 patients were included. Five patients were excluded (referral before surgery, loss of data, death before surgery [n = 1], and incorrect diagnosis [diverticulum, n = 1]). Among all patients, the overall survival rate was 96%, with no mortalities among outborn patients. The overall hospitalization period was shorter for outborn patients. The median follow-up durations were 3.8 years and 3.2 years for inborn and outborn patients, respectively. Overall, 15% of patients underwent delayed primary anastomosis (long-gap atresia [n = 4] and other reasons [n = 4]). Early complications included three anastomotic leakages and one post-operative fistula; 28% of patients developed strictures, which required dilatation, and 38% of patients showed relevant gastroesophageal reflux, which required fundoplication, without any differences between the groups. The two groups had comparable low mortality and expected high morbidity with no significant differences in outcome. The outborn group showed nonsignificant trends toward lower morbidity and shorter hospitalization periods, which might be explained by the overall better clinical status.
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Atresia Esofágica , Esofagoplastia , Fístula Traqueoesofágica , Anastomose Cirúrgica , Atresia Esofágica/complicações , Humanos , Lactente , Recém-Nascido , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/cirurgia , Estudos Retrospectivos , Fístula Traqueoesofágica/complicações , Resultado do TratamentoRESUMO
INTRODUCTION: Adequate patient volume is essential for the maintenance of quality, meaningful research, and training of the next generation of pediatric surgeons. The role of university hospitals is to fulfill these tasks at the highest possible level. Due to decentralization of pediatric surgical care during the last decades, there is a trend toward reduction of operative caseloads. The aim of this study was to assess the operative volume of the most relevant congenital malformations at German academic pediatric surgical institutions over the past years. METHODS: Nineteen chairpersons representing university-chairs in pediatric surgery in Germany submitted data on 10 index procedures regarding congenital malformations or neonatal abdominal emergencies over a 3-year period (2015 through 2017). All institutions were categorized according to the total number of respective cases into "high," "medium," and "low" volume centers by terciles. Some operative numbers were verified using data from health insurance companies, when available. Finally, the ratio of cumulative case load versus prevalence of the particular malformation was calculated for the study period. RESULTS: From 2015 through 2017, a total 2,162 newborns underwent surgery for congenital malformations and neonatal abdominal emergencies at German academic medical centers, representing 51% of all expected newborn cases nationwide. The median of cases per center within the study period was 101 (range 18-258). Four institutions (21%) were classified as "high volume" centers, four (21%) as "medium volume" centers, and 11 (58%) as "low volume" centers. The proportion of patients operated on in high-volume centers varied per disease category: esophageal atresia/tracheoesophageal fistula: 40%, duodenal atresia: 40%, small and large bowel atresia: 39%, anorectal malformations: 40%, congenital diaphragmatic hernia: 56%, gastroschisis: 39%, omphalocele: 41%, Hirschsprung disease: 45%, posterior urethral valves: 39%, and necrotizing enterocolitis (NEC)/focal intestinal perforation (FIP)/gastric perforation (GP): 45%. CONCLUSION: This study provides a national benchmark for neonatal surgery performed in German university hospitals. The rarity of these cases highlights the difficulties for individual pediatric surgeons to gain adequate clinical and surgical experience and research capabilities. Therefore, a discussion on the centralization of care for these rare entities is necessary.
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Enterocolite Necrosante , Atresia Esofágica , Hérnias Diafragmáticas Congênitas , Doenças do Recém-Nascido , Fístula Traqueoesofágica , Criança , Emergências , Enterocolite Necrosante/cirurgia , Atresia Esofágica/cirurgia , Hérnias Diafragmáticas Congênitas/cirurgia , Hospitais Universitários , Humanos , Recém-Nascido , Fístula Traqueoesofágica/cirurgiaRESUMO
INTRODUCTION: The majority of pediatric surgeons and hepatologists recommend the centralization of biliary atresia (BA) treatment within experienced liver units. We aimed to investigate whether voluntary self-restriction and acceptance of the need for this change in practice changed the BA referral policy in Germany during the last decade. MATERIALS AND METHODS: In cooperation with pediatric surgeons, gastroenterologists or hepatologists, and pediatric liver transplant units, the 2-year follow-up data of infants with BA born in Germany between 2010 and 2014 were collected using www.bard-online.com or pseudonymized data transfer. Results were compared with our previous analysis of the outcome data of infants with BA born between 2001 and 2005 in Germany. RESULT: Overall, 173 infants with BA were identified, of whom 160 underwent Kasai portoenterostomy (KPE; 92.5%) and 13 (7.5%) underwent primary liver transplantation at 21 German centers. At 2-year follow-up, overall survival was 87.7% (vs. 81.9% in 2001-2005 [p = 0.19]), survival with native liver post-KPE was 29.2% (vs. 22.8% in 2001-2005 [p = 0.24]), and jaundice-free survival with native liver post-KPE was 24.0% (vs. 20.1% in 2001-2005 [p = 0.5]). Compared with the 2001-2005 analysis, all criteria showed improvement but the differences are statistically not significant. CONCLUSION: Our observation shows that KPE management requires improvement in Germany. Centralization of BA patients to German reference liver units is not yet mandatory. However, European and national efforts with regard to the centralization of rare diseases support our common endeavor in this direction.
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Atresia Biliar , Transplante de Fígado , Atresia Biliar/cirurgia , Criança , Humanos , Lactente , Fígado/cirurgia , Portoenterostomia Hepática , Encaminhamento e Consulta , Resultado do TratamentoRESUMO
BACKGROUND: In order to avoid consequences of total splenectomy, partial splenectomy (PS) is increasingly reported. The purpose of this study was to compare perioperative outcomes of laparoscopic PS (LPS) and open PS (OPS) in children and adolescents. AIM: To compare perioperative outcomes of patients with LPS and OPS. METHODS: After institutional review board approval, a total of 26 patients that underwent LPS or OPS between January 2008 and July 2018 were identified from the database of our tertiary referral center. In total, 10 patients had LPS, and 16 patients underwent OPS. Blood loss was calculated by Mercuriali's formula. Pain scores, analgesic requirements and complications were assessed. The Wilcoxon rank sum test was used for comparison. To compare categorical variables, Fisher's exact test was applied. RESULTS: LPS was performed in 10 patients; 16 patients had OPS. Demographics (except for body mass index and duration of follow-up), indicating primary disease, preoperative spleen size and postoperative spleen volume, perioperative hematological parameters, postoperative pain scores, analgesic requirements, adverse events according to the Clavien-Dindo classification and the comprehensive complication index, median time from operation to initiation of feeds, median time from operation to full feeds, median time from operation to mobilization and median length of hospital stay did not differ between LPS and OPS. Median (range) operative time (min) was longer in LPS compared to the OPS group [185 (135-298) vs 144 (112-270), respectively; P = 0.048]. Calculated perioperative blood loss (mL of red blood cell count) was higher in the LPS group compared to OPS [87 (-45-777) vs -37 (-114-553), respectively; P = 0.039]. CONCLUSION: This is the first study that compared outcomes of LPS and OPS. Both operative approaches had comparable perioperative outcomes. LPS appears to be a viable alternative to OPS.
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BACKGROUND: Abdominal cocoon or "encapsulating peritoneal sclerosis" (EPS) is an uncommon and rare cause of intestinal obstruction. Only a few cases have been reported in paediatric patients. Typically, EPS is described as the primary form in young adolescent girls from tropical and subtropical countries because of viral peritonitis due to retrograde menstruation or a history of peritoneal dialysis. Most patients are asymptomatic or present with abdominal pain, which is likely to occur secondary to subacute bowel obstruction. Findings at imaging, such as ultrasound, computed tomography, and magnetic resonance imaging, are often nonspecific. When diagnosed, EPS is characterized by total or partial encasement of the bowel within a thick fibrocollagenous membrane that envelopes the small intestine in the form of a cocoon because of chronic intraabdominal fibroinflammatory processes. The membrane forms a fibrous tissue sheet that covers, fixes, and finely constricts the gut, compromising its motility. CASE SUMMARY: We present a case of EPS in a 12-year-old boy 8 wk after primary surgery for resection of symptomatic jejunal angiodysplasia. There was no history of peritoneal dialysis or drug intake. CONCLUSION: In this report, we sought to highlight the diagnostic, surgical, and histopathological characteristics and review the current literature on EPS in paediatric patients.