Acute abdomen after allogenic hematopoietic stem cell transplantation

We report a case of a patient who underwent allogenic hematopoietic stem cell transplantation complicated by acute colonic pseudo obstruction who required surgery after failure of conservative therapy

Asymptomatic congenital cystic adenomatoid malformation of the lung: Is it time to operate?

ObjectiveThe optimal management of congenital adenomatoid malformation of the lung remains controversial. Prenatal ultrasonographic analysis has increasingly discovered asymptomatic lesions, raising questions about the need for and timing of surgical treatment for asymptomatic congenital adenomatoid malformation. The aim of our study was to analyze the short-term postoperative outcome of symptomatic congenital adenomatoid malformations compared with asymptomatic malformations.MethodsAll the data of patients presenting with congenital adenomatoid malformations histologically diagnosed and operated on between 1998 and 2005 at our institution were retrospectively reviewed. Patients were divided into 2 groups: group A comprised asymptomatic infants, and group B comprised symptomatic infants. Major outcomes considered were the length of ventilation, pleural drainage, and hospital stay. Postoperative morbidity and mortality were also evaluated. Asymptomatic patients were further stratified for age at the time of the operation to evaluate whether age at surgical intervention affects the outcome. The Fisher's exact and Mann–Whitney tests were used as appropriate.ResultsFifty-seven patients were consecutively treated. Thirty-five patients were given diagnoses of asymptomatic lesions and were enrolled into group A, whereas 22 patients presenting with symptoms were entered into group B. The lengths of ventilation, pleural drainage, and hospital stay were significantly longer in patients with symptomatic congenital adenomatoid malformations. Moreover, symptomatic patients presented with a higher postoperative complication rate. The age-based stratification of asymptomatic children did not show any difference on either postoperative mortality or major outcome considered.ConclusionChildren with congenital adenomatoid malformations operated on when asymptomatic present a better short-term outcome than symptomatic children. In addition, age at the time of the operation does not negatively affect the outcome. Our findings support early surgical treatment for asymptomatic congenital adenomatoid malformation

Case report: A simple and reliable approach for progressive internal distraction of the sternum for Jeune syndrome (asphyxiating thoracic dystrophy): preliminary experience and literature review of surgical techniques

BackgroundDescribed for the first time in 1954, Jeune syndrome (JS), often called asphyxiating thoracic dystrophy, is a congenital musculoskeletal disease characterized by short ribs, a narrow thorax, and small limbs. In this study, we analyzed and presented our preliminary experience with a device for progressive internal distraction of the sternum (PIDS) in patients with symptomatic JS. In addition, we reviewed the contemporary English literature on existing surgical techniques for treating children with congenital JS.Material and methodsA retrospective analysis of pediatric patients (<18 years old) treated for symptomatic JS at our tertiary center between 2017 and 2023 was performed.ResultsWe presented two patients with JS who underwent surgery using an internal sternal distractor, a Zurich II Micro Zurich Modular Distractor, placed at the corpus of the sternum among the divided halves.ConclusionsWe obtained promising results regarding the safety and effectiveness of this less-invasive device for PIDS in patients with symptomatic JS. Further studies on long-term outcomes are needed to validate these findings

Epithelial thymic tumours in paediatric age: a report from the TREP project

<p>Abstract</p> <p>Background</p> <p>Thymic epithelial tumours (thymoma and carcinoma) are exceptionally rare in children. We describe a national multicentre series with a view to illustrating their clinical behaviour and the results of treatment.</p> <p>Methods</p> <p>From January 2000 all patients under 18 years of age diagnosed with "<it>rare paediatric tumours</it>" were centrally registered by the Italian centres participating in the TREP project (<b>T</b>umori <b>R</b>ari in <b>E</b>tà <b>P</b>ediatrica [Rare Tumours in Paediatric Age]). The clinical data of children with a thymic epithelial tumour registered as at December 2009 were analyzed for the purposes of the present study.</p> <p>Results</p> <p>Our series comprised 4 patients with thymoma and 5 with carcinoma (4 males, 5 females; median age 12.4 years). The tumour masses were mainly large, exceeding 5 cm in largest diameter. Based on the Masaoka staging system, 3 patients were stage I, 1 was stage III, 1 was stage IVa and 4 were stage IVb.</p> <p>All 3 patients with stage I thymoma underwent complete tumour resection at diagnosis and were alive 22, 35 and 93 months after surgery. One patient with a thymoma metastasizing to the kidneys died rapidly due to respiratory failure.</p> <p>Thymic carcinomas were much more aggressive, infiltrating nearby organs (in 4 cases) and regional nodes (in 5), and spreading to the bone (in 3) and liver (in 1). All patients received multidrug chemotherapy (platinum derivatives + etoposide or other drugs) with evidence of tumour reduction in 3 cases. Two patients underwent partial tumour resection (after chemo-radiotherapy in one case) and 4 patients were given radiotherapy (45-54 Gy). All patients died of their disease.</p> <p>Conclusions</p> <p>Children with thymomas completely resected at diagnosis have an excellent prognosis while thymic carcinomas behave aggressively and carry a poor prognosis despite multimodal treatment.</p

Local lymph node involvement in pediatric renal cell carcinoma: A report from the Italian TREP project

Background. One of the most important adverse prognostic factors for adult renal cell carcinoma (RCC) is the retroperitoneal lymph node involvement. The aim of this article is to study the prognostic significance of local lymph node involvement in pediatric RCC and the role of retroperitoneal lymph node dissection (RLND) at diagnosis. Procedure. The series included 16 patients with RCC and lymph nodes involvement registered in the Italian Rare Tumors Pediatric Age (TREP) project, accounting for 26.2% of 61 pediatric RCC observed at AIEOP centers. Results. A radical nephrectomy was performed in all cases: at diagnosis in 12 cases, after preoperative chemotherapy (CT) in 4 cases. As a part of the same procedure 9 patients underwent RLND, and 7 received a more limited lymph nodes resection. Five (31.2%) developed disease recurrence 2-34 months after diagnosis (median, 6 months) plus 1 developed progression; 6 patients died, 1 of them from secondary leukemia. Among the nine patients receiving RLND, eight are alive and disease free. This compares with only one patient surviving among the seven receiving a more limited lymph nodes resection. The estimated 25-year PFS and OS rates for all patients were 61.4% (95% CI 33.2-80.5) and 50.8% (95% CI 16.5-77.5), respectively. Conclusions. Lymph node involvement is an unfavorable prognostic factor in children with RCC. RLND appears to be a critical factor to improve the outcome. However, when compared to similar adult patients, the outcome in children appears to be better, suggesting that pediatric RCC, or the host, may be critical differences. © 2008 Wiley-Liss, Inc

Pheochromocytomas and paragangliomas in children: Data from the Italian Cooperative Study (TREP)

Pheochromocytomas (PCs) are neuroendocrine tumors arising from the chromaffin cells of the adrenal gland, and paragangliomas (PGLs) are their extra-adrenal counterparts arising from ganglia along the sympathetic/parasympathetic chain. Surgery is the cornerstone of treatment. A sporatic or inherited germline mutation is commonly associated

Do more with less? Lobectomy vs. segmentectomy for patients with congenital pulmonary malformations

Background: Congenital Pulmonary Malformations (CPMs) are rare benign lesions potentially causing infective complications and/or malignant transformation, requiring surgery even when asymptomatic. CPMs are rare in adulthood but potentially detected at any age. There is not a consensus on the correct extent of resection in both adults and paediatrics. This retrospective multicentric study aims to identify the appropriate surgical resection to prevent the recurrence of the related respiratory symptoms. Methods: Between 2010 and 2020, a total of 96 patients (adults and pediatrics) underwent surgery for CPMs in 4 centers. A 2:1 propensity score matching (considering sex and lesion side) was performed, identifying 2 groups: 50 patients underwent lobectomy (group A) and 25 sub-lobar resections (group B). Clinical and histopathological characteristics, early and late complications, and symptom recurrence were retrospectively analyzed and compared between the two groups by univariate and multivariate analysis. Results: Patients who underwent lobectomy had a statistically significant lower rate of recurrence (4% vs. 24% of group B, p = 0.014) and a lower rate of intraoperative complications (p = 0.014). Logistic regression identified sub-lobar resection (p = 0.040), intra- and post-operative complications (p = 0.105 and 0.022),and associated developed neoplasm (p = 0.062) as possible risk factors for symptom recurrence after surgery. Conclusions: Pulmonary lobectomy seems to be the most effective surgical treatment for CPMs, guaranteeing the stable remission of symptoms and a lower rate of intra- and postoperative complications. To our knowledge, this is one of the largest studies comparing lobectomy and sub-lobar resections in patients affected by CPMs, considering the low incidence worldwide

Case Report: An unusual case of wide ileoileal intussusception associated with intestinal volvulus in a 8-months-old infant

IntroductionMidgut volvulus and intussusception are prevalent paediatric abdominal emergencies. To the best of our knowledge, this is the first reported case of a connection between intestinal volvulus and a massive intussusception.Case reportAn 8-month-old male infant was brought to the emergency room with a history of abdominal pain and vomiting for &lt;24 h. On physical examination, the child appeared restless and was found to have a circumferential hard mass of approximately 4 cm in diameter in the epigastric region. Upon admission, laboratory results showed a C-reactive protein level of 0.4 mg/dl, LDH level of 351 U/L, mild leukocytosis with a white blood cell count of 12 × 103 /µl, and 67% neutrophils. A physical exam was significant for abdominal distention, hyperresonance in percussion, and a palpable, painful epigastric mass. The findings of the operation included a dilated and ischemic intestinal loop, approximately 25 cm from the ileocecal valve, twisted upon itself for three turns. After de-rotation, an extensive occluding ileo-ileal invagination with an ischemic intestinal loop was identified, and a length of approximately 55–60 cm of the distal ileum, including the ischemic segment, was resected.DiscussionThis is the first reported case of a connection between intestinal volvulus and a massive intussusception. Currently, only two reported cases describe the connection between volvulus and intussusception, which are insufficient to establish a direct link between the two clinical conditions

Aggressive approach for spontaneous pneumothorax treatment in children with Marfan syndrome?

Background and objectivesMarfan syndrome (MS) is a systemic disease of connective tissues consisting of a variable combination of anomalies. These patients have an increased risk of spontaneous pneumothorax (SP). However, there is a scarcity of pediatric literature on management, and no specific guidelines exist. Our aim was to analyze the management of spontaneous pneumothorax in children and adolescents with Marfan syndrome, comparing syndromic and non-syndromic patients.MethodsRetrospective analysis of pediatric patients (18 years) with SP diagnosed at our tertiary pediatric hospital (January 10–June 22), with special emphasis on diagnosis, treatment, and follow-up (FU).ResultsSixty-six patients with SP were identified, with nine (13%) having MS. In terms of baseline, there were no significant differences between the groups (age, sex, asthma, symptoms, and side, first-line treatment and hospitalization length). Overall, Marfan patients had significantly more first-line treatment failures requiring additional surgery, as well as more contralateral occurrences and the need for surgery/chest drain during the follow-up. Instead, conservative management resulted in significantly more ipsilateral recurrences and the need for surgery/chest drain in Marfan patients than controls during the follow-up.ConclusionsTreatment failure, contralateral occurrence, ipsilateral recurrence, and the need for surgery/chest drain during follow-up make management of patients with Marfan syndrome and spontaneous pneumothorax more difficult. In patients with a diagnosed MS a more aggressive first-line management should be considered, bearing in mind the higher risks of this population

Antimicrobial Prophylaxis in Neonates and Children Undergoing Dental, Maxillo-Facial or Ear-Nose-Throat (ENT) Surgery: A RAND/UCLA Appropriateness Method Consensus Study

Surgical site infections (SSIs) represent a potential complication in surgical procedures, mainly because clean/contaminated surgery involves organs that are normally colonized by bacteria. Dental, maxillo-facial and ear-nose-throat (ENT) surgeries are among those that carry a risk of SSIs because the mouth and the first respiratory tracts are normally colonized by a bacterial flora. The aim of this consensus document was to provide clinicians with recommendations on surgical antimicrobial prophylaxis in neonates (&lt;28 days of chronological age) and pediatric patients (within the age range of 29 days-18 years) undergoing dental, maxillo-facial or ENT surgical procedures. These included: (1) dental surgery; (2) maxilla-facial surgery following trauma with fracture; (3) temporo-mandibular surgery; (4) cleft palate and cleft lip repair; (5) ear surgery; (6) endoscopic paranasal cavity surgery and septoplasty; (7) clean head and neck surgery; (8) clean/contaminated head and neck surgery and (9) tonsillectomy and adenoidectomy. Due to the lack of pediatric data for the majority of dental, maxillo-facial and ENT surgeries and the fact that the recommendations for adults are currently used, there is a need for ad hoc studies to be rapidly planned for the most deficient areas. This seems even more urgent for interventions such as those involving the first airways since the different composition of the respiratory microbiota in children compared to adults implies the possibility that surgical antibiotic prophylaxis schemes that are ideal for adults may not be equally effective in children