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

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

A new custom-made bivalve brace for pectus carinatum in children and adolescents: preliminary promising experience of 140 patients from a tertiary center

IntroductionInternational research suggests that poor patient compliance is the main cause of tutor failures in the context of potential novel orthopedic bivalve braces for conservative treatment of pectus carinatum. Our entire experimental study is based on the hypothesis that a rigid bivalve brace that patients can accept could solve the main problem associated with the conservative approach—poor compliance. The hypothesis was to reduce the thickness and weight of the classic bivalve brace to ensure concealment and make it sustainable enough to be worn several hours a day without compromising its therapeutic efficacy.Materials and methodThe research was conducted from January 2020 to December 2022 to ensure follow-up of all participants for at least 6 months. In 36 months, 140 patients with pectus carinatum were assessed and conservatively treated with the studied guardian to analyze the therapeutic efficacy of the bivalve brace and patient compliance. From the initial visit, the parents and patient were informed that this is a 2-year therapeutic course during which the bivalve brace should be worn at least 23 h a day (with 1 h of abstinence per day for routine personal hygiene practices). Compliance is the key to therapy success, and the duration of treatment depends on patient adherence.ResultsThe exceptional effectiveness of the experimental brace was confirmed by both the questionnaire from the patients (with an average satisfaction rate of 8.9/10) and an assessment of the therapy's results by a properly selected medical committee (with a VAS scale satisfaction of 7.2/10 for symmetric forms and 7.1/10 for asymmetric ones).ConclusionIn conclusion, the analyzed data confirmed the research hypotheses. First, none of the 140 patients had cardiovascular diseases directly related to their condition, confirming that pectus carinatum is a pathology of a purely aesthetic nature. Second, a cheap, lightweight, and easily obscured brace significantly improved patient compliance. Along with this, the social relevance of the aesthetic aspect today may be an important factor in motivating the study cohort to adhere to therapy. In the past, esthetics and appearance were less relevant at the social level, which may have contributed to the high abandonment and reduced compliance rates of the many studies in the literature

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

The impact of pre-operative angiography on nephron-sparing surgery and outcome in wilms tumor arising from horseshoe kidney: case report and literature review

BackgroundThe horseshoe kidney (HSK) is a congenital renal anomaly characterized by the fusion of two distinct units, which are connected by a parenchymal bridge. HSK has a terminal vascular supply as a normal kidney. In the uncommon event that a Wilms Tumor (WT) (or Nephroblastoma) results from an HSK, Nephron-Sparing Surgery (NSS) is required. Since the anatomy expressed by HSK can vary substantially, an extensive preoperative evaluation of the vascularization is required to increase the likelihood of NSS and reduce complications. However, the role of pre-operative angiography (PORA) in this setting has not yet been defined. Our aim was to define the impact of PORA on NSS and its outcomes in WT arising from HSK.Case presentationWe presented a case of an 8-year-old girl with a WT in a HSK, for whom the PORA was safe and helpful in terms of NSS and surgical outcomes. Additionally, we performed a review of the literature regarding PORA's effects on the surgical and clinical outcomes of WT in patients with HSK.ConclusionPORA seemed potentially safe and useful in terms of NSS and complications. Despite its invasiveness, this interventional study may have a role in the extremely selected group of patients with WT arising from an HSK. Further studies are needed to validate our results

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

Videothoracoscopic management of hemoptysis due to anomalous bronchial vessel treated with multiple bronchial artery embolizations: a case report

IntroductionHemoptysis is an alarming clinical presentation caused by a vast number of primitive conditions (infectious, malignancies, malformations, vasculitis). However, at the root of hemoptysis, there is always a “noxa patogena” altering vessel structure, usually bronchial arteries, which are characterized by high pressure. Bronchial artery embolization (BAE) is the first-line treatment for hemoptysis for its technical and clinical success, although the long-term overall outcome is not equally adequate.Case reportA 12-year-old boy was referred to our hospital for massive hemoptysis after a history of recurrent episodes since the age of 3. The patient had been diagnosed with bilateral and widespread bronchial artery hypertrophy at another hospital and treated with several BAE procedures. We performed BAE to stabilize the child as well as an angio-CT scan, which confirmed the presence of the recently placed coil to embolize a hypertrophic bronchial arteriosus branch originating from the left thyrocervical trunk and directed to the right lower lobe. Results of previous embolization (metal coils) were found at the origin of the right inferior thyroid artery and the right costo-cervical trunk. After 21 months since his first admission to our hospital, the patient was transferred by air ambulance for a massive hemoptysis recurrence. Further BAE of the previously coiled vessel coming from the right succlavia (and right inferior thyroid artery) was impossible to perform due to the presence of the coils positioned in the past. A thoracoscopic approach was chosen: the previously identified anomalous vessel was isolated and ligated using double metal clips, two on both the proximal and distal sides. Accurate exploration of the thoracic cavity was accomplished, verifying the absence of collateral vessels coming from the diaphragmatic side. The patient was discharged in four days in good clinical.DiscussionAlthough bare-minimum invasive embolism (BAE) is still the gold standard for treatment, there are situations when it may not produce the desired clinical outcome and increase the risk of rebleeding. In these situations, minimally invasive surgical procedures using a videothoracoscopic approach can be beneficial if there is a suspicion of an aberrant vessel on a DSA or CT scan