A case of diffuse persistent pulmonary emphysema: When is difficult the diagnosis?

AbstractPersistent pulmonary interstitial emphysema (PPIE) is a rare condition that occurs in both preterm and term infants. It is thought to arise from a disruption of the basement membrane of the alveolar wall allowing air entry into the interstitial space. The characteristic CT scan appearance of PPIE can be used to differentiate it from other congenital cystic lesions that may present similarly. The management of infants suffering from diffuse persistent interstitial pulmonary emphysema varies according to severity and stability of the patient, being either conservative treatment or aggressive surgical treatment by pneumonectomy. We report a case of an unstable patient with diffuse persistent interstitial pulmonary emphysema successfully treated by lobectomy as a form of conservative surgical approach

Split-appendix technique: Alternative urinary diversion for pediatric complete incontinence

We report our series of selected patients with complete incontinence in whom the appendix was divided and utilized for creating two continent catheterizable stomas. All patients were treated for urinary and fecal incontinence by split appendix technique. The appendix was divided into two different parts preserving adequate perfusion and used for creating an appendicocecostomy (ACE) and an appendicovesicostomy at the same time. After a clinical and radiological follow up, our patients referred a good acceptance and an easily management of the stomas in order to stay dry for all day from urine and feces with improving of their quality of life. The combination of ACE and Mitrofanoff principle have revolutionized the management of urinary and fecal incontinence in patients who are unable to utilize their urethra to keep themselves dry

Surgical treatment of retrosternal extraosseous Ewing Sarcoma in a 6-years old female: a clamshell approach with hemysternectomy and application of a non-crosslinked extracellular matrix

Background Ewing Sarcoma (ES) and Neuroblastoma (NB) belong to a family of tumours of primitive neuroectodermal origin (PNET) that occurs in both bone and soft tissue. Notwithstanding ES and NB are two distinct malignant tumours, sometimes there could be a link between them. Case report We describe a case of an extraosseous ES localized in the retrosternal region and the upper lobe of the right lung, which had been previously treated for NB in a 6 years old female. We treated this case with a clamshell approach which allows, in a one-step surgery, a complete excision of the mass reconstructing the hemysternectomy with a non-crosslinked matrix. Conclusion the clamshell approach is therefore useful to achieve the retrosternal space and the lung with a single surgical access. According to our experience, we consider appropriate to use a non-crosslinked matrix for sternal reconstruction

Traumatic buccal fat pad herniation in an infant

Traumatic herniation of buccal fat pad (BFP) is very rare, usually seen in young children, from 5 months to 12 years of age. A minor injury or perforation of the buccinator muscle and buccal mucosa can cause the extrusion of the buccal fat pad into the oral cavity. A differential diagnosis is very important but a history of trauma, an absence of masses before the accident, anatomical site and fatty appearance should suggest the correct diagnosis. The treatment options are usually excision or repositioning of the herniated fat. For the present case report, a 7 month-old boy, diagnosed with traumatic buccal fat pad herniation, was successfully treated with surgical excision

Chromosome 18q-Syndrome and 1p terminal duplication in a patient with bilateral vesicoureteral reflux: case report and literature revision

Background: Vesico-ureteral reflux (VUR) is a dynamic event in which a retrograde flow of urine is present into the upper tracts. VUR may occur isolated or in association with other congenital abnormalities or as part of syndromic entities. We present a patient with a bilateral primary VUR, syndromic disease caused by a large deletion of 18q (18q21.3-qter) and terminal duplication of 1p (1p36.32-p36.33). Case report: The patient was 8 years old female with a disease including moderate growth retardation, psychomotor retardation, facial dysmorphism, single umbilical artery, umbilical hernia, urachal remnant, bilateral congenital clubfeet and renal-urinary disease. Chromosomal analysis and Array-CGH revealed two heterozygous chromosomal rearrangements: 1p terminal duplication and de novo 18q terminal deletion. She referred to our clinic to evaluation of bilateral hydronephrosis and right renal cortex thinning. Voiding cystourethrography demonstrated bilateral grade IV VUR and dimercaptosuccinic acid renal scintigraphy confirmed right renal cortex thinning and showed a cortical uptake of 75% of the left kidney and 25% of the right kidney. The patient underwent ureterovesical reimplantation after failure of 3 endoscopic submeatal Deflux injections with VUR resolution. Conclusions: This is the first report involving a patient with 18q-syndrome and contemporary presence of 1p chromosomal terminal duplication. The coexistence of two chromosomal rearrangements complicates the clinical picture and creates a chimeric disorder (marked by characteristics of both chromosomal anomalies). Kidney problems, primarily VUR is reported in 15% of patients affected by 18-q syndrome and no cases is reported in the literature regarding a correlation between VUR and 1p36 chromosomal duplication

Democratic evaluation of clinical clerkship in a medical school. A case study

We report a case study of democratic evaluation of a clinical clerkship for medical students. We used a mixed model combining interviews and questionnaires to students, teachers and nurses involved in 9 weekly clerkship rotations in 7 hospital wards. The main outcome variable in quantitative analysis was the score of perceived usefulness for professional development of 42 Observable Practice Activities (OPAs) that each student should perform during clerkship. Scores were higher when the OPA was performed more consistently, was performed in small groups, and when the teaching and the evaluation methods were perceived as valid. The overall satisfaction for the program of teacher was high and did not correlate with students’ perceptions. The burden for patients was perceived as slightly higher in nurses, and did not correlate with teachers’ perceptions. When returned to students, teachers and nurses, these data could contribute to improve the program. Contrariamente ai tipi di valutazione basati su standard normativi pre-determinati dalla istituzione stessa (valutazione burocratica) o da un corpo professionale esterno (autocratica), la valutazione democratica è un tentativo aperto, indipendente, non normativo (in quanto non vincolante per gli organizzatori né i partecipanti) di dare voce a tutti gli attori coinvolti nel programma valutato. Per questo è più probabile che fornisca una descrizione del programma più dal punto di vista dei partecipanti che da quello delle aspettative degli organizzatori, fornendo così ad entrambi i gruppi suggerimenti utili per svilupparlo e migliorarlo. Abbiamo utilizzato questo approccio per valutare l’impatto della introduzione in un tirocinio clinico del CdL in Medicina di una lista di attività cliniche che gli studenti dovevano effettuare, documentandole su un apposito libretto. Si è costituito un gruppo di valutazione indipendente autorizzato dal Comitato Didattico, costituito da un docente, una specializzanda, ed un gruppo di studenti. Abbiamo utilizzato una metodologia mista, basata su questionari, domande aperte e interviste. Quale principale variabile quantitativa abbiamo scelto la percezione dell’utilità di ognuna delle attività svolte per il proprio sviluppo professionale. Alcune attività non venivano svolte regolarmente. Sono risultate effettuate il 75%, con un massimo di 100% in due reparti ad un minimo del 44%. Limitando l’analisi a queste, la maggior parte delle attività è stata valutata positivamente. Fattori che sono risultati influenzare positivamente i punteggi delle singole attività sono la divisione in piccoli gruppi (6 o meno), la validità delle modalità di presentazione e di valutazione (se previsto), la consistenza di effettuazione fra le varie rotazioni. Questi dati, restituiti ai docenti e agli studenti, possono essere utili per migliorare il programma.

Democratic evaluation of clinical clerkship in a medical school. A case study

We report a case study of democratic evaluation of a clinical clerkship for medical students. We used a mixed model combining interviews and questionnaires to students, teachers and nurses involved in 9 weekly clerkship rotations in 7 hospital wards. The main outcome variable in quantitative analysis was the score of perceived usefulness for professional development of 42 Observable Practice Activities (OPAs) that each student should perform during clerkship. Scores were higher when the OPA was performed more consistently, was performed in small groups, and when the teaching and the evaluation methods were perceived as valid. The overall satisfaction for the program of teacher was high and did not correlate with students’ perceptions. The burden for patients was perceived as slightly higher in nurses, and did not correlate with teachers’ perceptions. When returned to students, teachers and nurses, these data could contribute to improve the program.

Congenital pouch colon associated with anorectal malformation: A rare anomaly of Asian Region-experience of Kurdish centre

Background: Congenital pouch colon (CPC) is an unusual malformation associated with anorectal malformations (ARMs) that are seen especially in Asia. The aim of this study was to analyse our series of CPC associated with ARM in our centre and report our experience based on the rarity of pathology. Materials and Methods: This is a retrospective study conducted at the Department of Paediatric Surgery in North Iraq. We identified patients from a prospectively kept database, including all patients managed between 1997 and 2014. Results: In total, 17 patients (3 females and 14 males) were included; all had CPC and high ARM; male:female ratio was 5.6:1. Two females had colo-vestibular fistula, 1 had a colo-cloacal fistula, all males had imperforate anus with colovesical fistula. Pre-operative diagnosis of CPC was made in eight patients (47%). Four patients (23.5%) had an incomplete pouch colon (Type III and IV), and 13 (76%) had complete pouch colon (Type I and II). All patients were managed with staged surgery. Mortality rate was 17.6% (3/17). Conclusions: CPC associated with ARM can be defined as 'Asian' complex malformation. Few cases are reported in Literature from Europe and USA. However, it is known that it is ever more frequent the collaboration between Asian and European surgeons (as in our study), so the suspicion should be considered in all patient high ARM who come from Asian region. We reviewed literature and report our experience of 15 years, after a retrospectively analysis, to share it and add our data to their reported

Robot-assisted Heller myotomy for achalasia

Achalasia is a rare neuromuscular esophageal disorder in children. There are many surgical options to treatment including botulinum toxin (Botox) injections, oral pharmacologic therapies with nitrates and calcium channel blockers, pneumatic dilation (PD), and surgical myotomy (open surgery, endoscopy, laparoscopy and recently robotic approach). In pediatric age, usually, Heller's myotomy is the main choice. Laparoscopic approach is known and standardized. Few robotic have been published. We decided to report our first case to share our experience with scientific community

Mesenteric cystic lymphangioma mimicking an ileo-colic intussusception

Lymphangioma accounts for 5% of benign pediatric tumors and less than 1% is located in the abdomen. Indeed mesenteric cystic lymphangioma is a rare benign abdominal mass and its pathogenesis is still unknown.Mesenteric cystic lymphangioma is often diagnosed incidentally. Nevertheless, in some cases its clinical presentation can mimic several common surgical pathologies. Here we describe a case of mesenteric cystic lymphangioma mimicking an ileo-colic intussusception