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1.
Ital J Pediatr ; 49(1): 9, 2023 Jan 16.
Article in English | MEDLINE | ID: mdl-36647148

ABSTRACT

Eosinophilic gastrointestinal disorders (EGIDs) are chronic/remittent inflammatory diseases associated with a substantial diagnostic delay, often attributable to misdiagnosis and variable clinical presentation in adults. In the pediatric population, few studies have been conducted worldwide reporting EGID diagnostic delay and its consequences on patients. This study aims to analyze and identify potential clinical factors and complications associated with a longer diagnostic time. We performed a retrospective analysis of pediatric patients with EGIDs followed at the Center for Pediatric EGIDs in Pavia, Italy. A total of 60 patients with EGIDs were enrolled. Thirty-nine (65%) patients had EoE, and 21 (35%) non-esophageal EGIDs. EGID diagnosis was achieved about 2 years after the symptom onset, and the median diagnostic time was 12 months (IQR 12-24 months). Diagnostic time was 12 months (IQR 12-69) in non-esophageal EGIDs and 12 months (IQR 4-24 months) in EoE patients. EoE patients presenting with FTT and feeding issues experienced a longer diagnostic time (p = 0.02 and p = 0.05, respectively) than children without growth and feeding impairments.In this study, symptoms appeared about 2 years before the definitive EGID diagnosis was reached, and this diagnostic time was shorter than the delay observed in other published studies. Especially in EoE children, the diagnostic time is significantly associated with impaired child growth, highlighting the importance of an early diagnosis to prevent esophageal stenosis and failure to thrive.


Subject(s)
Eosinophilia , Eosinophilic Esophagitis , Gastritis , Adult , Child , Humans , Retrospective Studies , Delayed Diagnosis , Eosinophilia/diagnosis , Gastritis/complications , Gastritis/diagnosis , Eosinophilic Esophagitis/diagnosis
2.
Surg Endosc ; 36(11): 7877-7897, 2022 11.
Article in English | MEDLINE | ID: mdl-36121503

ABSTRACT

BACKGROUND: Pediatric robotic-assisted surgeries have increased in recent years; however, guidance documents are still lacking. This study aimed to develop evidence-based recommendations, or best practice statements when evidence is lacking or inadequate, to assist surgical teams internationally. METHODS: A joint consensus taskforce of anesthesiologists and surgeons from the Italian Society of Pediatric and Neonatal Anesthesia and Intensive Care (SARNePI) and the Italian Society of Pediatric Surgery (SICP) have identified critical areas and reviewed the available evidence. The taskforce comprised 21 experts representing the fields of anesthesia (n = 11) and surgery (n = 10) from clinical centers performing pediatric robotic surgery in the Italian cities of Ancona, Bologna, Milan, Naples, Padua, Pavia, Perugia, Rome, Siena, and Verona. Between December 2020 and September 2021, three meetings, two Delphi rounds, and a final consensus conference took place. RESULTS: During the first planning meeting, the panel agreed on the specific objectives, the definitions to apply, and precise methodology. The project was structured into three subtopics: (i) preoperative patient assessment and preparation; (ii) intraoperative management (surgical and anesthesiologic); and (iii) postoperative procedures. Within these phases, the panel agreed to address a total of 18 relevant areas, which spanned preoperative patient assessment and patient selection, anesthesiology, critical care medicine, respiratory care, prevention of postoperative nausea and vomiting, and pain management. CONCLUSION: Collaboration among surgeons and anesthesiologists will be increasingly important for achieving safe and effective RAS procedures. These recommendations will provide a review for those who already have relevant experience and should be particularly useful for those starting a new program.


Subject(s)
Anesthesia , Anesthesiology , Robotic Surgical Procedures , Infant, Newborn , Child , Humans , Consensus , Critical Care
3.
Placenta ; 126: 119-124, 2022 08.
Article in English | MEDLINE | ID: mdl-35796063

ABSTRACT

INTRODUCTION: Glypican-3 (GPC3) is an oncofetal protein involved in cellular signaling, strongly expressed in the placenta, absent or diminished in postnatal life, but often increased in human malignancies. Germline loss-of-function variants of GPC3 gene are associated with Simpson-Golabi-Behmel syndrome type 1 (SGBS1), a rare recessive X-linked overgrowth disease characterized by typical facial features, congenital abnormalities, and an increased risk of developing childhood cancers. METHODS: A clinical suspicion of SGBS1 was postulated for a newborn with prenatal history of overgrowth and polyhydramnios, presenting with neonatal weight and length >99th percentile, coarse facies, iris and retinal coloboma, supernumerary nipples, and splenomegaly. While waiting for whole-genome sequencing (WGS) results, we investigated placental GPC3 immunohistochemical expression in the proband, in three additional cases of SGBS1, and disorders commonly associated with fetal macrosomia and/or placentomegaly. RESULTS: WGS in the proband identified a likely pathogenic maternally inherited missense variant in GPC3: c.1645A > G, (p.Ile549Val), and GPC3 immunohistochemistry demonstrated full-thickness loss of stain of the placental parenchyma. The same pattern ("null") was also present in the placentas of three additional cases of SGBS1, but not in those of unaffected controls. DISCUSSION: Immunohistochemical expression of GPC3 in the placenta is highly reproducible. Our findings showed that a "null pattern" of staining is predictive of SGBS1 and represents a valuable aid in the differential diagnosis of fetal macrosomias, allowing targeted genetic testing and earlier diagnosis.


Subject(s)
Genetic Diseases, X-Linked , Gigantism , Arrhythmias, Cardiac/diagnosis , Child , Female , Genetic Diseases, X-Linked/diagnosis , Genetic Diseases, X-Linked/genetics , Genetic Diseases, X-Linked/pathology , Gigantism/diagnosis , Gigantism/genetics , Gigantism/pathology , Glypicans/genetics , Heart Defects, Congenital/diagnosis , Humans , Immunohistochemistry , Infant, Newborn , Intellectual Disability/diagnosis , Placenta/pathology , Pregnancy
4.
Acta Biomed ; 93(S3): e2022196, 2022 06 06.
Article in English | MEDLINE | ID: mdl-35666113

ABSTRACT

BACKGROUND AND AIM: Lipschutz ulcers (LU) are idiopathic genital lesions characterized by the sudden appearance of painful, usually symmetric vulvar ulcers, typically occurring in sexually inactive adolescents. LU is a diagnosis of exclusion. As these lesions heal spontaneously, in the absence of tissue scarring, the therapy is mainly symptomatic and focuses on pain relief. Recurrence of LU associated with oral ulcers describes the clinical picture of complex aphthosis, which belongs to Behçet's disease (BD) pathological spectrum. Our work aims to analyze the correct diagnostic approach to recurrent aphthous, focusing on the importance of a multidisciplinary assessment and immunogenetic investigation to identify the subjects at risk of progression towards BD.   Methods: We present the case of a 12-year-old non sexually active Italian girl who was diagnosed with LU. After 15 months, she presented recurrent reactive non sexually related acute genital ulcer associated with a history of oral aphthous. According to clinical features and anamnesis, complex aphthosis was diagnosed. For diagnostic purposes, she underwent an immunogenetic analysis that showed HLA-B51 positivity. RESULTS: In the absence of clinical and laboratory criteria to define the risk of progression of complex aphtosis towards BD, we think that besides a strict follow-up, in pediatric patients with a suggestive clinical history, it is crucial to adopt a multidisciplinary approach, comprehensive of HLA investigation, in order to guarantee an early diagnosis and a prompt therapeutic intervention. CONCLUSIONS: In children and adolescents with genital ulcers, it is essential to consider all the possible differential diagnoses to undertake a timely and correct course of treatment.


Subject(s)
Behcet Syndrome , Stomatitis, Aphthous , Adolescent , Behcet Syndrome/complications , Behcet Syndrome/diagnosis , Behcet Syndrome/drug therapy , Child , Female , Genitalia , Humans , Risk Factors , Stomatitis, Aphthous/complications , Stomatitis, Aphthous/etiology , Ulcer/complications , Ulcer/diagnosis
5.
Front Pediatr ; 9: 721914, 2021.
Article in English | MEDLINE | ID: mdl-34712630

ABSTRACT

Aim: Video-assisted thoracoscopic surgery (VATS) has been widely used in the last decades. Nevertheless, the pros and cons of thoracoscopy vs. open surgery in pediatric oncology are still under debate. In literature, VATS has been applied for both diagnostic and ablative surgery to treat neurogenic tumors, thymic neoplasms, lung tumors and metastases, germ cell tumors, lymphoproliferative diseases, and other rare tumors. Recent reviews described excellent outcomes in pediatric oncology as well as in the treatment of adult lung cancer, with a significantly higher rate of mortality and complication in thoracotomy compared to VATS. We reviewed our experience on thoracoscopy in pediatric malignancy and compared it to the literature. Materials and Methods: This was a retrospective cohort-study of pediatric oncological patients who underwent VATS at our institution from 2007 to 2020, and a review of the recent literature on the topic. Results: A total of 43 procedures were performed on 38 oncological patients (18 males, 20 females). Median age was years 7.72 (0.35-18.6). Diagnosis: 10 neurogenic tumors, nine hematological diseases, five metastases, four lypoblastomas, three thymic pathologies, three germ cell tumors, two pleuropneumoblastomas, two myofibroblastic tumors, one myoepithelial carcinoma, one liposarcoma, and three suspected oncological mass. In three cases, a 3D model was elaborated to better plan the surgical approach. Diagnostic biopsies were 22 (51.1%), and ablative surgeries, 21 (48.9%). One neurogenic tumor was resected with the Da Vinci Robot. Median operative time was 120 min (30-420). A drain was left in place in 20 (46.5%) for a median of 4 days. Median length of hospitalization was 5 days (1-18). One case (2.3%) was converted (intraoperative bleeding). There were three post-operative complications (7.0%): one pneumonia, one pleural effusion, and one diaphragmatic paralysis (need for plication). Results were compared to recent literature, and morbidity and conversion rate were comparable to reviewed publications. Conclusion: VATS represents a valuable tool for diagnostic and therapeutic procedures in pediatric oncology. Nonetheless, it is a challenging technique that should be performed by expert surgeons on oncological and mini-invasive surgery. Three-dimensional reconstruction can optimize the pre-operative planning and guarantee a safer and more targeted treatment. Finally, the advent of robotics-assisted surgery represents a new challenge that may further implement the advantages of VATS.

6.
J Laparoendosc Adv Surg Tech A ; 31(12): 1367-1371, 2021 Dec.
Article in English | MEDLINE | ID: mdl-34491837

ABSTRACT

Background: Lymphangiomas represent 5% of all benign pediatric tumors. Abdominal lymphangiomas (ALs) are extremely rare. Therapy includes surgery, sclerotherapy, or pharmacological treatment. Laparoscopic resection (LR) has been already described, but mainly as case reports. The aim of this study is to present our series of ALs LR. Materials and Methods: From 2007 to 2020, 10 cases of ALs were electively treated by LR. Patients' age ranged from 4 months to 14 years. Preoperative diagnosis was achieved by ultrasonography and magnetic resonance images. In all cases LR was performed with four trocars: a 10 mm transumbilical trocar for camera and extraction and three 3-5 mm operative trocars. Results: Lymphangiomas arise from mesocolon in 5 giant cases, ileal mesentery in 3 and right adrenal gland in 2. LR was achieved without intraoperative complications and need of conversion in all cases. Two giant cases needed a percutaneous puncture under laparoscopic view to gain working space. A minimal ileal resection by video-assisted procedure was carried out in 2. Median hospital length was 4 days; no recurrence of disease at serial ultrasound examinations was seen at median follow-up of 5.9 years. Discussion: Several approaches have been proposed for AL treatment. The main challenges are the huge dimensions, the difficulty to achieve a complete resection, and the risk of recurrence. In this series, elective LR of ALs resulted as feasible and effective, and we consider it the standard surgical therapy.


Subject(s)
Digestive System Surgical Procedures , Laparoscopy , Lymphangioma , Mesocolon , Child , Humans , Infant , Lymphangioma/surgery , Magnetic Resonance Imaging
7.
Front Pediatr ; 9: 780830, 2021.
Article in English | MEDLINE | ID: mdl-35118030

ABSTRACT

AIM: The use of robotics-assisted surgery in oncology has been proved effective and safe in adults. Despite these results, the use of robotics has been rarely reported for pediatric oncology. Our review aims to evaluate the safety and feasibility of robotics-assisted surgery in this field, analyzing our experience and performing a systematic review of the most recent studies. METHODS: We reviewed all patients affected by an oncological disease who underwent a robotics-assisted procedure at our institute. We performed a systematic review of the literature from 2012 to 2021 on the subjects. FINDINGS: A total of 14 patients underwent robotics-assisted tumor resection. Eleven procedures (median age 13.2-years old) were carried out in children with adnexal lesions (seven tumor excision and four ovariectomies). Histological diagnosis was mature teratoma (six), serous papillary cystadenofibromas of the fallopian tube (two), ovarian serous cystadenoma (one), ovarian mucinous cystadenoma (one), and ovarian seromucinous cystadenoma. The median length of stay was 2 days. No recurrences or complications at a median follow-up of 2.1-years were observed. A 5-year-old girl underwent a complete posterior resection of a type 3 sacrococcygeal tumor with a robotics-assisted approach for the dissection of a possible intraabdominal residual component of the lesion. No intra- and postoperative complications were recorded. Complete excision of a recurrent differentiating neuroblastoma of the left para-renal region was performed on a 9-year-old girl. An idiopathic anaphylactic shock occurred 1 day after the procedure. At 9 months' follow-up, no local recurrences of the lesion were observed. Overall, we reported no conversion to open surgery. Lastly, a robotic excision of a growing left superior mediastinal intermixed ganglioneuroblastoma was performed on an 8-year-old girl with no postoperative complications. Follow-up was uneventful (7 months). In the literature, the rate of complications ranges from 0 to 28%, mainly related to difficult dissection and impaired anatomy. Conversion is reported in 5% of all oncological procedures, due to more invading tumors and altered anatomical features. No robotics-related complications were reported. CONCLUSION: Robotics-assisted surgery in pediatric oncology has proven to be feasible. Nevertheless, its use should be limited to selected cases and performed by highly trained oncological surgeons. Preparation and patient positioning, alongside a correct port placement, are crucial to carrying out these procedures. Further innovations in robotics may allow a wider application of this technology in pediatric oncology.

9.
J Pediatr Surg ; 55(4): 711-714, 2020 Apr.
Article in English | MEDLINE | ID: mdl-31130349

ABSTRACT

PURPOSE: The purpose of this study was to quantify the experience of five Italian centers on the diagnosis and management of isolated fallopian tube torsion (IFTT) in children. METHODS: We retrospectively reviewed the data of 20 patients aged 1-16 years of age with surgically diagnosed IFTT between 1991 and 2017 from five Italian centers of pediatric surgery. We analyzed common presenting signs, symptoms, and radiographic findings, as well as surgical interventions to describe management offering further insight into the diagnosis and treatment of this rare entity. RESULTS: Twenty cases of IFTT were collected. Median age was 13.1 years. Menarche was present in 14 cases. A clinical history of abdominal pain was present in 13 patients, whereas in 7 patients the clinical picture was an acute abdomen. Ultrasonography was the first diagnostic examination in 16 cases. Surgical approach was by laparoscopy in 16 cases and laparotomy in 4 cases. In 11 patients IFTT was associated with another pathologic condition. In 7 of the remaining 9 IFTT without pathologic association, the girls played sports. Salpingectomy was performed in 13 patients and de-torsion in 7. CONCLUSIONS: IFTT is a rare condition that seems to occur in younger adolescents. Vague clinical presentation contributes to low preoperative suspicion. IFTT should be considered in girls with abdominal pain who practice sports with sudden body movements. Preoperative suspicion may be increased based on radiographic findings of an enlarged tubular/cystic structure with adjacent normal ovary. Conservative management is controversial but could be preferred in order to provide the best option for future fertility of these girls. TYPE OF STUDY: Treatment Study (Retrospective Study) - Level IV.


Subject(s)
Fallopian Tube Diseases/diagnosis , Fallopian Tube Diseases/surgery , Torsion Abnormality/diagnosis , Torsion Abnormality/surgery , Abdomen, Acute/etiology , Adolescent , Child , Fallopian Tube Diseases/complications , Fallopian Tubes/diagnostic imaging , Female , Humans , Italy , Laparoscopy , Retrospective Studies , Salpingectomy , Sports , Torsion Abnormality/complications , Ultrasonography
10.
Pediatr Emerg Care ; 36(9): e534-e537, 2020 Sep.
Article in English | MEDLINE | ID: mdl-28441239

ABSTRACT

Traumatic abdominal wall hernias (TAWHs) can be defined as a herniation through disrupted musculature and fascia associated with blunt trauma. They are seen in approximately 1% of patients with blunt abdominal trauma. Data on TAWH in the pediatric population are very limited and principally based on case reports and a few case series. Past reports have indicated that the presence of the "handlebar sign" confers an increased risk of internal injury. Concomitant internal injuries are reported with an incidence between 25% and 70%, and occult hernias may also occur and are usually detected only by abdominal computed tomography scan and ultrasonography. The treatment of TAWH consists in surgical exploration through closure of the defect. We describe 2 cases of TAWH due to blunt impact by bicycle handlebars that occurred in our department with a brief literature review. Our objectives are to describe the variable clinical presentations and management of these events. We hope to provide a useful tool for the clinician to increase early clinical suspicion and detection of this insidious injury.


Subject(s)
Abdominal Injuries/etiology , Bicycling/injuries , Hernia, Abdominal/etiology , Wounds, Nonpenetrating/etiology , Abdominal Injuries/diagnostic imaging , Abdominal Injuries/surgery , Abdominal Wall , Adolescent , Child , Hernia, Abdominal/diagnostic imaging , Herniorrhaphy , Humans , Male , Wounds, Nonpenetrating/diagnostic imaging , Wounds, Nonpenetrating/surgery
11.
Medicine (Baltimore) ; 98(27): e15653, 2019 Jul.
Article in English | MEDLINE | ID: mdl-31277087

ABSTRACT

RATIONALE: Splenic cysts (SCs) are rare findings in children, particularly the youngest. Here, we discuss a case that is useful for the differential diagnosis and treatment of SCs. PATIENT CONCERNS: A 9-year-old Albanian boy was admitted for severe abdominal pain localized mainly in the left hypochondrium for approximately 24 hours. His medical history was without significant clinical problems. DIAGNOSIS: Splenomegaly was diagnosed during the first clinical examination, and laboratory tests showed an increase in CA 125 and CA19-9. Abdominal ultrasonography showed splenomegaly with a large hypoechoic oval formation with well-defined margins and the presence of internal fine suspension spots; abdominal magnetic resonance imaging revealed a well-defined SC. The cystic lesion caused major effects on the neighboring organs, shifting them from their normal sites. INTERVENTIONS: Considering the mass's volume, an open splenectomy was performed. Upon histopathological examination, the lesion was characterized by a stratified squamous keratinized thick lining and brownish liquid contents consisting of lymphocytes, erythrocytes, and hemosiderin-rich macrophages. These features informed the diagnosis of a giant epidermoid SC. OUTCOMES: No complications occurred in the post-operative period, and blood exams revealed the quick normalization of CA 19.9 and CA 125 levels. The boy was discharged on the eighth post-operative day. No complaints were documented during the regular follow-up. LESSONS: This case shows that modern imaging techniques are useful for the differential diagnosis between epithelial mass and SCs of different origins. Open splenectomy has been the treatment of choice for years, but future studies should clarify whether more conservative methods are associated with positive long-term outcomes and if they can also be used for large SCs.


Subject(s)
Epidermal Cyst/diagnosis , Splenic Diseases/diagnosis , Child , Diagnosis, Differential , Epidermal Cyst/pathology , Epidermal Cyst/surgery , Humans , Magnetic Resonance Imaging , Male , Splenectomy , Splenic Diseases/pathology , Splenic Diseases/surgery , Splenomegaly/diagnostic imaging , Ultrasonography
12.
Article in English | MEDLINE | ID: mdl-30970592

ABSTRACT

Complete androgen insensitivity syndrome (CAIS) is an X-linked recessive genetic disorder resulting from maternally inherited or de novo mutations involving the androgen receptor gene, situated in the Xq11-q12 region. The diagnosis is based on the presence of female external genitalia in a 46, XY human individual, with normally developed but undescended testes and complete unresponsiveness of target tissues to androgens. Subsequently, pelvic ultrasound or magnetic resonance imaging (MRI) could be helpful in confirming the absence of Mullerian structures, revealing the presence of a blind-ending vagina and identifying testes. CAIS management still represents a unique challenge throughout childhood and adolescence, particularly regarding timing of gonadectomy, type of hormonal therapy, and psychological concerns. Indeed this condition is associated with an increased risk of testicular germ cell tumour (TGCT), although TGCT results less frequently than in other disorders of sex development (DSD). Furthermore, the majority of detected tumoral lesions are non-invasive and with a low probability of progression into aggressive forms. Therefore, histological, epidemiological, and prognostic features of testicular cancer in CAIS allow postponing of the gonadectomy until after pubertal age in order to guarantee the initial spontaneous pubertal development and avoid the necessity of hormonal replacement therapy (HRT) induction. However, HRT is necessary after gonadectomy in order to prevent symptoms of hypoestrogenism and to maintain secondary sexual features. This article presents differential clinical presentations and management in patients with CAIS to emphasize the continued importance of standardizing the clinical and surgical approach to this disorder.


Subject(s)
Androgen Antagonists/therapeutic use , Androgen-Insensitivity Syndrome/drug therapy , Androgen-Insensitivity Syndrome/genetics , Androgens/therapeutic use , Hormone Replacement Therapy/methods , Neoplasms, Germ Cell and Embryonal/drug therapy , Testicular Neoplasms/drug therapy , Adolescent , Adult , Aged , Aged, 80 and over , Androgen-Insensitivity Syndrome/physiopathology , Child , Female , Humans , Male , Middle Aged , Neoplasms, Germ Cell and Embryonal/genetics , Neoplasms, Germ Cell and Embryonal/physiopathology , Prognosis , Testicular Neoplasms/genetics , Testicular Neoplasms/physiopathology , Young Adult
13.
Urol J ; 16(1): 83-85, 2019 02 21.
Article in English | MEDLINE | ID: mdl-30091128

ABSTRACT

INTRODUCTION: Testicular torsion is an emergency at any age; the aim of this study is to evaluate the role of mean platelet volume to assess the viability of the testes before surgeryMaterials and methods: We retrospectively analysed the medical records of consecutive patients who underwent surgical exploration for acute scrotal pathology between January 2014 and December 2016 in our institution. PATIENTS: were divided into two groups (detorsion of testes and orchyectomy); a third group was created as control group. All patients underwent blood exam before surgery; inclusion and exclusion criteria were created. We also evaluated the association between mean platelets volume and the testicular recovery during surgeryResult: After reviewing medical charts following the inclusion and exclusion criteria, 8 patients were enrolled inGroup 1 and 11 patients in Group 2. 33 healthy controls were enrolled in Group 3. MPV value in Group 1 resultedsignificantly different (p < 0.01) from the value in Group 2 and 3. The duration of symptoms was shorter than6 hours in 4/8 (50%) patients in Group 1; this early referral to hospital allowed prompt detorsion and testicularrecovery. In these "early-presenting" patients, MPV value was significantly lower than in patients with torsion oftesticular appendage (p = 0.01) and in controls (p = 0.001). CONCLUSION: MPV could be a useful adjunct in diagnosing TT, aiding its differential diagnosis with Torsion of thetesticular appendage. The lower MPV value in "early-presenting" patients with TT suggests a role in predicting thetestis viability, and therefore the appropriate treatment.


Subject(s)
Mean Platelet Volume , Spermatic Cord Torsion/diagnosis , Spermatic Cord Torsion/surgery , Testis/physiopathology , Adolescent , Humans , Male , Orchiectomy , Retrospective Studies , Spermatic Cord Torsion/physiopathology , Time-to-Treatment , Young Adult
14.
Article in English | MEDLINE | ID: mdl-30029490

ABSTRACT

Background: Childhood asymmetry labium majus enlargement (CALME) is an uncommon, benign condition that occurs in pre- and early pubertal girls and is characterized by a painless, fluctuating, non-tender labial swelling with normal overlying skin. Recognition of this benign condition is essential. Differentiation with several other diseases that mimic CALME and require different diagnostic and therapeutic approaches is mandatory. Two cases of CALME are described in this report. Differential diagnoses and therapeutic approaches are highlighted. Case presentation: The first case was an 11-year-old Caucasian girl referred to our hospital for the evaluation of right labium majus, which showed a palpable, painless, soft, non-tender, non-erythematous enlargement measuring approximately 2 cm with indistinct borders. Ultrasound showed a mass 23 × 18 × 12 mm in diameter. Surgical excision of the mass was performed and in the histopathological evaluation, the tissue specimens were composed of haphazardly arranged vascular channels, adipose tissue and nervous elements that were components of the vulvar soft tissue and were compatible with the diagnosis of CALME. Case 2 was a 6-year-old Caucasian girl who presented a post-traumatic painless mass of left labium majus swelling that progressively increased in volume. Ultrasound study evidenced an ill-defined heterogeneous echotexture mass 26 × 15 × 10 mm in diameter and magnetic resonance imaging confirmed these findings. Histopathological examination was performed after bioptic sampling evidencing normal constituents of vulvar soft tissue, including fibroblast, collagen, adipose tissue, blood vessels and nerves compatible with CALME. Conclusions: CALME is a particular clinical condition that occurs mainly in pre-pubertal girls and has a benign course but poses numerous problems in differential diagnosis that can be solved only with careful clinical observation and with a careful use of radiological imaging techniques. Our cases, in agreement with recent literature, suggest that radical excision is not recommended and that surgical biopsy should be taken into consideration only in cases of doubt.


Subject(s)
Cell Differentiation/physiology , Fibroblasts/physiology , Vulva/diagnostic imaging , Vulva/physiopathology , Vulvar Diseases/diagnosis , Vulvar Diseases/surgery , Child , Diagnosis, Differential , Female , Humans , Magnetic Resonance Imaging , Treatment Outcome , Ultrasonography
15.
Article in English | MEDLINE | ID: mdl-29751600

ABSTRACT

Introduction: Phylloides tumours (PTs) are rare fibroepithelial neoplasms that account for 0.3⁻0.9% of all breast tumours. These tumours typically occur in women aged 30⁻70 years. The occurrence of these tumours in older children and adolescents poses particular diagnostic and therapeutic problems. However, early diagnosis is mandatory because although most of the cases of PTs in children are benign, the borderline and malignant cases with potential negative outcomes cannot be excluded. Case presentation: A 12-year-old girl presented at the Paediatric Emergency Department for hyperaemia and warmth of the left breast that occurred a few days prior without fever. The girl experienced menarche 8 months previously. She experienced no previous trauma and she had no family history of breast cancer. On physical examination, the left breast was painful, enlarged and tender. The overlying skin was erythematous and warm. A breast ultrasonography (US) revealed a large mass with features of an abscess, including a hyperechoic wall, scattered internal echoes and hypoechoic peripheral lacunae of apparent colliquative nature. After 4 days of unsuccessful antibiotic therapy, surgical drainage was performed due to the suspicion of a mammary abscess. At the surgical incision site, the lesion was not-well circumscribed and lacked a capsule. In addition, purulent material was not detected. Histological examination revealed that the tissue alterations were compatible with benign PT. With this diagnosis, the girl underwent definitive surgical removal of the lesion. The postoperative period passed without negative events. An US performed 6 months later revealed that no new mass was present at this time, suggesting no recurrence of the tumour. Conclusion: This case shows that in the presence of a clinical picture suggesting the inflammation of the breast in adolescent females, PT should be considered as a possible diagnosis and US-guided core biopsy should be considered to confirm this suspicion. Thereafter, when surgical excision is performed, particular attention must be paid to both the preservation of all the normal breast parenchyma and future aesthetic problems.


Subject(s)
Breast Neoplasms/diagnosis , Phyllodes Tumor/diagnosis , Phyllodes Tumor/physiopathology , Child , Diagnostic Imaging , Female , Humans , Pediatrics , Phyllodes Tumor/surgery , Treatment Outcome , Ultrasonography
16.
19.
Medicine (Baltimore) ; 96(46): e8313, 2017 Nov.
Article in English | MEDLINE | ID: mdl-29145243

ABSTRACT

INTRODUCTION: The mesodiverticular band (MDB) is an embryologic remnant of the vitelline circulation, which carries the arterial supply to the Meckel diverticulum. In the event of an error of involution, a patent or nonpatent arterial band persists and extends from the mesentery to the apex of the antimesenteric diverticulum. This creates a snare-like opening through which bowel loops may herniate and become obstructed. This report describes 2 rare cases of small bowel occlusion owing to an internal hernia caused by a MDB. CASES: Case 1 was a 5-year-old boy who presented to our Emergency Department with colicky abdominal pain diffused to all abdominal quadrants. He also had 5 episodes of emesis, the last with bilious vomiting. Case 2, a 12-year-old boy, presented to our Emergency Department complaining of colicky abdominal pain. He had 2 episodes of nonbilious emesis. On physical examination, both children showed distension and tenderness of the abdomen and abdominal x-ray and ultrasound confirmed an occlusive picture without an apparent etiology. In case 1, an urgent laparotomy was performed and the MDB was ligated and cut, whereas in case 2 diagnosis and excision were performed in laparotomy. In both patients, there was a positive clinical evolution. CONCLUSION: Although MDB causing internal hernia is very rare, it should be considered in patients with a clinical picture of small bowel obstruction. In these cases, early surgery is important to prevent strangulation and gangrene of the bowel and to avoid dramatic events. Moreover, laparoscopy seems a safe and effective technique in these patients, especially in children with mild abdominal distention without surgical or trauma history, highlighting that further studies on the value of laparoscopy for the treatment of small bowel obstruction in pediatric patients are urgently needed.


Subject(s)
Hernia/embryology , Intestinal Obstruction/etiology , Child , Child, Preschool , Hernia/complications , Humans , Intestinal Obstruction/surgery , Laparoscopy , Laparotomy , Male
20.
Medicine (Baltimore) ; 96(30): e7087, 2017 Jul.
Article in English | MEDLINE | ID: mdl-28746173

ABSTRACT

RATIONAL: Patent urachus (PU) is due to an incomplete obliteration of the urachus, whereas patent omphalomesenteric duct (POMD) is due to an incomplete obliteration of the vitelline duct. These anomalies are very rarely associated with one another. We describe a case of a newborn with a PU associated with a POMD, who was diagnosed by an abdominal ultrasound (US) and laparoscopy, and managed with a minimally invasive excision. PATIENT CONCERN: A 28-day-old male neonate was referred to our hospital to investigate a delay in umbilical healing, with blood-mucinous material spillage for 3 weeks prior to the referral. The baby had no symptoms and was in good general health. DIAGNOSIS: After a thorough cleaning of the umbilical stump, a clear granuloma with a suspected fistula was evident under the seat of the ligature of the stump. An abdominal US examination revealed the formation of a full communication, starting below the umbilical stump and developing along the anterior abdominal wall that connected with the bladder dome. The US also revealed a tubular formation containing air, which was compatible with POMD, in the deepest portion of the same umbilical stump. Considering these findings, the rare diagnosis of a PU associated with a POMD duct was suspected. INTERVENTIONS: The child was then hospitalized for an elective laparoscopy that confirmed the US picture, and a minimally invasive excision was performed. OUTCOME: The postoperative course was favorable and uneventful. LESSONS: Our case underlines the importance of evaluating all persisting umbilical lesions without delay when conventional pharmacological therapies fail. Using a US as the first approach is valuable and should be supported by laparoscopy to confirm the diagnosis; a minimally invasive excision of the remnants appears to be an effective therapeutic approach.


Subject(s)
Urachus/abnormalities , Urachus/surgery , Humans , Infant, Newborn , Laparoscopy , Male , Minimally Invasive Surgical Procedures , Ultrasonography , Umbilicus/diagnostic imaging , Umbilicus/pathology , Umbilicus/surgery , Urachus/diagnostic imaging
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