IntroductionNeonatal gastric perforation (NGP) is a rare entity. Our aim was to report our experience and review the recent literature to characterize NGP, describe associated factors, and define prognostic factors.Materials and methodsRetrospective review of all consecutive patients with NGP treated between June 2009 and December 2017 in a third level pediatric hospital. In addition, a systematic review of Medline and Scopus database was performed using a defined strategy. All articles referring to NGP published between 2005 and 2017 were retrieved. Variables considered: prematurity (<37 weeks gestation), birth weight (BW), Apgar score, prenatal complications, age at diagnosis, bag ventilation, pathogenetic events, site of perforation, treatment of perforation, sepsis, and outcome. Mann–Whitney or Fisher’s test were used as appropriate. Results are median (range) or prevalence.ResultsBetween 2009 and 2016 we treated 8 consecutive patients for NGP and 199 further cases were retrieved from the systematic review (total of 207 patients). Overall survival was 73%. Most frequently reported pathogenesis: iatrogenic (20 patients), hypoxic/ischemic or infection stress (13 patients), duodenal/jejunal obstruction (11 patients), drugs (11 patients), esophageal atresia (10 patients). 60% patients had only primary repair of the perforation as gastric surgery. Sepsis developed in 56 patients (34%).ConclusionAlthough the pathogenesis of NGP is pleomorphic, prematurity and low BWs are frequent in these patients. Reviewing our experience and the available literature, none of the variables considered, but sepsis was associated with mortality.

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Adult sclerosing rhabdomyosarcoma (ASRMS) is a rare variant of rhabdomyosarcoma with a characteristic histological appearance of small, round cells in a dense, hyalinized stroma. Although nodal metastases of soft-tissue sarcomas are considered uncommon, up to 5% overall are associated with lymph node metastases. Nonetheless, there is little literature on the cytologic characteristics of metastatic soft-tissue sarcomas in lymph nodes, and to our knowledge, there are no reports of nodal metastasis of ASRMS diagnosed by fine-needle aspiration (FNA) cytology. We report here a 55-year-old woman who presented with a right thigh mass and associated ipsilateral inguinal lymphadenopathy. Biopsy of the mass revealed a uniform population of small, round cells in a dense, sclerotic background. A diagnosis of ASRMS was rendered. Subsequently, the patient underwent FNA of an enlarged inguinal lymph node, which revealed an identical population of small, round cells in a dense, myxoid background. This case highlights the cytologic features of a rare form of rhabdomyosarcoma, and emphasizes the utility of FNA in the assessment of lymphadenopathy in the setting of a soft-tissue sarcoma.

Aim of the study: Lymphatic malformations (LMs) are rare entities, sometimes difficult to treat, that may be life-threatening when intricately connected to airway structures. Invasive treatments are occasionally required, with sclerotherapy considered the treatment of choice and surgery as a second-line approach. The aim of the present study was to evaluate our multidisciplinary team experience in treating newborns affected by LMs requiring operative management, while defining early outcomes.Methods: Retrospective review of all consecutive patients admitted for LMs requiring operative management between January 2000 and January 2019. Patients were mainly characterized based on anatomical district of the LM (and further stratified based on the development of respiratory distress), need for tracheostomy, number of sclerotherapies, indication for surgery, and residual disease beyond the 1st year. Morbidity and mortality were also evaluated. Fisher exact test and Mann–Whitney test were used as appropriate. Statistical significance was set at p &lt; 0.05.Results: Fifty-seven patients were included in the study, 36 with cervicofacial and/or mediastinal LMs and 21 with LMs of other anatomical districts. Due to the risk of developing respiratory distress at birth, patients with cervicofacial and/or mediastinal LMs were divided into two groups (8/36 group A vs. 28/36 group B). Group A patients are at higher risk for tracheostomy (7/8 group A vs. 1/28 group B, p = 0.0001) and more often require surgical reduction of the residual lymphatic abnormality (5/8 group A vs. 4/28 group B, p = 0.013). They also require sclerotherapies more often, but the difference is not statistically significant (8/8 group A vs. 19/28 group B, p = 0.15). Patients with cervicofacial/mediastinal LMs frequently suffer from persistent residual disease beyond the 1st year of life, significantly more often in group A (7/8 group A vs. 12/28 group B, p = 0.043).Conclusion: LMs are rare conditions with potential life-threatening behavior. Their intrinsic clinical complexity requires a multidisciplinary approach to the affected patients. Planning a long-term follow-up is essential because of the late-term problems those patients may experience.

Purpose To analyze the outcome after untethering surgery in patients with spinal dysraphism (SD), with or without associated anorectal malformation (ARM). Methods Patients operated on for SD, with (Group A) or without (Group B) associated ARM (1999ARM ( -2015, were included. The post-operative outcome was analyzed in the two groups in terms of improving of clinical symptoms (neuro-motor deficits, bladder dysfunction, bowel dysfunction) and of instrumental examinations (urodynamics, bladder ultrasound, neurophysiology). Fisher's exact test and χ 2 test were used as appropriate; p < 0.05 was considered statistically significant. Main results Ten patients in Group A and 24 in Group B were consecutively treated. One patient was lost at follow up. Six patients (25%) in Group B underwent prophylactic surgery. The analysis of the pre-operative symptoms in the two groups showed that a significantly higher number of patients in group A needed bowel management and presented with neuro-motor deficits, compared to group B (p = 0.0035 and p = 0.04, respectively). Group A showed a significant post-operative neuromotor improvement as compared to group B (p = 0.002). Conclusions Based on our results, untethering seems to be effective in neuro-motor symptoms in selected patients with ARM. In ARM patients, untethering surgery does not seem to benefit intestinal and urinary symptoms. The presence of the associated ARM does not seem to impact the medium-term outcome of patients operated for SD.

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