Follow-up study of three cases of congenital microgastria.

Current knowledge on congenital microgastria is limited due to its extreme rarity, and the paucity of nutritional and quality of life follow-ups. Patients affected by congenital microgastria cases followed at out center were screened, and general and nutritional status were evaluated at follow-up visits through validated questionnaires. Three cases were included: one patient died because of a complex syndromic picture where microgastria was imperatively approached conservatively. The remaining cases underwent Hunt-Lawrence at 2 and 17 months. After 2 years and 27 years postoperatively, both patients are on full oral intake. The 28-yearold patient did not reach a BMI higher than 18. She rated her quality of life as unimpacted, with a Gastrointestinal Quality of Life Index of 111. In the other case, parents reported about their 2-yearold child an Infant Gastrointestinal Symptom Questionnaire of 13, corresponding to "no distress". Our findings confirm the literature trend supporting the role of early surgery in microgastria to improve outcomes. We presented the nutritional status and quality of life in two cases of congenital microgastria operated according to Hunt-Lawrence at a 2-year and 27-year distance, which is the longest follow-up reported to date.

Engineering Auxetic Cylinders and Intestine to Improve Longitudinal Intestinal Lengthening and Tailoring Procedure.

Auxetic materials can be exploited for coupling different types of tissues. Herein, we designed a material where the microorganism metabolic activity yields the formation of buckled/collapsed bubbles within gelling silicone cylinders thus providing auxetic properties. The finite element model of such hollow auxetic cylinders demonstrated the tubular structure to promote worm-like peristalsis. In this scenario, the described hybrid auxetic structures may be applied to the longitudinal intestinal lengthening and tailoring procedure to promote enteral autonomy in short bowel syndrome. The presented material and analytical design synergistic approach offer a pioneering step for the clinical translation of hybrid auxetic materials.

Dirofilaria repens Testicular Infection in Child, Italy.

Testicular Dirofilaria repens infection was identified and confirmed by sequence analysis in a child in northeastern Italy. Because human dirofilariasis is emerging in southern and eastern Europe, this parasitic infection should be considered in the differential diagnosis of scrotal swelling in disease-endemic countries to avoid unnecessary interventions, such as orchiectomy.

Intestinal twin-to-twin transplant for short gut: Review of the literature and discussion of a complex case.

Paediatric Intestinal Transplantation (IT) presents the highest mortality on the waiting-list due to anatomical disproportion. Living-Donor IT (LDIT) offers the best advantages and when performed among identical monozygotic twins, it also benefits from unique immunology. According to MEDLINE/Pubmed, twin-to-twin LDIT has been performed in seven cases (6:7 males, median age of 32 years). None of the patients received immunosuppression postoperatively. Only one paediatric twinto- twin LDIT was carried out with a 160-cm mid-ileum tract: an interposed 4/5-cm arterial graft was required to ensure a tensionfree anastomosis to the anterior wall of the infra-renal abdominal aorta. In contrast, venous anastomosis was done directly to the inferior cava vein. We present a case for debate of a 13- month-old SBS patient where a twin-LDIT was discussed with parents, who decided to wait after careful analysis and ethical considerations.

Uniportal Video-Assisted Thoracic Surgery in a Pediatric Hospital: Early Results and Review of the Literature.

Background: Uniportal video-assisted thoracic surgery (U-VATS) is an implemented technique in adult surgery that may aid to extend offer the benefits of thoracoscopy to a wide number of pediatric patients. Materials and Methods: Consecutive cases treated between July 2019 and July 2021 were retrospectively analyzed. Simultaneously, a MEDLINE systematic search was conducted. Results: Twelve patients (median age 13 years, median weight 44.5 kg) underwent 4 major procedures (n = 2 lobectomy, n = 2 segmentectomy) and 11 minor procedures (n = 1 bronchogenic cyst resection, n = 4 apical wedge resections and pleurodesis for pneumothorax, n = 4 wedge resections for lung nodules, and n = 2 debridement for empyema). The median observed operative time was 77 minutes. We recorded one conversion to biportal VATS. No intraoperative complications or 30-day morbidity-mortality was reported. A rate of 40% adverse postoperative events was observed (Clavien-Dindo grade I-IVa). Visual analog scale for postoperative pain recorded a median value of 0 on days 1, 2, and 3. The systematic review provided 15 full-text articles reporting 76 pediatric interventions (4 major and 72 minor procedures); among them, 1 biportal conversion, 3 mild postoperative complications, and 1 redo surgery are presented. Conclusions: As emerged from the literature review, U-VATS remains scarcely adopted by pediatric surgeons. Its feasibility is supported by the four reported major lung resections plus the four cases added on by our series. Thanks to a more rapid learning curve over conventional VATS, the uniportal technique could be accessible to a wider number of centers.

Case Report: Spontaneous lung intercostal hernia series and literature review.

Spontaneous lung intercostal hernia (SLIH) is a rare condition potentially carrying severe morbidity. About 120 cases have been described so far, with an apparently increasing number of reports in recent years. The main presenting findings are chest pain and bulging, with ecchymosis in the affected area, hemoptysis, respiratory distress, and signs of infection or incarceration being described as well. The gold standard treatment has not been established, and conservative management has been advocated as first-line treatment for asymptomatic patients. Here, we report a case series of five patients, and surgical repair was deemed necessary for four of them either at first evaluation or after failure of conservative management. One patient remains under surveillance and conservative management. We believe that SLIH surgical repair should be considered as first-line treatment for fit patients, due to the uncertainty of its mid- and long-term impact and described pejorative trend/defect enlargement. A proposed algorithm for SLIH management is also presented.

Current Surgical Concepts and Indications in the Management of the Short Bowel State: A Call for the Use of Multidisciplinary Intestinal Rehabilitation Programs.

The mainstay of management for short bowel syndrome (SBS) is to promote access to the best quality of care provided by the intestinal rehabilitation program (IRP) in specialized centres. When treating SBS patients, the main goal is to minimize disease-associated complications, as well as achieve enteral autonomy. Surgical strategies should be selected cautiously upon the actual state of the bowel with respect to what it is clinically relevant for that specific patient. To this aim, a personalized and multidisciplinary approach for such a complex syndrome is needed.

Sudden unexpected death in infancy: Severe pulmonary anatomopathological findings in spite of inconsistent clinical features.

Respiratory tract infections play a considerable pathogenetic role in many cases of sudden infant death (SID). Frequently, clinicians encounter difficulties in diagnosing the disease because of its often unspecific clinical and radiological presentation. We report three cases of sudden unexpected death in infancy (SUDI), involving two males and one female admitted to hospital due to mild respiratory distress. In all three cases, complete post-mortem investigations were successful in uncovering interstitial lung disease as the cause of death. These cases highlight the key role of infection-related interstitial lung diseases in the pathogenesis of some currently unexplained SUDI/SIDS and the diagnostic difficulties due to the variable clinical and histological pattern, thereby explaining the importance of performing complete post-mortem investigations whenever an infant dies suddenly and unexpectedly.

Coping With Oral Tongue Cancer and COVID-19 Infection.

To date, April 19, 2021, the coronavirus disease 2019 (COVID-19) caused about 140,886,773 confirmed cases and more than 3,000,000 deaths worldwide since the beginning of the pandemic. Oncology patients are usually frail due to the fear of prognosis, recurrence, and outcomes of treatments. Thus, coping with cancer is a complicated process that is necessary to overcome oncological challenge, even more in case of Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) disease. This is a brief case report on a middle-aged man affected by advanced oral tongue cancer and COVID-19, describing his experience of cancer diagnosis, surgical treatment, and rehabilitation during the hospital quarantine for COVID-19. Besides the traumatic experience due to the functional alteration in breathing, eating, and speaking caused by major surgery and the concurrent facial disfigurement, our patient had to face a COVID-19 diagnosis, which implied hospital and social isolation. The aim of this perspective work is to focus on the role of the psychological support in the management of hospital distress related to COVID-19 psychophysical loneliness or alienation. In our experience, such support should anticipate patients' oncological surgery or treatment and should be implemented through telemedicine in case of isolation or after hospital discharge.

Nasal Endoscopy in the Clinical Diagnosis of Hereditary Hemorrhagic Telangiectasia.

OBJECTIVES: To evaluate the role of nasal endoscopy for early clinical diagnosis of hereditary hemorrhagic telangiectasia (HHT) in children and to investigate the characteristics of epistaxis and mucocutaneous telangiectases in our pediatric population. STUDY DESIGN: From May 2016 to December 2019, a cross-sectional observational study was conducted, recruiting children aged 2-18 years with a parent affected by HHT. To identify the Curaçao criteria, all children underwent collection of clinical history, mucocutaneous examination, and nasal endoscopy. The clinical data were then compared with the genetic data acquired subsequently. RESULTS: Seventy children (median age, 10.8 years) were included. All underwent nasal endoscopy without complications. Forty-six children were positive by genetic testing; of these, 26 % had skin and oral telangiectases and 91 % had nasal telangiectases. The diagnostic sensitivity of the Curaçao criteria increased from 28 % (95 % CI, 16%-43 %) to 85 % (95 % CI, 71%-94 %; P < .0001) when the nasal telangiectases were included. CONCLUSIONS: The magnified and complete endoscopic view of the nasal cavities proved useful in increasing the diagnostic sensitivity of the Curaçao criteria. Such an examination turned out to be feasible and safe. For this reason, we believe that nasal endoscopy should be included in the diagnostic assessment of pediatric patients with suspected HHT.

Endoscopic surgical treatment of epistaxis in hereditary haemorrhagic telangiectasia: our experience.

OBJECTIVES: Hereditary haemorrhagic telangiectasia (HHT) is a rare autosomal dominant disease characterised by epistaxis. Surgical procedures for epistaxis vary from diathermocoagulation to nasal closure. The aim of this paper is to report our experience in endoscopic surgical management of epistaxis in HHT patients. METHODS: This is a descriptive, longitudinal study carried out at the Otorhinolaryngology Department of IRCCS Policlinico San Matteo in Pavia, a reference centre for the treatment and diagnosis of HHT. We retrospectively evaluated HHT patients who underwent surgery for epistaxis from 1996 to 2015, including only those treated with endoscopic surgery. RESULTS: Among the 591 patients hospitalised and screened for HHT, 323 (54.7%) underwent endoscopic surgery for epistaxis, for a total of 679 procedures. General anaesthesia was used in 77.2% of procedures; argon plasma coagulation was the instrument of choice in the majority of patients, followed by lasers and quantum molecular resonance technology. CONCLUSIONS: We report one of the largest cohorts undergoing endoscopic treatment of epistaxis in HHT patients. This mini-invasive surgical treatment allowed us to control epistaxis without major complications and nasal packaging and can be repeated over time. For these reasons, we recommend it as first choice in case of epistaxis in HHT patients.

An alternative way to perform diagnostic nasopharyngeal swab for SARS-CoV-2 infection.

On March 11, 2020, WHO has defined the novel coronavirus disease SARS-CoV-2 (COVID-19) outbreak as a pandemic and still today continues to affect much of the world. Among the reasons for the rapid spread of SARS-CoV-2 infection, there is not only the high transmissibility of the virus, but also the role of asymptomatic or minimally symptomatic carriers. Therefore diagnostic testing is central to contain the global pandemic. Up to now real-time reverse transcriptase polymerase chain reaction (RT-PCR)-based molecular assays for detecting SARSCoV-2 in respiratory specimens is the current reference standard for COVID-19 diagnosis. Nasopharyngeal swab is the preferred choice for SARS-CoV-2 testing; however is not always a free of complications procedure. In patients with severe coagulopathies or diseases such as HHT, the risk of nosebleeding may be high. As in all those conditions like advanced stage sinonasal neoplasms or unfavorable anatomical characteristics, the nasopharyngeal swab may not be feasible. This work reports a safe and effective procedure of nasopharyngeal swab collection for COVID-19 testing, through the transoral way, in patients with contraindication to perform it transnasally. The procedure proved feasible and well tolerated. The discomfort for the patient is comparable with the execution of an oropharyngeal swab without exposing him to additional complications. In selected cases, the procedure described represents a valid alternative to nasopharyngeal swab performed transnasally. In particular, it allows reaching the area with the highest diagnostic sensitivity. Moreover it can be performed by Otolaryngology and, with adequate training, also by non-specialist staff.

Adenoidectomy: Anatomical variables as predictive factors of intraoperative adenoid residues.

OBJECTIVES: Adenoid hypertrophy is a common cause of upper airway obstruction in children. However, after adenoidectomy, nasal obstructive symptoms may persist or recur, requiring surgical revision. The aim of this study is to evaluate if individual patient features can influence the efficacy of the traditional technique. METHODS: A retrospective observational study was conducted by recruiting patients from candidates for adenoidectomy. All children underwent conventional transoral curettage adenoidectomy with endoscopic control at the end of procedure, and in presence of adenoid residues, a concomitant revision adenoidectomy was performed. For each patient, the following data were collected: age, sex, weight, height, length of the soft palate and surgical technique used. RESULTS: In 18% of patients (113/612), the most critical areas of the nasopharynx were not reached by standard surgery, making a complete adenoidectomy difficult. In this group, the average length of the soft palate was 3.1 cm, 5 mm more than the average of the sample, and 6 mm more than the average length of patients undergoing standard surgery alone (p < 0.001). CONCLUSION: Our study confirms the hypothesis that a greater length of the soft palate conditions the results of the intervention. The length of the soft palate can be considered an intraoperative criterion to select the cases in which perform endoscopic control after the standard procedure.

Diagnostic testing for SARS-CoV-2 infection in HHT patients: nasopharyngeal versus oropharyngeal swab.

On March 11, 2020, WHO has defined the novel coronavirus disease SARS-CoV-2 (COVID-19) outbreak as a pandemic that still today continues to affect much of the world. Among the reasons for the rapid spread of SARS-CoV-2 infection, there is the role of asymptomatic or minimally symptomatic carriers. Therefore diagnostic testing is central to contain the global pandemic. Up to now real-time reverse transcriptase polymerase chain reaction-based molecular assays for detecting SARS-CoV-2 in respiratory specimens is the current reference standard for COVID-19 diagnosis. Based on current knowledge regarding the sensitivity of the molecular test, the highest positive detection rate is from lower respiratory tract specimens; alternatively it is possible to perform a nasopharyngeal or oropharyngeal swab. Nasopharyngeal swab is the preferred choice for SARS-CoV-2 testing since it seems to have a greater sensitivity; however the procedure is not always free of complications and an epistaxis can occur. Among patients with greatest risk of massive nosebleed there are HHT patients. Hereditary hemorrhagic telangiectasia is an autosomal dominant disease that leads to multiregional mucocutanous telangiectases and visceral arteriovenous malformations. Clinically, the presence of telangiectases in nasal mucosa is the cause of recurrent epistaxis. In HHT patients the execution of the nasopharyngeal swab can determine from little or no consequences to a massive epistaxis leading to the necessity of nasal packing generally followed by hospital admission. In HHT patients undergoing a diagnostic test to evaluate the SARS-CoV-2 infection status, especially in those patients with frequent epistaxis with a history of anemia and repeated hospitalizations, it is therefore advisable to perform an oropharyngeal swab. This, compared to the nasopharyngeal swab, exposes to a lower risk of severe nosebleeds related treatments, such as blood transfusions or invasive procedures. According to the risk-benefit assessment and based on our experience, we consider that, despite a lower diagnostic sensitivity, oropharyngeal swab is preferable to nasopharyngeal swab for the diagnosis of SARS CoV-2 infection in patients with HHT.

European Reference Network for Rare Vascular Diseases (VASCERN) position statement on cerebral screening in adults and children with hereditary haemorrhagic telangiectasia (HHT).

Hereditary haemorrhagic telangiectasia (HHT) is a multisystemic vascular dysplasia inherited as an autosomal dominant trait. Approximately 10 % of patients have cerebral vascular malformations, a proportion being cerebral arteriovenous malformations (AVMs) and fistulae that may lead to potentially devastating consequences in case of rupture. On the other hand, detection and treatment related-risks are not negligible, and immediate. While successful treatment can be undertaken in individual cases, current data do not support the treatment of unruptured AVMs, which also present a low risk of bleeding in HHT patients. Screening for these AVMs is therefore controversial.Structured discussions, distinctions of different cerebrovascular abnormalities commonly grouped into an "AVM" bracket, and clear guidance by neurosurgical and neurointerventional radiology colleagues enabled the European Reference Network for Rare Vascular Disorders (VASCERN-HHT) to develop the following agreed Position Statement on cerebral screening:1) First, we emphasise that neurological symptoms suggestive of cerebral AVMs in HHT patients should be investigated as in general neurological and emergency care practice. Similarly, if an AVM is found accidentally, management approaches should rely on expert discussions on a case-by-case basis and individual risk-benefit evaluation of all therapeutic possibilities for a specific lesion.2) The current evidence base does not favour the treatment of unruptured cerebral AVMs, and therefore cannot be used to support widespread screening of asymptomatic HHT patients.3) Individual situations encompass a wide range of personal, cultural and clinical states. In order to enable informed patient choice, and avoid conflicting advice, particularly arising from non-neurovascular interpretations of the evidence base, we suggest that all HHT patients should have the opportunity to discuss knowingly brain screening issues with their healthcare provider.4) Any screening discussions in asymptomatic individuals should be preceded by informed pre-test review of the latest evidence regarding preventative and therapeutic efficacies of any interventions. The possibility of harm due to detection of, or intervention on, a vascular malformation that would not have necessarily caused any consequence in later life should be stated explicitly.We consider this nuanced Position Statement provides a helpful, evidence-based framework for informed discussions between healthcare providers and patients in an emotionally charged area.