Cryoanalgesia has been recently described as alternative technique for immediate and persistent pain treatment after pectus excavatum repair. Cryoanalgesia has the potentiality to reduce analgesic ...consumption and length of hospitalization. However, cryoanalgesia has not been standardized yet: the previous reports describe different techniques and systems and include only small series. In Europe, no reports on cryoanalgesia for pectus repair have been published so far.
This is a prospective single center pilot study performed in adolescents undergoing minimally invasive pectus excavatum repair with a new cryoanalgesia system, using a probe designed specifically for thoracoscopy. This new double lumen probe has the theoretical advantage of freezing only in its tip, so reducing the risk of complications.
Seven patients undergoing pectus excavatum repair were treated with cryoanalgesia performed with the new probe. No complications of cryoanalgesia were reported. Total consumption of morphine during hospital stay was between 0.1 and 0.35 mg/kg, with no side effects reported. Mean time to discharge was 2.4 days. All patients reported a good pain control with a fair need of rescue medications for pain relief during the first week after discharge, and a very good pain control without need of rescue medications during following weeks.
Our pilot study showed that the new cryoanalgesia device is efficacious in terms of pain control, hospital stay and resumption of post-operative activities. The cryoprobe designed allowed an easy and safe maneuver. A prospective trial is needed to better define the risks and benefits of this technique.
Zinner syndrome (ZS) is the association of congenital seminal vesicle cysts and ipsilateral upper urinary tract anomalies, such as multicystic displastic kidney (MCDK). This condition is rare in ...pediatric age and both diagnosis and treatment are challenging. The aim of this study was to analyze the issues in diagnosis, management, and treatment of ZS in pediatric age. The medical records of two patients with ZS were examined. Furthermore, a review of the literature on this topic in pediatric age was performed. In our experience the diagnosis of ZS was incidentally achieved in the first months of life, as a consequence of studies performed for a prenatal diagnosis of MCDK. The first patient presented unspecific and transient symptoms, the second infant was completely asymptomatic. They were conservatively treated, with a long-term follow-up planned at least until adolescence. Fifty cases of ZS in pediatric age have been reported in the literature up to now. Only 12/50 were diagnosed in the first year of life. The diagnosis was demanding, as the clinical presentation was unspecific and the results at imaging studies needed a differential diagnosis with other retrovesical masses. More than 80% of these cases were asymptomatic at long-term follow-up. Therefore, a conservative management of ZS has been accepted for asymptomatic or poorly symptomatic patients, with occasional, transient, and unspecific symptoms, such as urinary tract infections or orchyepididimytis. As the surgical management is challenging, it is proposed only in those symptomatic patients. In conclusion, ZS is rare in pediatric age. However, it should be considered in the differential diagnosis of cystic masses within the pelvis in males with ipsilateral renal anomalies. A conservative treatment with a long-term follow-up is a safe option in the management of asymptomatic or poorly symptomatic patients, thus reserving the surgical approach only in those cases with symptoms.
Bar dislocation has always been considered a fearsome complication of Minimally Invasive Repair of Pectus Excavatum (MIRPE), therefore multiple techniques and types of stabilization have been ...introduced. The aim of the study is to compare different stabilization techniques in a cohort of patients operated by the same first operator.
MIRPE was adopted at our institution in 2005. Data on MIRPE patients from January 2013 to December 2022 were collected prospectively and reviewed. Patients with a follow-up of at least 12 months were included. Throughout the years different ways of stabilization were used. Patients were divided in 3 groups according to the stabilization strategy adopted- Group A: no stabilizer; Group B: single bar fixation; Group C: bridge fixation. Dislocation was diagnosed if a bar rotated more than 30° or displaced laterally for more than 1.5 cm. We compared bar dislocation percentage of each group.
We positioned 733 bars in 468 patients. Group A included 113 bars (15.4%), Group B 415 bars (56.6%), Group C 205 bars (28%). No patients were lost at follow-up. Total dislocation rate was 4.1% (30 bars). Dislocation was observed in 10 bars of group A (8.8%), 20 bars of group B (4.8 %), 0 bars of group C (0%). Differences between groups were statistically significant.
The use of stabilizers reduced dislocation percentage. In particular, bridge fixation technique reduced to zero bar dislocation and is now our preferred technique of stabilization.
III.
•What is currently known about this topic?
Bar displacement is a common and fearsome complication after MIRPE, potentially hazardous and often requiring additional intervention. Different stabilization techniques have been described over the years.•What new information is contained in this article?
Comparing three different techniques in a large series of patients, we found bridge stabilization as the optimal technique for preventing bar dislocation.
: Complication risk in minimally invasive repair of pectus excavatum (MIRPE) is not negligible, particularly during learning curve. We reviewed the complications of a large series, evaluated the ...correlation with technical details and learning curve, and presented the strategies implemented to reduce them.
: Data on MIRPE patients from 2005 to 2020 (divided in two groups: before and after 2013) were collected prospectively and reviewed. Complications were correlated to the number and type of bar/stabilizers, and to the surgeon learning curve.
: We placed 783 bars (484 Biomet, 273 Intrauma and 26 others) in 600 patients and removed 524 bars in 436 patients. Complications occurred in 108 MIRPE (18%): 3.7% intraoperative, 14.3% postoperative. No cardiac perforations were reported; in the second period we had fewer complications (15.3% versus 28.2%) particularly in intraoperative ones (0.3% versus 9%), the intervention was faster (64 versus 83 min and 92 versus 127 for 1 and 2 bars) and hospitalization shorter (5 versus 7.5 days). Complication rate of bar removal was 2.7% (all Biomet bars): one intra-thoracic bleeding and 2 lung injuries.
: MIRPE has a significant rate of complication, especially during learning curve. Postoperative complications are more frequent and less dependent on learning curve. Severe complications can be observed during bar removal. We have adopted new bars to reduce complications, but dislocation remains an unresolved problem.
Many studies on ex-preterm babies were conducted to evaluate their respiratory sequelae, but, to our knowledge, the condition described in this paper was never reported before and is not included in ...the classifications of thoracic anomalies proposed so far.
Clinical data and images of a novel thoracic deformity observed in the last 10 years are shown. This anomaly is characterized by an indentation of the ribs on both (less frequently one) anterolateral parts of the chest wall. All our patients with this condition were ex-preterm babies. We named this novel thoracic anomaly as “postprematurity thoracic dysplasia” (PPTD). Possible etiopathogenetic mechanisms and treatment options are discussed.
We observed 8 patients with variable range of respiratory symptoms. In 2 cases the malformation caused a severe functional restriction of lung volumes and surgery was performed to improve respiratory symptoms; in other cases the symptoms were mild or absent and the malformation was a matter of concern only for cosmesis.
PPTD is a novel thoracic anomaly typical of ex-preterms. Clinical relevance is variable. In severe cases surgery can be considered.
IV.
•Postprematurity thoracic dysplasia is a novel anomaly, typical of ex-preterms.•Chest wall is typically deformed on both anterolateral aspects, owing to rib anomaly.•Ribs more frequently affected were from 6th to 8th rib.•Respiratory symptoms owing to lung volume reduction can be severe.•Surgery is proposed in severe cases; vacuum bell can be a conservative alternative.
Jeune's syndrome, or asphyxiating thoracic dystrophy (ATD), is a rare autosomal recessive disorder characterized by skeletal dysplasia. Ribs are typically short and horizontal resulting-in lethal ...variant-in severe lung hypoplasia, progressive respiratory failure, and death. Lateral thoracic expansion (LTE) consists in staggered bilateral ribs osteotomy leading to chest expansion and lung development. Studies on LTE in ATD patients report encouraging data, but the rarity of ATD implies the lack of a standardized surgical path. The aim of this report is to present our experience with LTE, the technical modification we adopted, and patients' clinical outcome.
We retrospectively reviewed data of 11 LTE performed in 7 ATD patients with lethal variant. Information regarding pre- and postoperative clinical conditions and surgical details was collected. We adopted a single-stage or a two-stage approach based on patient clinical condition. Computed tomography (CT) scan was performed before and after surgery and lung volume was calculated.
Five patients are alive, while two died in intensive care unit for other than respiratory cause (sepsis). Most patients experienced clinical improvement in terms of decreased respiratory infections rate, need for ventilation, and improved exercise tolerance. Postoperative CT scan demonstrated a median lung volume increase of 88%.
Mortality in ADT patients is high. However, LTE is a feasible and safe surgical approach, which could improve clinical conditions and survival rate. Survived patients showed postoperatively less oxygen requirement and improved clinical conditions.
Neurofibromatosis type 1 (NF1) is a neurocutaneous genetic disorder with a broad spectrum of associated signs and symptoms, including skeletal anomalies. The association of NF1 with anterior chest ...wall deformities has been recently reported, especially the pectus excavatum (PE). Over the years, several authors have suggested loss of heterozygosity (LOH) as the possible pathogenic mechanism underlying the development of the typical NF1 skeletal features. Here, we report a NF1 patient with severe chest deformity and harboring the germline heterozygous pathogenic NF1 variant NM_001042492.3: c.4271delC p.(Ala1424Glufs∗4). Through next-generation sequencing (NGS), we investigated the affected cartilage from the PE deformity and identified the additional frameshift variant NM_001042492.3: c.2953delC p.(Gln985Lysfs∗7), occurring as a somatic NF1 second hit mutation. Exome sequencing confirmed the absence of additional variants of potential pathogenic relevance. Western blot analysis showed the absence of wild-type NF1 protein in the cartilage of the patient, consistent with a somatic double inactivation (SDI) of NF1. Taken together, our findings support the role of SDI in NF1-related PE, widening the spectrum of the pathophysiological mechanisms involved in NF1-related skeletal features.
Teamwork is one of the most important trend in modern medicine. Airway team were created in many places to respond in a multidisciplinary and coordinated way to challenging clinical problems which ...were beyond the possibility of an individual management. In this chapter, we illustrate the historical steps leading to the development of an airway team in a pediatric referral hospital, describe the present teamwork activity defining the key points for the creation of a team and discussing different organization models; finally we delineate possible future directions for the airway teams in the globalized world.
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