Cryopreserved Thyroid Tissue Autotransplant in Pediatric Age Patients: A Feasibility Study and Literature Review.

BACKGROUND AND AIMS: This paper aims to study an alternative solution to hormonal replacement therapy in specific groups of patients who underwent thyroidectomy during childhood or adulthood. After cryopreservation, thyroid autotransplantation could be an alternative solution which would allow us to use the ability of the thyroid tissue of producing hormones according to the physiological needs of the body. MATERIALS AND METHODS: A feasibility study about the effects of the most modern cryopreservation techniques on the structural and functional integrity of the follicular cells of the thyroid tissue has been carried out. Patients who could benefit from the treatment have been found for both autotransplant techniques. Additionally, a literature review has been conducted. RESULTS: The histological analysis has shown that cryopreservation does not alter the original architecture, and the culture examination that cell viability is successfully preserved. Moreover, both thyroid autotransplantation studies on animals and those on humans that were found in the literature have shown good results regarding the viability and functionality of the transplant. CONCLUSIONS: The viability of cryopreserved thyroid tissue found in this study is encouraging. Further studies to evaluate the levels of FT3, FT4 and thyroglobulin in thyroid tissue after cryopreservation are needed to verify that the secretory properties of the thyrocytes have been maintained intact. Furthermore, autotransplanted cases found in the literature do not have a long-term follow-up.

Does thyroglossal duct arborization play a role in the post-surgical outcome of Sistrunk procedure in children?

PURPOSE: The purpose of the present study is to analyze thyroglossal duct cyst (TGDC) histopathological features, with focus on "arborization", in a cohort of pediatric patients who underwent surgical removal, and evaluate a possible correlation with clinical recurrences. METHODS: A retrospective analysis of all patients who underwent surgical resection for TGDC at the division of Pediatric Surgery of the University of Pisa from 2015 to 2020 was performed; for each patient, the following data were recorded: age, sex, clinical presentation, localization, size of the lesion, diagnostic tools, histopathological features, perioperative complications, recurrence and follow-up. RESULTS: With respect to arborization, following histopathological analysis 25/30 patients (83.3%) presented thyroglossal duct branching. After a median follow-up of 3.5 years, only 2 out of 30 patients (6.7%), one male and one female, respectively aged 4 y.o. and 6 y.o., presented recurrence within one year from first surgery. CONCLUSION: Surgery for TGDC remains a challenge for pediatric surgeons, while arborization was present in most of our cases which underwent surgery. With respect to the role of arborization, our study did not highlight sufficient conclusive data regarding their role in recurrence: instead, it showed wide resection as satisfactory, being the arborization present in most of the cases at histopathology.

Simultaneous Occurrence of Medullary Thyroid Carcinoma and Papillary Thyroid Carcinoma: A Case Series with Literature Review.

BACKGROUND: Papillary thyroid carcinoma (PTC) is the most common type of differentiated TC, while medullary TC (MTC) accounts for 4%. The concomitant presence of PTC and MTC is rare. METHODS: This is a retrospective, single-center observational study conducted over 16 years (2001-2017). The data were collected from the clinical records of patients who underwent total thyroidectomy at the Endocrine Unit-Department of Medicine of the University Hospital of Pisa, Italy. RESULTS: Over 690 analyzed cases, 650 (94.2%) were exclusive DTC, 19 exclusive MTC (2.75%) and 5 PTC/MTC (0.7%). No case of mixed medullary/follicular TC or hereditary MTC (familial MTC/multiple endocrine neoplasia type 2) was found. Among the five PTC/MTC cases, there was a male prevalence (M:F = 3:2), and all PTC components were at stage I, whereas 40% of MTC were at stage I and III and 20% of MTC were at stage II; microPTC (mPTC) was prevalent (80%) and also microMTCs were frequent (40%); 60% of MTC patients recovered, while 40% of patients developed metastatic disease. The search for germline mutations of the RET gene resulted in being negative in all cases. CONCLUSIONS: The incidence of PTC/MTC has been increasing over the past 30 years. The etiology of PTC/MTC forms is still unknown, and although this simultaneous occurrence could be only a coincidence, we cannot exclude the hypothesis of a shared genetic origin.

DICER1 Syndrome: A Multicenter Surgical Experience and Systematic Review.

DICER1 syndrome is a rare genetic disorder that predisposes patients to the development of malignant and non-malignant diseases. Presently, DICER1 syndrome diagnosis still occurs late, usually following surgical operations, affecting patients' outcomes, especially for further neoplasms, which are entailed in this syndrome. For this reason, herein we present a multicenter report of DICER1 syndrome, with the prospective aim of enhancing post-surgical surveillance. A cohort of seven patients was collected among the surgical registries of Pediatric Surgery at the University of Pisa with the General and Oncologic Surgery of Federico II, University of Naples, and the Pediatric Surgery, Regina Margherita Hospital, University of Turin. In each case, the following data were analyzed: sex, age at diagnosis, age at first surgery, clinical features, familial, genetic investigations, and follow-up. A comprehensive literature review of DICER1 cases, including case reports and multicenter studies published from 1996 to June 2022, was performed. Eventually, the retrieved data from the literature were compared with the data emerging from our cohort of patients.

Intradiaphragmatic pulmonary sequestrations: a surgical challenge. Case series.

Bronchopulmonary sequestrations (BPSs) are rare congenital anomalies characterized by non-functioning embryonic lung tissue receiving anomalous blood supply. They are most commonly located within the thorax (supradiaphragmatic) or into the abdominal cavity (infradiaphragmatic). Intradiaphragmatic extralobar BPs (IDEPS) are an exceptionally rare finding, representing a diagnostic and operative challenge. We report three cases of IDEPS and their surgical management, describing our experience and approach to such rare clinical entities. From 2016 to 2022, we treated 3 cases of IDEPS. Surgical techniques, histopathological findings and clinical outcomes were retrospectively evaluated for each case and compared. Three different surgical techniques were used to approach each lesion, from open thoracotomy to a combined laparoscopic and thoracoscopic approach. Histopathological analysis of the specimens revealed hybrid pathological features, proper of both congenital pulmonary airway malformation (CPAM) and extralobar pulmonary sequestration. IDEPS represent a surgical challenge for pediatric surgeons, given their complex surgical planning. In our experience, the thoracoscopic approach is safe and feasible when performed by trained surgeons, even though a combined thoracoscopic-laparoscopic approach allows for optimal vessels control. The presence of CPAM elements within the lesions supports their surgical removal. Additional studies are required to better characterize IDEPS and their management.

Unexpected Pediatric Uretero-Inguinal Hernia: Case-Report and Literature Review.

Uretero-inguinal hernia (UIH) is a rare condition in children. We present our experience with an unexpected UIH in a male infant with associated anomalies and a literature review of pediatric cases. A full-term male newborn was referred to our hospital for microcolon and right hydroureteronephrosis. Voiding cystourethrography showed grade-IV right vesicoureteral reflux and a dilated, convoluted, ureter protruding in the right inguinal canal, suggesting a right UIH, which was uneventfully repaired at 2 months of life. UIH is a rare type of inguinal hernia in children that pediatric urologists and surgeons should be aware of. In experienced hands, and when preoperatively suspected, surgical management is safe and effective.

Ovarian torsion in the pediatric population: predictive factors for ovarian-sparing surgery-an international retrospective multicenter study and a systematic review.

STUDY OBJECTIVE: Ovarian torsion (OT) in pediatric age is a challenging condition to diagnose and treat. To date, there is still no clear consensus about its management. Our aim was to assess some possible associated factors that can help surgeons in decision-making. DESIGN: We conducted a retrospective multicentric study of pediatric OT surgically treated between 2010 and 2020 in six Italian and German institutions, comparing our findings with a literature review of the last 10 years (2010-2020). PARTICIPANTS: Patients aged 0-18 years with a diagnosis of OT intraoperatively confirmed and surgically treated at the involved institutions. RESULTS: Ninety-seven patients with a mean age at diagnosis of 8.37 years were enrolled in the study. Severe abdominal pain was present in 82 patients (84.5%). Eighty children (82.5%) presented an enlarged ovary with an US diameter > 5 cm and only 32 (40%) of them underwent conservative surgery. A laparoscopic approach was performed in 60 cases (61.9%) although in 15 (15.5%) conversion to open surgery was deemed necessary. A functional cyst was present in 49 patients (50.5%) while 11 children (11.3%) suffered from OT on a normal ovary. CONCLUSIONS: Our results showed that a post-menarchal age (p = .001), a pre-operative US ovarian size < 5 cm, (p = .001), the presence of severe abdominal pain (p = .002), a laparoscopic approach (p < .001), and the presence of a functional cyst (p = .002) were significantly associated with conservative surgery.

Increased trend of thyroid cancer in childhood over the last 30 years in EU countries: a call for the pediatric surgeon.

Given the increasing incidence of thyroid cancer cases affecting the pediatric population in Europe, an epidemiological analysis of the available data is essential in order to update standards of care and recommend diagnostic and therapeutic strategies coherent to the new epidemiological trend. An observational study was conducted, considering the time interval 1991-2012, acquiring data collected by the International Agency for Research on Cancer (IARC) through the "Cancer Incidence in 5 continents plus" (CI5 plus) project and dividing patients into four groups of age (0-4, 5-9, 10-14, 15-19 years old). Data retrieved were compared to the latest report from Global Cancer Observatory, collected through the GLOBOCAN 2020 project, dividing patients in two groups: age 10-14, 15-19. Our findings highlighted an increasing prevalence of thyroid cancer rates in patients aged from 10 to 15 years old and from 15 to 19 years old, mainly in the female population, in Belarus (from 1991 to 2007), Cyprus, and Italy (from 2008 to 2012). Moreover, a further increase in incidence was highlighted in Italy according to data from GLOBOCAN 2020, with respect to other European countries. CONCLUSION: An increasing prevalence of thyroid cancer is seen in age groups 10-19. Further research is required to understand the underlying cause and concomitantly identify the most proper screening and diagnostic techniques of thyroid nodules. Indeed, this is crucial to reach optimal surgical standards and better prognosis, possibly with more conservative approaches and maybe less postoperative complications. WHAT IS KNOWN: • Thyroid cancer is the 4th most common malignancy in children. WHAT IS NEW: • In the last decades, an increasing pattern has been recorded. • In the US, it has outlined breast cancers as the most common cancer in females.

Targeting SIRT1 Rescues Age- and Obesity-Induced Microvascular Dysfunction in Ex Vivo Human Vessels.

BACKGROUND: Experimental evidence suggests a key role of SIRT1 (silent information regulator 1) in age- and metabolic-related vascular dysfunction. Whether these effects hold true in the human microvasculature is unknown. We aimed to investigate the SIRT1 role in very early stages of age- and obesity-related microvascular dysfunction in humans. METHODS: Ninety-five subjects undergoing elective laparoscopic surgery were recruited and stratified based on their body mass index status (above or below 30 kg/m2) and age (above or below 40 years) in 4 groups: Young Nonobese, Young Obese, Old Nonobese, and Old Obese. We measured small resistance arteries' endothelial function by pressurized micromyography before and after incubation with a SIRT1 agonist (SRT1720) and a mitochondria reactive oxygen species (mtROS) scavenger (MitoTEMPO). We assessed vascular levels of mtROS and nitric oxide availability by confocal microscopy and vascular gene expression of SIRT1 and mitochondrial proteins by qPCR. Chromatin immunoprecipitation assay was employed to investigate SIRT1-dependent epigenetic regulation of mitochondrial proteins. RESULTS: Compared with Young Nonobese, obese and older patients showed lower vascular expression of SIRT1 and antioxidant proteins (FOXO3 [forkhead box protein O3] and SOD2) and higher expression of pro-oxidant and aging mitochondria proteins p66Shc and Arginase II. Old Obese, Young Obese and Old Nonobese groups endothelial dysfunction was rescued by SRT1720. The restoration was comparable to the one obtained with mitoTEMPO. These effects were explained by SIRT1-dependent chromatin changes leading to reduced p66Shc expression and upregulation of proteins involved in mitochondria respiratory chain. CONCLUSIONS: SIRT1 is a novel central modulator of the earliest microvascular damage induced by age and obesity. Through a complex epigenetic control mainly involving p66Shc and Arginase II, it influences mtROS levels, NO availability, and the expression of proteins of the mitochondria respiratory chain. Therapeutic modulation of SIRT1 restores obesity- and age-related endothelial dysfunction. Early targeting of SIRT1 might represent a crucial strategy to prevent age- and obesity-related microvascular dysfunction.

Case Report: Congenital Perineal Lipoma Associated With Additional External Genitalia Anomalies.

Perineal lipoma is an uncommon congenital benign tumor sometimes associated with genitourinary or anorectal malformations. Accessory scrotum and accessory labioscrotal fold are infrequent features, often concurrent with perineal tumors. We describe a single institution experience with three consecutive cases of perineal lipoma associated with external genital anomalies, and a literature review.

Development of a porcine acellular bladder matrix for tissue-engineered bladder reconstruction.

PURPOSE: Enterocystoplasty is adopted for patients requiring bladder augmentation, but significant long-term complications highlight need for alternatives. We established a protocol for creating a natural-derived bladder extracellular matrix (BEM) for developing tissue-engineered bladder, and investigated its structural and functional characteristics. METHODS: Porcine bladders were de-cellularised with a dynamic detergent-enzymatic treatment using peristaltic infusion. Samples and fresh controls were evaluated using histological staining, ultrastructure (electron microscopy), collagen, glycosaminoglycans and DNA quantification and biomechanical testing. Compliance and angiogenic properties (Chicken chorioallantoic membrane [CAM] assay) were evaluated. T test compared stiffness and glycosaminoglycans, collagen and DNA quantity. p value of < 0.05 was regarded as significant. RESULTS: Histological evaluation demonstrated absence of cells with preservation of tissue matrix architecture (collagen and elastin). DNA was 0.01 µg/mg, significantly reduced compared to fresh tissue 0.13 µg/mg (p < 0.01). BEM had increased tensile strength (0.259 ± 0.022 vs 0.116 ± 0.006, respectively, p < 0.0001) and stiffness (0.00075 ± 0.00016 vs 0.00726 ± 0.00216, p = 0.011). CAM assay showed significantly increased number of convergent allantoic vessels after 6 days compared to day 1 (p < 0.01). Urodynamic studies showed that BEM maintains or increases capacity and compliance. CONCLUSION: Dynamic detergent-enzymatic treatment produces a BEM which retains structural characteristics, increases strength and stiffness and is more compliant than native tissue. Furthermore, BEM shows angiogenic potential. These data suggest the use of BEM for development of tissue-engineered bladder for patients requiring bladder augmentation.

Factors associated with postoperative hypocalcemia following thyroidectomy in childhood.

BACKGROUND: Postoperative hypocalcemia is a frequent complication after thyroidectomy. Hypoparathyroidism may develop as transient (TtHP), with normalization within six months from surgery, or permanent (PtHP) if the patient requires replacement therapy. We analyzed factors associated with the development of postoperative hypoparathyroidism and in detail PtHP following thyroid surgery in a pediatric population. PROCEDURE: A retrospective multicenter study analyzing 326 patients was carried out. We recorded gender, age, tumor size, thyroiditis, extrathyroidal extension, lymph node dissection (central/lateral compartment, unilateral/bilateral), parathyroid autotransplantation, and histology. Additionally, calcium levels were acquired postoperatively. RESULTS: We analyzed pediatric patients ≤18 years who underwent thyroidectomy clustered into age groups (≤15 or > 15). Patients' mean follow-up was 5.8 years (1-11 years). Postoperative hypoparathyroidism occurred in 36 (11.0%): 20 cases (6.13%) developed PtHP. Postoperative hypoparathyroidism was more frequent in younger patients (P = 0.014), in larger tumors (P < 0.001), in case of extrathyroidal extension (P = 0.037), and in central compartment (P = 0.020) and bilateral lymph node dissection (P = 0.030). PtHP was more frequent in older patients (P = 0.014), in case of thyroiditis (P < 0.001), and extrathyroidal extension (P < 0.001). Concerning the first postoperative calcium level measurement, in the postoperative hypoparathyroidism group, we registered a 8.17 mg/dL value with 14% pre/postoperative decrease (ΔCa ), whereas in PtHP patient group calcium level was 7.91 mg/dL with 16.7% ΔCa . CONCLUSIONS: The risk of postoperative hypoparathyroidism is related to younger age, tumor size, central compartment and bilateral lymph node dissection, extrathyroidal extension, and decrease in postoperative calcium levels. The risk of PtHP is related to older age, thyroiditis, extrathyroidal extension, and decrease in postoperative calcium levels.

Epidemiology and Surgical Management of Foreign Bodies in the Liver in the Pediatric Population: A Systematic Review of the Literature.

Retention of foreign bodies (FB) in the liver parenchyma is a rare event in children but it can bring a heavy burden in terms of immediate and long-term complications. Multiple materials can migrate inside the liver. Clinical manifestations may vary, depending on the nature of the foreign body, its route of penetration and timing after the initial event. Moreover, the location of the FB inside the liver parenchyma may pose specific issues related to the possible complications of a challenging surgical extraction. Different clinical settings and the need for highly specialized surgical skills may influence the overall management of these children. Given the rarity of this event, a systematic review of the literature on this topic was conducted and confirmed the pivotal role of surgery in the pediatric population.

The fat anchor orchiopexy technique: results and outcomes from 150 cases surgical experience.

PURPOSE: The purpose of the study is to evaluate results and outcomes in a long-time follow-up period, by performing a novel testicular fixation procedure, known as "fat anchor orchidopexy" (FAO), for the treatment of palpable low inguinal undescended testis. MATERIALS AND METHODS: We retrospectively reviewed all patients who underwent scrotal orchiopexy technique, from May 2013 to May 2019, at the Pediatric Surgery Division of Department of Surgical Pathology, University of Pisa (Italy). FAO (Spinelli's technique) consists in anchoring the testicles to sub-scrotal fat with a single trans-scrotal incision. All the patients enrolled had history of unilateral or bilateral undescended testis. Data collected included patient's age, operative times and complications. RESULTS: A total of 150 children with cryptorchidism were treated using a single trans-scrotal orchiopexy. Of them, 130 patients (86.7%) had unilateral undescended testis and 20 (13.3%) bilateral cryptorchidism. Mean patient's age was 21 months (range: 14-28 months). All the procedures were planned in a day-surgery setting. Trans-scrotal orchiopexy was successful in all cases and no patients required an additional groin incision. No intraoperatively and postoperatively major complications were observed. Patients' post-operative pain was mild (mean pediatric visual analog scale = 2). In all cases, the healing process was rapid and no surgical wounds infections were reported during the post-operative period, referring excellent cosmesis results. During a mean 48-month follow-up period, no testicular retraction, recurrence or testis atrophy was reported. CONCLUSION: The original Spinelli's technique (FAO) proves to be a safe and effective method for the treatment of palpable or distal-to-external-inguinal-ring testes. No immediate and delayed post-surgery complications were reported. In all cases, the anchored testicle remained in the scrotal position with normal vascularization. This novel surgical technique could give better options for scrotal fixation in case of low-lying cryptorchid testes.

Correlation between testicular volume and histological findings in children with unilateral cryptorchidism: Potential impact on future fertility.

OBJECTIVES: Our aim is to assess the correlation between testicular volume and histological findings in children with unilateral cryptorchidism. METHODS: From September 2016 to August 2018, from 60 patients surgically treated for cryptorchidism, 45 children were enrolled in this single-center prospective study. Depending on the degree of testicular volume reduction, patients were divided into Group 1 with <20% reduction and Group 2 with reduction ⩾20%. Patients underwent unilateral orchidopexy and simultaneous biopsy of the undescended testis. Tanner stage was assigned. Tubular Fertility Index was measured. RESULTS: Group 1 included 20 patients (44.4%) and Group 2 included 25 patients (55.5%). Mean age was 2.10 years (range 12 months-3.8 years) in Group 1 and 2.8 years (range 18 months-4.41 years) in Group 2. Although there is a positive correlation between testicular volume and Tubular Fertility Index, no significant association was found between groups (p-value = 0.29). Furthermore, histological patterns did not differ significantly among groups. CONCLUSIONS: The degree of volume reduction in undescended testis does not seem to correlate significantly with the severity of histological changes that accompany cryptorchidism. Tubular Fertility Index could serve as objective tool for the assessment of future fertility.

Testicular germ cells tumors in adolescents and young adults: Management and outcomes from a single-center experience.

OBJECTIVE: To investigate and compare the effectiveness of active surveillance versus post-surgical active treatment, in patients with testicular germ cells tumor (TGCT). MATERIALS AND METHODS: We retrospectively analyzed 52 patients who underwent surgery for TGCT from January 2009 to December 2014. All the patients were divided into two age groups: the Group A included children-adolescents from 18 months to 21 years old, while the Group B comprised young adults from 22 to 39 years old. Clinical, histopathological, therapeutic and follow-up data were collected. RESULTS: Overall, 22 patients (42,3%) were enrolled in the Group A and 30 patients (57.7%) were categorized in the Group B. Inguinal orchiectomy was performed in all patients. Retroperitoneal lymphadenectomy was performed in 4 patients (7.7%). Post-surgical management differed based on clinical stage, resulting in active surveillance or adjuvant therapy. After an average 7 years follow-up period (range: 3.5-9.0 years), the overall survival rate is 100%. The relapse risk is significantly higher for the patients in the Group B, displaying a recurrence free-survival rate of 72% versus 95% (Group A); 11 relapses (21.1%) were recorded 2 years after surgery. Of these, 3 recurrences (12.0%) occurred in patients undergoing an active surveillance approach, while 8 (29.6%) in patients subjected to an active treatment. CONCLUSIONS: The excellent prognosis in both age groups confirms the high curability of this neoplasia. The active surveillance could represent an optimal option for low recurrence risk tumors. However, post-surgical treatments should be taken into consideration for TGCT with high risk factors, including tumor size, lymphovascular and rete testis invasion.

Surgical management of ovarian teratomas in childhood: a multicentric study on 110 cases and a literature review.

AIM OF THE STUDY: To compare a multicentric surgical experience on ovarian teratomas in childhood with the current management trends. DESIGN: A retrospective multicentric pediatric ovarian teratomas surgically treated between January 2000 and August 2020 at four Italian institutions. PubMed database was used to search for Reviews and Systematic Reviews published between January 2010 and August 2020: 15 manuscripts reported 3633 ovarian neoplasms in pediatric age, 1219 (33,5%) of which were ovarian teratomas. RESULTS: A hundred-ten patients with a mean age at diagnosis of 11.8 years were enrolled. Mature cystic teratomas accounted for the 90% of the masses. At surgery, 78 were oophorectomies and 32 were ovary sparing surgeries. Laparoscopy occurred in 16.3% of the surgeries.As regarding the current management trends, the mean age at diagnosis was 11.9 years and 80.5% of the cases were represented by mature teratomas. Of 430 procedures, 331 were oophorectomies while 99 were ovary sparing surgeries and laparoscopy was performed in 23.8% of cases. CONCLUSIONS: Ovary-sparing surgery with laparoscopic approach is increasingly offered as standard treatment for benign masses that fit the criteria for mature teratomas, in the attempt to achieve the best compromise between the preservation of fertility and the prevention of recurrences. Awareness should be raised among pediatric surgeons to reduce unnecessary radical surgery.

Thyroglossal duct cyst: Factors affecting cosmetic outcome and recurrence.

BACKGROUND: Thyroglossal duct cyst (TDC) is the most common congenital abnormality in the neck in children. The purpose of this study was to perform a comprehensive review of all cases of TDC surgically treated at a single institution and to evaluate the factors that influence the rate of recurrence, and the aesthetic outcome of the surgery on follow up. METHODS: All cases of TDC surgically treated at the Department of Pediatric Surgery at Meyer Hospital from January 2005 to December 2016 were selected. Charts from 248 patients were reviewed and risk factors for recurrence evaluated. A questionnaire was submitted to the patients' parents to determine if postoperative complications were present  and standardized neck pictures were requested, to evaluate the cosmetic result . Microsoft Office Excel 2007 for Windows and Graphpad Prism 6 were used for data management and statistical analysis. RESULTS: Simple cyst excision and post-inflammatory fibrosis (P < 0.05) were assessed as important risk factors for the recurrence of TDC. Recurrence rate on Sistrunk procedure was 5%. Variables such as post-inflammatory fibrosis before surgery (P < 0.001), the positioning of a drain (P < 0.01) and the development of recurrence (P < 0.001), negatively influenced the cosmetic result. No thyroglossal duct cyst carcinoma and no long-term postoperative complications were observed. CONCLUSIONS: Recurrence rates were higher in patients who underwent simple cyst excision instead of the Sistrunk procedure, as already reported in literature. Presence of post-inflammatory fibrosis and positioning of the drain at surgery were associated with higher rates of recurrence, as well as worse cosmetic outcome.

Pectus Carinatum: a non-invasive and objective measurement of severity.

To date, standard methods for assessing the severity of chest wall deformities are mostly linked to X-ray and CT scans. However, the use of radiations limits their use when there is a need to monitor the development of the pathology over time. This is particularly important when dealing with patients suffering from Pectus Carinatum, whose treatment mainly requires the use of corrective braces and a systematic supervision. In recent years, the assessment of severity of chest deformities by means of radiation-free devices became increasingly popular but not yet adopted as standard clinical practice. The present study aims to define an objective measure by defining a severity index (named External Pectus Carinatum Index) used to monitor the course of the disease during treatment. Computed on the optical acquisition of the patients' chest by means of an appositely devised, fast and easy-to-use, body scanner, the proposed index has been validated on a sample composed of a control group and a group of Pectus Carinatum patients. The index proved to be reliable and accurate in the characterization of the pathology, enabling the definition of a threshold that allows to distinguish the cases of patients with PC from those of healthy subjects. Graphical abstract.

Customized Cutting Template to Assist Sternotomy in Pectus Arcuatum.

PURPOSE: Pectus arcuatum is an anterior chest wall deformity that requires transverse wedge sternotomy. Determining and delivering the correct cutting angle are crucial for successful correction. This report describes the early clinical experience with a novel cutting template technology able to deliver the optimal cutting angle. DESCRIPTION: From patients' computed tomographic scans, the optimal cutting angle is obtained using computer-aided design. A template comprising slots tilted at the right cutting angle and a safety block to avoid damaging the posterior periosteum is printed through additive manufacturing. EVALUATION: The template allows surgeons to perform a precise wedge sternotomy, safely sparing the posterior periosteum in all patients, without complications. Postoperative chest roentgenograms and clinical photographs demonstrate optimal sternal realignment and cosmetic outcome. In this report, the mean operative time was 110 minutes. All patients were successfully discharged, with a mean length of stay of 4 days. CONCLUSIONS: Transverse wedge sternotomy aided by a computer-aided design-devised cutting template may reduce the technical challenge of this procedure, thereby increasing its safety and reducing operative times and hospital stay. Further research on long-term patient outcomes is necessary.