Long-Term Sensory Function 3 years after Minimally Invasive Repair of Pectus Excavatum with Cryoablation.

INTRODUCTION: Minimally invasive repair of pectus excavatum (MIRPE) with intercostal nerve cryoablation (Cryo) decreases length of hospitalization and opioid use, but long-term recovery of sensation has been poorly described. The purpose of this study was to quantify long-term hypoesthesia and neuropathic pain after MIRPE with Cryo. METHODS: A prospective cohort study was conducted single-institution of patients ≤21 years who presented for bar removal. Consented patients underwent chest wall sensory testing and completed neuropathic pain screening. Chest wall hypoesthesia to cold, soft touch, and pinprick were measured as the percent of the treated anterior chest wall surface area (TACWSA); neuropathic pain was evaluated by questionnaire. RESULTS: The study enrolled 47 patients; 87% male; median age 18.4 years. The median bar dwell time was 2.9 years. A median of 2 bars were placed; 80.9% were secured with pericostal sutures. At enrollment, 46.8% of patients had identifiable chest wall hypoesthesia. The mean percentage of TACWSA with hypoesthesia was 4.7 ± 9.3% (cold), 3.9 ± 7.7% (soft touch), and 5.9 ± 11.8% (pinprick). Hypoesthesia to cold was found in 0 dermatomes in 62%, 1 dermatome in 11%, 2 dermatomes in 17% and ≥3 dermatomes in 11%. T5 was the most common dermatome with hypoesthesia. Neuropathic symptoms were identified by 13% of patients; none required treatment. CONCLUSION: In long-term follow up after MIRPE with Cryo, 46.8% of patients experienced some chest wall hypoesthesia; the average TACWSA with hypoesthesia was 4-6%. Hypoesthesia was mostly limited to 1-2 dermatomes, most commonly T5. Chronic symptomatic neuropathic pain was rare. LEVEL OF EVIDENCE: Level IV.

Risk Factors and Techniques for Safe Pectus Bar Removal in Adults After Modified Nuss Repair.

BACKGROUND: The Nuss repair involves implants designed for removal after 2 to 3 years. Although rare, significant complications can occur with bar removal, and the incidence of these complications may be higher in adults. This study was performed to review complications and risk factors associated with bar removal and discuss strategies to improve operative safety. METHODS: A retrospective study was performed including all patients after pectus excavatum repair who underwent Nuss implant removal at Mayo Clinic Arizona (Phoenix, AZ) from 2013 to 2022. RESULTS: In total, 1555 bars were removed (683 patients; 71% men; median age, 34 years[(range, 15-71 years]). Of the removals, 12.45% of patients had bars placed at outside institutions. Major complications were rare, with bleeding most common (2.05%), followed by pneumothorax (0.88%), infection (0.59%), and effusions (0.44%). Most major bleeding (85.71%) occurred from the bar track during removal and was controlled by packing the track. One patient required subsequent hematoma evacuation and transfusion. Bleeding secondary to lung injury was also successfully controlled with packing. Bar removal in 1 patient with significantly displaced bars required sternotomy and cardiopulmonary bypass as a result of aortic injury. Risk factors identified for bleeding included sternal erosion (P < .001), bar migration (P < .001), higher number of bars (P = .037), and revision of a previous pectus repair (P = 0.001). Bar migration was additionally associated with major complications (P < .001). Older age, although a risk factor for overall complications (P = 0.001), was not a risk factor for bleeding. CONCLUSIONS: Bar removal can be safely performed in most patients; however, significant complications, including bleeding, may occur. Identifying potential risk factors and being prepared for rescue maneuvers are critical to prevent catastrophic outcomes.

Epidural analgesia and abnormal coagulation in patients undergoing minimal invasive repair of pectus excavatum.

Background: Epidural analgesia (EA) is effective in patients undergoing minimal invasive repair of pectus excavatum (MIRPE) but is associated with major complications such as epidural hematomas. It is recommended to assess coagulation status in patients receiving anticoagulant therapy prior to EA, although no consensus exists in patients without a history of bleeding tendency or anticoagulant therapy. Thus, the aim of this paper was to assess 1) the prevalence of abnormal routine coagulation parameters, i.e., international normalized ratio (INR) and platelet count, and 2) the safety of EA in patients undergoing MIRPE. Methods: In this retrospective study, we identified 1,973 patients undergoing MIRPE at our center between 2001 and 2019. Complications related to EA were registered for all patients. Information on coagulation parameters was present in 929 patients. Patients with spontaneously elevated INR ≥1.5 were referred for assessment of coagulation factor VII in order to assess the cause of the elevated INR. Results: Of 929 patients with coagulation information available, 18 patients had spontaneously elevated INR ≥1.5 (1.9%). In patients with INR ≥1.5, 12 patients underwent further assessment of factor VII, with all patients having a slightly reduced factor VII close to the lower reference range. The majority of the 1,973 patients undergoing MIRPE received EA (99.6%) with very low complication rates (0.2%) and no incidence of epidural hematomas. Conclusion: In patients undergoing MIRPE, coagulation screening prior to EA should not be mandatory as it revealed no clinically relevant consequences. EA is safe with very low complication rates.

Postoperative management of pediatric patients undergoing minimally invasive repair of pectus excavatum: Where are we now?

PURPOSE: Minimally invasive repair of pectus excavatum (MIRPE) often leads to a painful and challenging recovery period. This study aims to describe the postoperative management of pediatric patients undergoing MIRPE and compare postoperative outcomes between patients using different routes of postoperative analgesia. METHODS: Retrospective chart review of pediatric patients who underwent MIRPE from July 2003 to September 2019 at a single pediatric tertiary care center. Data on pain management and course of hospital stay were ascertained. Descriptive statistics, Mann-Whitney U and Pearson Chi-Square tests were used to analyze data. A p-value <0.05 was considered significant. RESULTS: Of the 115 patients identified, 58 (50.4%) managed pain postoperatively using thoracic epidural and 57 (49.6%) used intravenous patient-controlled analgesia (IVPCA). The transition from the predominant use of epidural to IVPCA for MIRPE occurred between 2012 and 2013. Higher pain scores were reported by the IVPCA group at 6 h (p<0.001) and 12 h (p<0.001) postoperative. Patients using IVPCA had lower postoperative opioid consumption (p<0.001) and switched to oral opioids sooner than the epidural group (p<0.001). Fewer patients in the IVPCA group required urinary catheterization (p<0.001). Patients using IVPCA had a shorter hospital stay (4 days [IQR 4-5]) compared to the epidural group (5.5 [IQR 5-6]; p<0.001). Readmission was comparable at 3.48% in the total sample. CONCLUSION: Patients using intravenous patient-controlled analgesia reported higher pain scores however, this route of analgesia was associated with shorter hospital stay. Prospective studies designed to address moderator variables are required to confirm findings and develop standardized recovery protocols.

The Epidemiology behind Pectus Excavatum: Clinical Study and Review of the Literature.

INTRODUCTION: Pectus excavatum (PE) is a funnel-shaped indentation of the sternum and is the most common deformity of the chest wall. It is associated with syndromic diseases but can occur as an isolated form. Familial occurrence is assumed in up to 40% of cases, but large-scale studies are lacking. Most of the data are obtained from case reports which postulate autosomal recessive, dominant with reduced penetrance, X-linked, and multifactorial patterns of inheritance. No monogenetic cause has been identified to date. This study was designed to provide basic information on the epidemiology, family history, and comorbidity for a large cohort of isolated PE and to show that there is an inheritance pattern for PE that indicates a genetic background. MATERIALS AND METHODS: A retrospective study was done using a paper-based questionnaire for all PE patients attending two specialized centers for chest wall deformities. Patients with isolated PE were included and asked to provide information on family history and comorbidities. RESULTS: Family history was available for 78 patients. A positive family history was found in 42 patients (54%) with a total of 53 affected family members. CONCLUSION: The described family histories indicate an underlying genetic cause for PE. Identification of the genetic factors may contribute to characterize patients who are at risk of inheriting isolated PE.

Musculoskeletal deformities after thoracic surgery in children: An observational long-term follow-up study.

PURPOSE: This study reports the incidence, severity, and predictors of musculoskeletal deformities (MD), including scoliosis and chest wall anomalies, following thoracic procedures in children. METHODS: Children younger than 14 years who had thoracic surgery between 1997 and 2012 and had no other predispositions to MD, underwent longitudinal follow-ups with dedicated musculoskeletal examination performed in an esophageal atresia, orthopedic, or research clinic. Incidence of MD was calculated, and logistic regression methods were used to determine independent predictors, including sex, gestational age, age at procedure, serratus anterior muscle division, and chest tube placement. RESULTS: The study cohort consisted of 104 patients followed for a median of 10.8 years (range 3-21). A total of 56 MD developed in 41 patients (39%), including scapular winging (24; 23%), scoliosis (17; 16%), and chest wall anomalies (15; 14%). The majority of MD were subclinical, with only 8 patients [8% (6 thoracotomies, 2 thoracoscopies)] requiring intervention. Among patients who underwent thoracotomies (93, 89%), serratus anterior muscle division was the only significant predictor of the development of MD [OR 8.9; 95% CI 2.8-32.6]. CONCLUSION: Musculoskeletal deformities develop in a significant proportion of children following thoracic surgery, but most are subclinical. A muscle-sparing technique decreases the incidence of these deformities. TYPE OF STUDY: Prospective Cohort Study. LEVEL OF EVIDENCE: Level II.

Study on the correlation between OSAS and thoracic deformity in children: A retrospective single-center study in China.

OBJECTIVE: To evaluate the correlation between obstructive sleep apnea syndrome (OSAS) and the development of thoracic deformity in Children. METHODS: A retrospective analysis was performed with the medical records of 39 pediatric OSAS patients with thoracic deformity and matching 39 without thoracic deformity as control group between January 2015 and June 2019. The contrast was performed with age, gender, height, weight, body mass index (BMI), apnea/hypopnea index (AHI), the lowest oxyhemoglobin saturation (loSpO2)at night, tonsil and adenoid size, Alkaline phosphatase (ALP)and trace elements and metals between two groups. RESULTS: BMI, AHI, the lowest SpO2, Phosphorus and Zinc were the risk factors of thoracic deformity. Age, gender, disease history, the size of tonsil and adenoid, ALP and other trace elements were no significant difference occurred between two groups. CONCLUSION: OSAS characterized by apnea and hypoxia which are caused by narrow upper airway may be one cause of thoracic deformity in children. Pediatricians, thoracic and otolaryngologic surgeons should be alert to OSAS when thoracic deformities are diagnosed in children.

Analyzing Outcomes of Nuss and Ravitch Repair for Primary and Recurrent Pectus Excavatum in Adults.

BACKGROUND: Limited data exist for pectus excavatum repair in adults. We reviewed outcomes in adult patients undergoing Ravitch or Nuss pectus excavatum repairs to determine whether there was a statistical difference in postoperative complications and recurrence between primary and redo operations. METHODS: Patients undergoing pectus excavatum repair between 2001 and 2018 were excluded if they were receiving a concurrent unrelated operation, aged younger than 18 years, or had less than 1 year of follow-up (for recurrence analysis). Postoperative complications were recorded based on procedure type (Ravitch/Nuss) and iteration of repair (primary/redo). Continuous patient data were compared using Student t tests for variables such as age, length of stay, estimated blood loss, body mass index, and number of bars inserted. Fisher exact or χ2 tests were performed for postoperative complications and recurrence rates between groups. RESULTS: Of 290 patients, there were no significant differences in postoperative complications or recurrence rates between all Nuss repairs (n = 237; P = .59) and all Ravitch repairs (n = 53; P = .48), redo Nuss repairs (n = 53; P = .26) and Ravitch repairs (n=26; P = .99), and primary (P = .26) and redo Nuss (P = .10) repairs or primary (P = .99) and redo Ravitch (P = .99) repairs. There were significant differences in age, length of stay, follow-up, bars inserted, and estimated blood loss between all Nuss and Ravitch repairs (P < .05). CONCLUSIONS: Postoperative complication and recurrence rates were not statistically different between Nuss and Ravitch procedures of all types, suggesting either procedure may have utility in recurrent pectus excavatum. Further research may look to expand sample size and a prospective study investigating long-term outcomes.

Nuss procedure for patients with pectus excavatum with a history of intrathoracic surgery.

BACKGROUND: The aim of this study was to demonstrate the feasibility and safety of the Nuss procedure for patients with pectus excavatum (PE) with a history of intrathoracic surgery. PATIENTS: From April 2010 to December 2013, we performed 6 cases of PE repair in patients with a history of intrathoracic surgery. The causes of previous operations were congenital cystic adenomatoid malformation in 4 patients and congenital diaphragmatic hernia in 2. The patients' median age was 5 years (range, 4-9 years) and median preoperative pectus severity index was 4.63 (range, 3.42-10.03). Their intraoperative and postoperative courses were reviewed retrospectively. RESULTS: The mean overall operation time was 127.5 ±â€¯17.0 minutes, and the mean operation time for endoscopic pneumolysis was 28.8 ±â€¯12.3 minutes. Intraoperative exploration for pleural adhesion revealed that the endoscopic approach in the previous operation was associated with low pleural adhesion, and the open thoracotomy or laparotomy approach was associated with low to high pleural adhesion. One patient developed a pneumothorax on the first postoperative day. All the other patients had uneventful postoperative courses. All the patients received bar removal 2-3 years after bar insertion. One patient developed atelectasis after bar removal. All the other patients had an uneventful postoperative course. The mean postoperative follow-up time after bar removal was 20.1 ±â€¯14.7 months. CONCLUSIONS: History of intrathoracic surgery seems not a contraindication for the Nuss procedure. However, perioperative complications should be carefully monitored in both the bar insertion and removal operations.

Is pectus excavatum a risk factor for radiation-induced lung disease in patients undergoing radiation therapy following breast-conserving surgery?

BACKGROUND: The relationship between radiation dose to the ipsilateral lung and subsequent radiation-induced lung disease (RILD) in breast cancer patients with pectus excavatum (PE) undergoing radiation therapy (RT) to residual breast tissue after breast-conserving surgery has not yet been established. The incidence of RILD in such patients with PE, meaning that a large volume of the lung is within the radiation field, has not been determined. Therefore, the aim of this study was to determine the relationship between these factors. METHODS: The study cohort comprised 133 women who underwent three-dimensional conformal RT to residual breast tissue after breast-conserving surgery for breast cancer. Diagnoses of PE were based on Haller's, frontosagittal, and Monden's depression indices. Radiation doses to the ipsilateral lung were established from dose-volume histograms. RESULTS: Fifty of the 133 participants (37.6%) were diagnosed with RILD; all were asymptomatic. Multivariate analysis revealed a significant correlation between the incidence of RILD and the administration of > 30 Gy (V30). Surprisingly, although patients with PE received higher ipsilateral lung doses, they were less likely to develop RILD than those without PE. CONCLUSIONS: Our data indicate that the incidence of RILD is correlated with the administration of > 30 Gy (V30) and that PE is not a risk factor for RILD after RT to residual breast tissue after breast-conserving surgery for breast cancer. Surprisingly, individuals with PE may have a lower incidence of RILD than those without this condition.

[Pectus excavatum y carinatum en el síndrome de Marfan y síndromes similares: prevalencia e impacto clínico pulmonar y cardiovascular].

INTRODUCTION: Pectus excavatum (PE) and carinatum (PC) are common in Marfan syndrome (SM) and similar syndromes (SS). Patients can evolve without symptoms. In some there is depression, social adjustment disorders, pulmonary and cardiovascular symptoms in which there is controversy about their relationship with the structural damage of the thorax. OBJECTIVE: To assess the prevalence of the type of thoracic deformity in patients with MS and SS in a historical and current cohort and to analyze the clinical, pulmonary and cardiovascular impact. METHOD: Prospective study. Subjects who met the Ghent criteria and who had a complete clinical record, an echocardiogram and/or magnetic resonance imaging, computed tomography and respiratory function tests were included. RESULTS: Of a total of 338 patients with MS and SS, 112 cases with thoracic deformity were detected, the prevalence of PE and PC in SM 13.6 and 12.4, respectively, was lower in SS. There is compression and displacement of lung and right cardiac cavities by PE and the correlation between the Haller Index and the increased PASP is 44 (p = 0.009). CONCLUSIONS: The prevalence of PE and PC in SM and SS is high, which impacts on lung function and cardiovascular damage, requires corrective management of the thoracic deformity and not only implies for aesthetic purposes.

INTRODUCCIÓN: El pectus excavatum (PE) y el pectus carinatum (PC) son frecuentes en el síndrome de Marfan (SM) y en síndromes similares (SS). Los pacientes pueden evolucionar sin síntomas. En algunos hay depresión, trastornos de adaptación social, síntomas pulmonares y cardiovasculares, en los cuales hay controversia de su relación con el daño estructural del tórax. OBJETIVO: Evaluar la prevalencia del tipo de deformidad torácica en pacientes con SM y SS en una cohorte histórica y analizar el impacto clínico, pulmonar y cardiovascular. MÉTODO: Estudio prospectivo. Se incluyeron sujetos con criterios de Ghent y características específicas de cada síndrome, con expediente completo, ecocardiograma o resonancia magnética y tomografía computada, y pruebas de función respiratoria. RESULTADOS: De un total de 338 pacientes con SM y SS, se detectaron 112 casos con deformidad torácica. Prevalencia de PE y PC en SM: 13.6 y 12.4; fue menor en SS. Hay compresión y desplazamiento de pulmón y cavidades cardiacas derechas por PE. Hay correlación entre el Índice de Haller y la presión sistólica de la arteria pulmonar incrementada es de 44 (p = 0.009). CONCLUSIONES: La prevalencia de PE y PC en el SM y SS es alta, lo cual impacta en la función pulmonar y cardiovascular, en esas condiciones se requiere del manejo correctivo de la deformidad torácica y el objetivo no es estético.

Different Nuss procedures and risk management for pectus excavatum after surgery for congenital heart disease.

PURPOSE: In Nuss procedure for pectus excavatum (PE) after surgery for congenital heart disease (CHD), retrosternal adhesion may increase the risk of cardiac injury. This study aimed to explore different Nuss procedures and their implications for reducing the incidence of serious complications and improving surgical safety. METHODS: We retrospectively reviewed 49 cases (29 male and 20 female) of Nuss procedures for PE after surgery for CHD that were performed between April 2003 and December 2016. The median age was 5.8 years (range, 3.0-17.9), and the median Haller index was 4.6 (3.2-17.7). All patients were evaluated on their cardiac function and severity of PE by echocardiography and computed tomography scan, respectively. Three surgical procedures were used. Perioperative conditions were analyzed, including CHD type, interval between two operations, blood loss, operation time, hospital stay, complications, and postoperative results. RESULTS: All 49 cases were completed successfully. Fourteen cases (28.6%) involved the standard three-incision thoracoscopic Nuss procedure, 30 cases (61.2%) involved the Nuss procedure assisted by a median sternum incision, and 5 cases (10.2%) involved the Nuss procedure with sternal suspension. The median interval between the CHD surgery and Nuss procedure was 4.0 years (0.5-12.0). The median blood loss was 2.0 mL (1.0-150.0 mL). The median operation time was 45.0 min (27.0-230.0), and the median hospital stay was 6.0 days (5.0-9.0). Three patients (6.1%) experienced severe surgical complications: 2 experienced a rupture of the right atrium and 1 had pericardial injury. Patients were followed up for 7-120 months after surgery. The postoperative results were excellent in 46 cases (93.9%) and good in 3 (6.1%). Twenty-four of the 49 patients have had their bars removed. The median time for bar removal was 36.0 months (24.0-47.0). The outcome after bar removal surgery was excellent in 20 cases and good in 4. CONCLUSIONS: Patients may develop PE or worsening of preexisting PE after open heart surgery for CHD. Surgery for PE can still be performed by the standard Nuss technique without increasing the risk of cardiac injury for the patients that have had interventional cardiology procedures for CHD previously. However, the risk of cardiac injury during the Nuss procedure dramatically increases due to retrosternal adhesions that develop after open heart surgery for CHD. In our experience, the Nuss procedure is safe and feasible after open heart surgery for CHD when performed by an experienced pectus surgeon using an individualized surgical plan for each patient. TYPE OF STUDY: Retrospective study. LEVEL OF EVIDENCE: Level IV.

Force required for correcting the deformity of pectus carinatum and related multivariate analysis.

OBJECTIVE: To measure the force required for correcting pectus carinatum to the desired position and investigate the correlations of the required force with patients' gender, age, deformity type, severity and body mass index (BMI). METHODS: A total of 125 patients with pectus carinatum were enrolled in the study from August 2013 to August 2016. Their gender, age, deformity type, severity and BMI were recorded. A chest wall compressor was used to measure the force required for correcting the chest wall deformity. Multivariate linear regression was used for data analysis. RESULTS: Among the 125 patients, 112 were males and 13 were females. Their mean age was 13.7±1.5 years old, mean Haller index was 2.1±0.2, and mean BMI was 17.4±1.8 kg/m2. Multivariate linear regression analysis showed that the desirable force for correcting chest wall deformity was not correlated with gender and deformity type, but positively correlated with age and BMI and negatively correlated with Haller index. CONCLUSIONS: The desirable force measured for correcting chest wall deformities of patients with pectus carinatum positively correlates with age and BMI and negatively correlates with Haller index. The study provides valuable information for future improvement of implanted bar, bar fixation technique, and personalized surgery. TYPE OF STUDY: Retrospective study. LEVEL OF EVIDENCE: Level 3-4.

Hybrid Nuss Procedure for Pectus Excavatum With Severe Retrosternal Adhesions After Sternotomy.

BACKGROUND: The most striking feature of pectus excavatum (PE) after previous congenital heart disease (CHD) surgery through a median sternotomy is the postsurgical adhesions between the sternum and heart. For patients with severe adhesions, passing the introducer can be difficult and hazardous when performing a Nuss repair. We describe a hybrid Nuss procedure using a small subxiphoid incision for blunt and sharp anterior mediastinal dissection and using a thoracoscope to ensure the whole process of dissection is under direct visualization. METHODS: A retrospective case review was conducted of PE patients (previous CHD operation) who had undergone the hybrid Nuss procedure between January 2012 and June 2015. Demographic, treatment, and outcome variables were recorded. RESULTS: Eleven patients were included. The mean age was 4.7 ± 1.7 years (range, 3.2 to 8.9). The mean Haller index based on computed tomography was 4.15 ± 0.78 (range, 3.2 to 5.8). All cases were the symmetric type. Mean operating time was 95.5 ± 8.4 minutes (range, 80 to 110); mean blood loss was 15 ± 2.3 mL; and mean length of hospitalization was 6.0 ± 1.4 days (range, 4 to 8). There was no pneumothorax, cardiac injury, wound infection, discomfort requiring removal, or bar rotation at the average 27-month (range, 9 to 50) follow-up. Two patients had the bars removed. No reoperations were performed owing to recurrence. CONCLUSIONS: The hybrid Nuss procedure is a safe, simple modification of the Nuss repair for patients with severe retrosternal adhesions. This procedure achieves dissection with direct visualization. Hence, the risk of death due to cardiac perforation can be prevented.

Risk factors for thoracic and spinal deformities following lung resection in neonates, infants, and children.

PURPOSE: We aimed to identify the risk factors for thoracic and spinal deformities following lung resection during childhood and to elucidate whether thoracoscopic surgery reduces the risk of complications after lung resection. METHODS: We retrospectively examined the medical records of all pediatric patients who underwent lung resection for congenital lung disease at our institution between 1989 and 2014. RESULTS: Seventy-four patients underwent lung resection during the study period and were followed-up. The median age of the patients at the time of surgery was 5 months (range 1 day-13 years), and 22 were neonates. Thoracotomy and thoracoscopy were performed in 25 and 49 patients, respectively. Thoracic or spinal deformities occurred in 28 of the 74 patients (37%). Univariate analyses identified thoracotomy, being a neonate (age: <1 month) at the time of surgery, and being symptomatic at the time of surgery as risk factors for these deformities. However, a multivariate analysis indicated that only thoracotomy and being a neonate were risk factors for deformities. CONCLUSIONS: Thoracoscopic surgery reduced the risk of thoracic and spinal deformities following lung resection in children. We suggest that, where possible, lung resection should be avoided until 2 or 3 months of age.

[Complete Resection of Non-seminomatous Germ Cell Tumor with Plastron Approach].

A 17-year-old man was admitted to our hospital for the abnormal chest shadow. Chest computed tomography(CT) demonstrated mediastinal tumor, measuring 13 cm in diameter with high serum level of alpha fetoprotein (AFP) and human chorionic gonadotropin (hCG). The lesions were diagnosed as mixed germ cell tumors including a non-seminomatous malignant component by CT guided needle biopsy. After 5 courses of chemotherapy, the serum AFP and hCG were decreased almost normal level but the tumor size was not changed. Because it seemed to be difficult to get sufficient operating field with standard median sternotomy and patient wanted to treat funnel chest, we selected tumor resection with plastron approach. The tumor was completely resected with a good operation field by this procedure.

Rib overgrowth may be a contributing factor for pectus excavatum: Evaluation of prepubertal patients younger than 10years old.

BACKGROUND/PURPOSE: We compared the costal cartilage and rib length between prepubertal patients with symmetric pectus excavatum and age- and sex-matched controls without anterior chest wall depression to evaluate if rib overgrowth is a contributing factor for pectus excavatum METHODS: The sample included 18 prepubertal patients <10years old with symmetric pectus excavatum and 18 age-and sex-matched controls without chest wall deformity. The full lengths of the fourth to sixth ribs and costal cartilage were measured using three-dimensional volume-rendered computed tomography and curved multiplanar reformatting techniques. The rib and costal cartilage lengths, total combined rib and costal cartilage length, and costal index ([length of cartilage/length of rib]×100 [%]) at the fourth to sixth levels were compared between the groups. RESULTS: The rib lengths in the patient group were significantly longer than in the control group for the 6th right rib and 4th, 5th, and 6th left ribs. The costal cartilage lengths and costal indices were not different between two groups. CONCLUSIONS: In patients with symmetric pectus excavatum aged <10years old, several of the ribs were longer than those of controls, suggesting that abnormal rib overgrowth may be a contributing factor responsible for pectus excavatum rather than cartilage overgrowth.

Z-type pattern pectus excavatum/carinatum in a case of Noonan syndrome.

Noonan syndrome is a genetic condition that can present with complex thoracic defects, the management of which often presents a surgical challenge. We present the surgical approach applied to a severe combined excavatum/carinatum deformity that had resulted in a Z-type configuration of the chest in a 9-year-old girl with Noonan syndrome.

[Respiratory manifestations of Marfan's syndrome]. / Manifestations respiratoires du syndrome de Marfan.

Marfan's syndrome is a rare genetic disorder caused by a mutation of the gene FBN1, coding for the protein fibrillin-1. Cardiovascular, musculoskeletal and ophthalmic manifestations are the most commonly observed, but minor diagnostic criteria also include pulmonary manifestations. Pneumothorax, frequently relapsing, affects 5 to 11% of patients. Rib cage abnormalities (pectus excavatum or pectus carinatum) and apical blebs may contribute to their occurrence. Treatment does not require any specific procedure but there is an increased risk of recurrence. Pectus excavatum affects up to 60% of the patients, without any functional impairment in most cases. Surgery may be required (using the Nuss procedure) in case of cardiovascular or psychological symptoms. Marfan's syndrome is frequently associated with obstructive sleep apnoea, which may itself contribute to aortic dilatation. Some studies suggest a potential role of craniofacial abnormalities in the pathogenesis of sleep apnea in these patients. Pulmonologists should consider Marfan's syndrome when treating patients for recurrent spontaneous pneumothorax or rib cage abnormalities, since early detection of cardiac abnormalities improves the prognosis significantly.

Pectus excavatum and carinatum.

Pectus excavatum and carinatum are the most common morphological chest wall abnormalities. For both pectus excavatum and carinatum the pathogenesis is largely unknown although various hypotheses exist. Usually, exclusion of an underlying syndromal or connective tissue disorder is the reason for referral for genetic evaluation. A detailed anamnesis and family history are needed as well as a complete dysmorphological physical examination. If no features of an underlying disorder are detected, then the pectus excavatum/carinatum can be considered as an isolated abnormality and no further genetic studies seem indicated. Although cases of non-syndromal pectus excavatum/carinatum with a positive family history fitting Mendelian inheritance have been described, it is possible that these pedigrees represent multifactorial inheritance, as no genetic cause for familial isolated pectus excavatum/carinatum has been described yet. The recurrence risk for a non-familial iolated pectus excavatum/carinatum is unknown, but thought to be low. If other symptoms are found then appropriate further diagnostic studies are indicated as pectus excavatum/carinatum can be part of many syndromes. However, the most important and most frequently observed monogenic syndromes with pectus excavatum/carinatum are Marfan Syndrome and Noonan Syndrome.