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José Miguel Alves Lobato

The Surgical approach to Pectus Excavatum:

Present and Future insights

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Mestrado Integrado em Medicina

Área: Cirurgia Pediátrica

Trabalho efetuado sob a Orientação de: Prof. Dr. Tiago Henriques-Coelho

Trabalho organizado de acordo com as normas da revista: European Journal of Pediatric Surgery

Present and Future insights

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Agradecimentos

Ao Prof. Dr. Tiago Henriques-Coelho, meu orientador, pela disponibilidade que demonstrou para ouvir um aluno de 5ºano com uma proposta e encetar os esforços para que uma pequena ideia possa quiçá crescer e dar frutos mais tarde.

Aos Professores, que mesmo órfãos do título, foram-no integralmente neste meu longo percurso académico, apoiando-me e fazendo-me crescer passo a passo, em especial a Prof. Dr. Augusta Silveira.

À minha Mãe e ao meu Pai pela força e crença que sempre demonstraram em mim, com um beijo muito sentido à minha Mãe por me fazer questionar tudo e um especial abraço ao meu Pai por sempre me ter tentado mostrar que quando pomos um pé na Lua devemos ter Marte no horizonte.

À Marta por ser quem é e pela força que me transmite diariamente, nunca esmorecendo e sendo peça fundamental neste meu percurso na Faculdade de Medicina.

Finalmente à minha Avó, que certamente reluziria um orgulho que empalideceria o sol ao me ver Médico formado, adorava que me pudesses ver agora.

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Resumo

Pectus Excavatum é uma patologia prevalente em idade pediátrica, pode afectar até 1 em cada 400 indivíduos e levar a disfunção cardíaca e pulmonar. Esta doença presume-se ser de origem genética, levando a um crescimento excessivo de estruturas cartilaginosas esternais. O gene responsável por este fenótipo ainda é desconhecido. Esta patologia caracteriza-se habitualmente por diminuição na capacidade de exercício assim como perda de auto-estima com consequências tardias no normal desenvolvimento social da criança.

Uma abordagem cirúrgica pode minimizar as complicações no desenvolvimento psico-social e fisiológico da criança. As cirurgias mais utilizadas são os procedimentos de Ravitch e de Nuss. O procedimento de Ravitch consiste numa cirurgia com exposição de estruturas ósseas e correção local de defeitos costocondrais. O procedimento de Nuss é minimamente invasivo e recorre a uma barra pré-formada que é colocada na cavidade torácica com auxílio de fibroscopia.

Neste artigo os autores sumarizam o que é correntemente empregue para corrigir esta patologia de forma cirúrgica e não-cirúrgica assim como procedimentos de diagnóstico utilizados para categorizar a mesma. Também avaliamos as principais diferenças entre ambas as técnicas cirúrgicas enfatizando as vantagens e desvantagens entre ambas. De olhos postos no futuro, os autores também estabelecem o que consideram ser o primeiro modelo experimental para Pectus Excavatum assim como referem pela primeira vez uma possível nova abordagem cirúrgica para a correcção desta deformidade.

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The Surgical Approach to Pectus Excavatum: Present and

Future insights

José Miguel A. Lobato

Faculty of Medicine of the University of Oporto Al. Prof. Hernâni Monteiro 4200 – 319 Porto, Portugal

med06054@med.up.pt

Address of the corresponding author: José Miguel A. Lobato

Faculty of Medicine of the University of Oporto

Al. Prof. Hernâni Monteiro 4200 – 319 Porto, Portugal

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Abstract

Pectus Excavatum is a prevalent pathology in the pediatric age group, it can afflict up to 1 in 400 individuals and lead to pulmonar and cardiac dysfunction. This disease appears to have a genetic origin, causing an excessive growth of cartilaginous sternal structure. The gene responsible for this phenotype is not yet known. This pathology is commonly characterized by diminished exercise capacity as well as a lower self-esteem with later consequences in the child’s normal social development.

A surgical approach can minimize the psycho-social developmental and physiological distress caused by this disease. The most frequently used surgical approaches are the Ravitch and Nuss procedures. The Ravitch procedure consists of an open chest surgery with exposition local correction of the costochondral defects. The Nuss procedure is minimally invasive and resorts to a pre-formed bar that is placed inside the thorax using fibroscopy.

In this article, the authors summarize what is currently employed to surgically and non-surgically correct this pathology, as well as diagnostic procedures utilized to categorize the disease. We also assess the main differences between both surgical techniques emphasizing their advantages and disadvantages regarding each other. Looking towards the future, the authors also establish what they consider to be the first experimental model for pectus excavatum as well as referring for the first time a putative new approach to the surgical correction of this illness.

Key words: Pectus Excavatum; Experimental model; Ravitch procedure; Nuss Procedure

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Introduction

Pectus Excavatum (PE) is a major congenital anomaly which affects mostly men over women on a 5:1 ratio and occurs in 1:400 childbirths. It consists of changes in the development of the costal arches and the sternum which cause sternal depression, often compressing mediastinic structures and usually involving the lower sternum and costal cartilages. It is present at birth but most frequently manifests itself during early childhood, the severity of the deformity is usually exacerbated when puberty ends. Cardiac and pulmonary compressions are responsible for physiologic and structural changes. [1-3]

Psychosocial issues can arise from this pathology and its exacerbation occurs in the adolescent.

Some surgical procedures have been successfully applied to the correction of this pathology. The current trend is Nuss procedure that has an obvious esthetical advantage compared to Ravitch procedure.

The purpose of this study is to review the main treatment options for PE, focus on the advantages and disadvantages, as well as propose an experimental protocol for an alternative approach to this pathology.

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Etiology

- Familial Association

Pectus Excavatum remains a pathology of unknown origin. It is believed that the cause for the disease is an exaggerated axial growth of the costal cartilages determined by an unknown genetic insult [4] There are reports of familial association of this disease, but there is no known gene or cluster of genes responsible for this pathology. [5] The inheritance pattern of the disease is varied, there are cases of autossomic dominant, recessive, X-linked and multivariate trait manifestations. Several phenotypic manifestations often occur with PE such as long extremities and easy bruising for instance. [6] Reports have also shown that up to 40% of patients have affected family members. [7] There is also a strong association between PE and Noonan and Marfan syndromes, providing the basis for suspicion of these pathologies and connective tissue diseases whenever a patient presents himself with PE. [8]

- Diagnosis and Markers

The clinical identification of PE depends heavily on the appearance of the child’s chest. Willital et al recently reported a classification procedure for congenital chest pathologies based on a flexible meter, chest CT and MRI and color-coded videorasterstereography evaluation that provides a thorough analysis for the surgeon on the numerous intricacies present in a chest congenital anomaly. [9] In the daily clinical assessment of PE, the Haller index, which is defined as the ratio of the shortest antero-posterior distance to the widest transverse distance of the chest, has been widely used as an indicator of the disease severity. The accepted value for which surgery is considered is 3,25. The value of the Haller index does not necessarily represent a direct correlation between itself and post-surgical complications although one can assume that higher indexes represent more complicated clinical situations. [10] The fact that the Haller index merely reflects the distance, and not other morphologic aspects such as the symmetry of the sternal shape, can raise the question of whether or not it is the best way to assess a patient pre-operatively concerning the severity of his illness. Integration of CT evaluation on multiple planes and standardized calculations has been proposed to reach a

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standardized Haller index (operator independent) result which is often prone to different values in the same individual when done by different clinicians.

Evaluation of the patient with PE involves numerous functional tests since the cardiac and pulmonary function can be compromised. [7, 11, 12] Decreased exercise tolerance has been one of the common findings in patients with PE. [13] Another alteration found is a decrease in stroke volume, putatively caused by the impending osseous structure on the right-sided heart. Both these changes arise in tandem and it is presumed that the resting stroke volume is preserved in affected individuals and only an inability to rise it during exercise accounts for the reported decreased exercise tolerance. [14] Electrocardiogram and echocardiography should be utilized to document alterations caused by PE. [7]

The diagnosis of the pathology can be further aided by respiratory function tests, using spirometry. There seems to be a relationship between diminished pulmonary function and increased severity of PE (increase in Haller Index) manifested by lung capacity restriction. [15] As mentioned earlier in this article, although severity is apparently associated with increased Haller index, the overall morphology and symmetry of the sternum impending on underlying structures must be taken into account as a determining factor of severity. [9]

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Treatment

- Non-surgical Approach

The Vacuum bell conservative approach is a non-surgical method resorted to avoid the scarring associated with the traditional surgical approach and the post-operative pain associated with surgical correction. It consists of placing a suction cup with a pressure to a minimum of 85% of the atmospheric pressure over the affected sternum. The use of the suction cup is non-continuous, applied twice daily at least 30 minutes each time. [16]

This approach has been suggested for patients with a symmetrical PE of mild to moderate severity and as a pretreatment modality to posterior surgical correction. Described complications are minor, mainly hematoma in the sternal region and parasthesia although rib fractures have been also reported (though rare). The initial outcome is quite positive but in the long run, the results are not so satisfying, leaving this option as somewhat less useful by itself.[17]

- Surgical Approach

The surgical approach to PE has been the established solution for this pathology since 1911, when Wilhelm Meyer performed the first repair of PE. The modern repair of PE dates to 1949 when Ravitch described a procedure that was based on the removal of deformed cartilages, division of the xiphisternal articulation and transverse cuneiform osteotomy of the sternum at the upper level of the deformity. These principles are still followed in nowadays Ravitch approach. [18] Several modifications were added afterwards in an attempt to improve the results of this procedure as well as minimize the scarring associated with an “open chest” surgery. Internal fixation of the sternum with curved steel bars, homologous rib provided support and many other techniques resorting to the likes of titanium miniplates, bioabsorbable weave and mesh bands have been used with the intent of fixing the corrected sternum in place. The Ravitch procedure though with excellent results, is a technique with several side-effects. The resection of costal cartilage in the young patients could lead to irreversible growth impairment in the chest wall with obvious complications associated with respiratory function. The asphyxiating chondrodystrophy or

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acquired Jeune syndrome is a consequence of replaced cartilage tissue with bony scar tissue, which is one of the factors that deters a surgical approach before the age of 5. [4, 5] This restrictive chest wall deformity although rare is serious and can lead to vastly diminished pulmonary function in the adult life in the context of limited thoracic volume. [19]

The most recent surgical approach to PE appeared was described in 1998 by Nuss and colleagues. The Nuss procedure, or Minimally Invasive Repair of Pectus Excavatum (MIRPE) consists of applying a substernal bar made of steel or titanium resorting to thorascopy to guide it’s placement in the thoracic cavity. The incision is minimal and the scarring is limited. [20] This technique has excellent results and minor complications, and has gradually become the surgical option for most patients. Though safe and with limited morbidity, this surgery requires a long hospital stay and opiate strong analgesia. [21] There have been reports of thoracic outlet syndrome, pericardial perforation and pneumothorax on rare occasions.[22, 23] The suggested ideal age for surgical correction via this technique is when the child is approximately 10 years old. [24]

A new experimental approach involves the use of magnets to place the sternum in its normal position. It requires the implantation of a magnetic bar under the sternum which is then pulled anteriorly by an extracorporeal magnet thus correctly placing the sternum. [25, 26] The basic physiological assumption is analogous to the one behind the Nuss procedure. Since the osseous structures still possess cartilaginous mobile elements and the individuals are treated while still growing, the pliability of the underlying tissues is enough that a small force exerted throughout time can slowly place the osseous structures in its desired local.

- Comparison between Nuss and Ravitch procedure

The absence of randomized control studies involving both techniques limits the comparison between them.

There are no discernible differences between both procedures regarding complications albeit a higher rate of reoperation was found in the Nuss

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procedure when compared to Ravitch procedure.[27] (A result opposite that of our Hospital’s clinical experience.) This is probably due to the nature of the procedure itself which depends on the anchorage of one or more metal bars under the sternum, versus a much more localized approach in the Ravitch technique. There are also more accounts of pneumothorax and hemothorax in those who underwent the Nuss procedure. This procedure has also been documented to successfully correct previously failed surgical attempts that underwent both Ravitch and Nuss procedures. [27] A higher pulmonary function test was present in those patients who underwent a previous Nuss procedure compared with those that had been operated via Ravitch technique. [28]

The Nuss procedure is generally quicker although the length of hospital stay is also slightly longer in the cases of patients who underwent the same procedure. In postoperative pain lies another difference between both procedures. The Nuss procedure frequently requires the patient to undergo epidural analgesia and for a longer duration than those who perform the Ravitch procedure. This postoperative pain may be caused by the strain that the metal bar causes on the chest wall. Since the bar is placed with the aimed final curvature, there is no adaptation period and the tissue is “pushed” abruptly towards its intended conformation.

This difference in pain is diluted thanks to the correct employment of analgesia, with which the patients seem to find the ordeal manageable. Patient controlled analgesia may be necessary in the postoperative period.

Later assessment of patient satisfaction with the surgical result and quality of life changes show that overall, patients are satisfied with the end result, especially in the Nuss procedure group. Patients also seem to manifest a lesser number of complaints of chest discomfort and pain than those who performed the Ravitch procedure. Overall satisfaction with the end result is very similar and overall can be qualified as good-excellent by approximately 90% of those patients who underwent either surgery.[27, 29]

Reoperation can be complicated in those patients who had Ravitch procedure done to them since the costochondral cartilage resection can lead to poor chest

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wall motion thus causing decreased chest compliance with corresponding diminished ventilator function. [28] It should be noted that another study found that the reoperation rate was higher in adults who underwent a Nuss procedure being older than 16 years old than those who had a Ravitch procedure performed, leading them to recommend the latter as the best choice in the adult with PE. [30] Other studies favor the Nuss procedure as the recommended approach when reoperating patients. [28] We find that because there is no apparent consensus on the adequate reoperation techniques, it is up to the clinician to determine, taking in to account the patient’s wishes and the underlying clinical conditions, which procedure to perform.

Another advantage found with either procedure was in the self-assessment made by the patients and their relatives after the procedure. A documented improvement in patient self-image, social self-consciousness and diminished emotional and physical difficulties can be obtained with the correction of the PE. Of note it should be said that in the study by Kelly et al, most patients opted for the Nuss procedure (283 out of 326). [13] This data suggests that essentially, it is the patient, or the parents that ultimately choose the Nuss procedure probably because of the perceived better aesthetic outcome.

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Conclusion

Currently there are two well documented and well established surgical approaches to PE. Both the Ravitch and the Nuss procedure have their advantages and disadvantages. If the decision is on the patient’s side, Nuss procedure is preferred probably because of the final aesthetic results without an anterior chest scar.

Other alternatives such as Vacuum bell appear to be limited in applicability whilst the magnetic mini-mover is just now showing its first results undergoing clinical trials.

Proposal for an experimental protocol

Pectus Excavatum is an interesting area of research. There is no well-established animal model. There is a mouse strain for Marfan disease that carries PE and the work of Haje et al which document a surgical animal model for PE. [31]

- Experimental design

Young Wistar rats will be weaned at 21 days of age. At the 30th day of life they will be anesthetized and subjected to a midline longitudinal incision in the sternum with division of pectoral muscles and subsequent exposition of the costal and sternal cartilages. The anterior portion of the sternal growth plate will be destroyed by electrocoagulation (Haje et al). The incision will be closed with sutures through the pectoral muscles and skin.

The control group will consist of animals without any surgical procedure.

At 90 days of age the surviving rats will be radiographically analyzed to compare the length and curvature of the sternum. It is expected that the Group submitted to surgical procedure will have acquired some form of Pectus deformity.

The animals with induced PE will be submitted to the placement of orthodontic thermolabile wires across 4 adjoining ribs and its contralateral counterparts. The

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fixation of these wires will resort to microimplants that will attach the metal arches to the osseous structures. One implant will be placed on the costal angle on the rib in its anterior surface and another one on the contralateral rib in the exact same place. The other two implants which sustain each arch will be fixated to the sternum. The arch for each set of contralateral ribs will thus be fixated by 4 implants. A Sham group will undergo the exact same surgical procedure but the arches will not be attached to the bone and will remain subcutaneously under the pectoral muscles.

Radiographic evaluation will be assessed at 120 and 150 days of age in both groups and the animals will be euthanized and target of assessment both by imaging and histologically at 180 days of age.

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Articles in journals:

1 Metzelder ML, Kubler J, Petersen C et al. Laparoscopic nephroureterectomy in children: a prospective study on Ligasure versus Clip/Ligation. Eur J Pediatr Surg 2006; 16: 241–244

2 Wedel T, Tafazzoli K, Sollner S et al. Mitochondrial myopathy (complex I deficiency) associated with chronic intestinal pseudo-obstruction. Eur J Pediatr Surg 2003; 13: 201–205

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Chapters from books have to be cited as follows: author(s), title of chapter, editor(s), title of book, edition, place of publication: publisher; year of publication; first and last page of the chapter.

Chapters in books:

3 Elger CE, Kurthen M. Paediatric Epilepsy Surgery. In: Panteliadis CP, Korinthenberg R. Paediatric Neurology. 1st ed. Stuttgart: Georg Thieme Verlag; 2005; 622–645

Articles in press (accepted for publication) at the time of submission of your manuscript have to be designated “in press” at the end of the reference. Abstracts should only be cited if published in a scientific journal and have to be marked with “abstr” followed by the page number. Manuscripts which have been submitted but not yet accepted, unpublished data or personal communications should not be mentioned in the references but indicated in the text.

Figures: The editors and publishers make every effort to publish your submitted figures in maximum quality. Therefore it would be helpful to abide by the guidelines which are listed below.

If you have any questions concerning these guidelines please do not hesitate to contact the production manager stated in the masthead.

each figure has to be uploaded separately (not embedded into the main document)

entitle the files precisely and number the figures consecutively but independent of the tables

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indicate in the main document where the figures should appear

file format and resolution of halftone images: *.eps, *.tif, *.jpg; minimal resolution: 300 dpi

file format and resolution of line drawings: *.eps, *.tif, *.jpg; minimal resolution: 800 dpi

save *.jpg files with the quality adjustment “high” or better “maximum” colour space: RGB or CMYK

colour depth: 8-bit image

If you use figures from books or other journals, be sure to mention the accurate reference in the legend and to obtain the permission for reproduction.

Before publication of any illustration in which the patient may be recognized, the author of the article must provide the publisher with the patient’s written consent to publication of the respective photograph. Appropriate standard letters for obtaining this consent are available on request from the publisher or can be downloaded from http://www.thieme.de/ejps under “Forms” in the left navigation bar. Please note that a black bar placed across the eyes may no longer be considered to be sufficient to preserve the patient’s anonymity.

Tables:

each table has to be uploaded separately

entitle the files precisely and number the tables consecutively but independent of the figures

indicate in the main document where the table should appear file format and resolution: *.doc or *.xls

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Please do not forget to enter the legends for the figures and tables in the appropriate text box while uploading the file AND to include a list of all legends on a separate page in the main document.

Videos: It is also possible to upload videos as supplemental file for review. The preferred video file format is *.mpg (MPEG-1). Alternatively, also *.avi, *.mov, *.rm and *.wmv will be accepted. Maximum length is 1 minute. Please do not forget to give a descriptive legend at the end of the main document. This legend will be published together with an internet link to your video at the end of your manuscript.

General Information

Attention: Liability

The new product liability legislation makes increased demands on the duty of care to be exercised by authors of scientific research and medical publications. This applies in particular to papers and publications containing therapeutic directions or instructions and doses or dosage schedules. We therefore request you to examine with particular care also in your own interest the factual correctness of the contents of the correction and galley proofs. You may make this easier for youself by arranging to have your statements counter-checked in accordance with the risks theoretically involved in the same by expert colleagues and co-workers.

During the technical production of books and journals checks are repeatedly made for print errors, with particular reference also to doses and dosage schedules. Failing expert knowledge on the part of proofreaders, however, all that can be done is to see whether the printed matter and manuscript agree.

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The responsibility for the correctness of data and statements made in the manuscript rests entirely with you as the author.

We should join hands in reducing the risk of right of recourse or claim to damages, and in this regard your responsible cooperation is fundamental.

Revision: The corresponding author obtains recommendations from the reviewers for revision of the manuscript. Please respond to these comments when submitting your revision by referring to page and line numbers and indicate all changes made in the manuscript. Your manuscript should be revised within 3–6 months to be represented to the reviewers.

Proofs: Proofs will be sent by the publisher to the corresponding author as a PDF file with the request that they be returned immediately after correction. Close examination by the author is necessary as the author is solely responsible for the correctness of the manuscript approved for print. The publisher reserves the right to charge for extensive changes in the proof. After imprimatur by the author no further changes are possible.

Reprints: 25 reprints of each article will be supplied free of charge to the corresponding author. These will be shipped after publication of the journal issue. An order form for further reprints for personal use (in increments of 50) will be sent with the proofs and should be signed and returned with the corrected proofs even if additional reprints are not required.

Copyright: All rights are held by the publishers, including the right to reproduce all or part of any publication.

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Referências

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