WEB EXCLUSIVE: View Jeune's Syndrome clinical images and surgical video
|Non-cardiac thoracic diseases most often treated by the Nationwide Children’s Hospital cardiothoracic team, generally fall into the three categories listed below. However, the team also diagnoses and provides surgical intervention to those thoracic cases which are anomalies, such as Chronic Effusion resulting from prior congenital heart surgical interventions. (See case study.) Details regarding the surgical interventions for the non-cardiac thoracic diseases are provided in the patient case studies in this article, as well as online.
Most of the patients require a multi-disciplinary approach led by the cardiothoracic surgical team. The cardiothoracic surgical team also is part of The Heart Center, which is nationally recognized for the treatment of congenital heart disease. Surgical approaches used at Nationwide Children’s are either minimally invasive, using video assisted thorascopy, or open surgical approaches. The approach that is selected depends on the patient and the disease or deformity that has been diagnosed.
Treatment of Major Thoracic Disorders
Chest Wall Deformities
Jeune’s Syndrome - is a rare syndrome, and a form of congenital dwarfism which results in a very narrow thoracic cage that slowly leads to asphyxiation for the patient. Other associated problems with this syndrome are lung hypoplasia, bony abnormalities, nephritis, and congenital heart disorders. Patients frequently get pulmonary infections and benefit from tracheotomy.
The surgical intervention for patients with Jeune’s Syndrome and other asphyxiating disorders was pioneered by Terrance Davis, MD and Robert Castile, MD at Nationwide Children’s Hospital. An extensive team of specialists are available to help treat all patients with thoracic dystrophies, including Jeune’s Syndrome. This treatment team will address individual patient needs. Cardiothoracic patients receive additional follow-up care in the Cardiothoracic Intensive Care Unit. Patients are referred from all over the world to Nationwide Children’s for treatment of Jeune’s Syndrome. To meet the needs of this growing thoracic dystrophy population, Nationwide Children’s now offers a multi-disciplinary Thoracic Dystrophy Clinic. Led by Drs. Melissa Holtzlander and Alistair Phillips, the clinic serves both current thoracic dystrophy patients and new referrals. In addition to Pulmonary Medicine and Cardiothoracic Surgery physicians, patients will also have the opportunity to see physicians from Clinical Genetics and Plastic Surgery. Nutritionists and social workers are available to assist and manage the many complex needs of patients with thoracic dystrophies. Diagnostic testing will be scheduled for the day of the clinic, if needed. The clinic will be held two to three times per year, as determined by need.
Other Chest wall deformities include pectus carinatum, the outward protrusion of the sternum or thoracic cage, and pectus excavatum, the inward depression of the sternum, and it is the most common deformity that occurs in approximately one out of 1,000 children. It is characterized by a concavity of the sternum. The disorder is usually noted at birth as a mild condition but can become progressively more pronounced over time. As children grow older, additional symptoms can include:
There are two surgical approaches to pectus repair to answer individual patient needs:
Pectus defects can be repaired via the modified Ravitch procedure which involves a midline lower sternal incision and removal of usually the lower four costal cartilages leaving the perichondrium foreshorting costal cartilage. The sternum is then elevated and stabilized with wires.
The Nuss Procedure
The Nuss procedure is another approach for pectus excavatum repair which requires placement of a custom fitted titanium bar inserted laterally and positioned behind the sternum to push the sternum outward. The bar is removed after three years and the defect has remodeled into a more symmetrically positioned sternum. The appropriate timing of surgery is typically in the early adolescent years, but patients may undergo evaluation for this condition at any age.
In the case of a pectus deformity, it is important to note most insurance companies do not cover this repair as it is felt to be a cosmetic issue. Often insurance companies will consider the Haller Index and any associated symptoms such as decreased pulmonary function, when reviewing the case for approval. The typical length of stay for pectus repair via the Ravitch procedure is approximately four days. Repair for those under age 10 is uncommon as these defects can worsen with growth spurts. .
Benign Thoracic Disorders
Spontaneous Pneumothorax: For the first episode, most will resolve with conservative drainage with a chest tube. For recurrent episodes and patients with blebs (air pockets within the lung usually at the apex of the lung), surgical resection is recommended. General approach is via video assisted thorascopy (VAT) with two incisions each approximately 1 centimeter (one camera port and one working port). A pleurodesis is performed with doxycyline which is injected into the pleural space to promote adhesions between the pleura and the lung; along with an apical pleurectomy where the lining of the chest wall is removed to improve adhesion between the lung and the chest wall.
For CF patients who experience spontaneous pneumothorax, conservative treatment with a chest tube is the preferred management strategy as scarring from a blebectomy and pleurodesis may be a contraindication to transplant in some centers. However, at Nationwide Children’s a patient who has undergone a pleurodesis can still be a candidate for lung transplant.
For patients diagnosed with bronchiectasis, a CT of the chest evaluation is performed and if surgical intervention is warranted, this can be performed via a VATS approach. For a large affected area however, a segmentectomy is usually needed which requires an open approach.
Mature and Immature Teratomas
A diagnosis for these patients is usually made based on the presentation of a mass on x-ray. A majority of the masses are benign but local recurrence can occur if margins are positive.
Vascular rings are typically formed by a double aortic arch or by an aberrant subclavian artery. Patients may have symptoms of difficulty swallowing, stridor or recurrent lung infections. These abnormalities are often noted as indentations on the trachea or esophagus if an upper gastrointestinal study has been performed. Evaluation by either CT scan or MRI with contrast confirms the abnormality. Surgical resection is usually via a left thoracotomy with division of an atretic left arch and ligementous arteriosus, which is the most common approach.
Lung biopsies are performed to obtain quality tissue samples when a definitive diagnosis is inconclusive or when other testing modalities have failed to produce an adequate pathological specimen. Lung biopsies are performed via either an open approach or using the Viking 3Di Vision Systems which gives excellent clarity. Indications for lung biopsy include persistent interstitial lung disease (without definitive diagnosis), mediastinal lymphadenopathy, presence of pulmonary nodules, and to determine rejection classification in lung transplantation. These procedures can be helpful in diagnosing causes of infection like histoplasmosis which is pandemic in the Ohio River Valley.
Congenital Pulmonary Deformities
Included in this category are the diagnosis of: congenital lobar emphysema, congenital pulmonary cysts, bronchogenic cysts, and pulmonary sequestration. Surgical approach depends upon the actual malformation, size, and location. CT scans and pulmonary function tests are beneficial in determining timing of surgical intervention. Typically, these defects require an open approach, however, with the use of the Viking 3Di Vision Systems more surgeries can be done minimally invasive.
Primary and Secondary Malignant Pulmonary/Thoracic Cavity Disease
Patients who fall in this category often present with the following:
Traditionally, the patients outlined in this article may have fallen through the gaps between adult thoracic surgery, general pediatric surgery and cardiovascular surgery. However, now these patients who have non-cardiac thoracic disease can be diagnosed and treated by an integrated team consisting of hematology/ oncology, pulmonary disease, physical therapy, radiology, plastic surgery, in addition to cardiothoracic surgery.
Case Study: Chronic Effusion
Kylie, 13-years-old, had repair for congenital heart disease at Nationwide Children’s Hospital when she was a baby. Her family had moved to Atlanta, Georgia and she was doing well until a year ago, when it was decided that she needed her pacemaker repaired, which had not been in operation since she was 7-years-old. At the time of receiving her pacemaker repair she was also found to have pulmonary hypertension. Although she had surgery for the pacemaker, it was complicated by persistent pleural effusions. As a result she underwent three additional surgeries to address the effusions in her chest. She was in and out of the hospital in Georgia from May 13, 2008 until she left for Ohio, September 29, 2008 when she was admitted to Nationwide Children’s for a potential heart/lung transplant.
When Kylie arrived at Nationwide Children’s ICU on September 29, she was critically ill, in bed on oxygen. The cardiothoracic team evaluated her and decided that it would be possible to improve the function of her right lung. She was taken to the OR for thoracic surgery on October 2 to free up her right lung, by removing extensive scar tissue entrapping the right lung (as a result of her previous surgeries). Kylie recovered well from the seven hour surgery.
Pre-operative images, Figures 1-3 show compressed lung with fluid collection and heart shifted into left chest
|Figure 1: PA chest X-ray||Figure 2: Axillary CT scan||Figure 3: Coronal CT scan|
Kylie’s underlying problem was chronic effusion. The result was she did not need a heart/lung transplant. In fact her problem was a non-cardiac thoracic problem, which the cardiothoracic team was able to treat. The team included experts in pulmonary hypertension, led by Curt Daniels, MD, director of the Adolescent and Adult Congenital Heart Disease Program and the Pulmonary Hypertension Program. After surgery she was in the cardiothoracic ICU until October 8, and discharged from the hospital October 22, 2008. However, even after being discharged from the hospital she stayed close by for four weeks of pulmonary rehabilitation before returning home to Atlanta, Georgia. The rehabilitation helped her improve her overall lung function. Kylie is back at school and no longer requires supplemental oxygen.
|Post-operative images, Figures 1-3 show re-expanded lung and heart shifted back to normal orientation|
|Figure 1: PA chest X-ray||Figure 2: Axillary CT scan||Figure 3: Coronal CT scan|
Case Study: Jeune’s Syndrome
Jeune’s Syndrome Clinical Images Pre- and Post-Surgery
|Michael “Blake” Ford was born on December 23, 2002. Shortly after his birth Blake was diagnosed as having Jeune’s syndrome, also known as asphyxiating thoracic dystrophy. Since his chest wall and lungs were too small to support his needs without assistance, he required a tracheostomy and ventilator support. In March of 2004, he traveled to Columbus, Ohio, to undergo the lateral thoracic expansion surgery pioneered at Nationwide Children’s by Dr. Terrance Davis for patients with Jeune’s syndrome.
Blake had his first side expanded in March of 2004. Several months later, in August of the same year, he underwent expansion on the second side. Because of the life-saving surgeries, Blake’s family decided to make Ohio their permanent home, so they could be near the Jeune’s team that was caring for Blake.
In 2008, Blake was able to breathe completely on his own, without ventilator assistance. In June of that year, he was able to have his tracheostomy removed. Today, he is thriving and able to do much more than his parents anticipated prior to their travel from Oklahoma to Ohio five years ago. Blake is an active 6 ½ year old boy, who enjoys playing outdoors, swimming and jumping on his trampoline.
Case Study: Pleural Pulmonary Blastoma
WEB EXCLUSIVE: View a video slideshow of this surgical procedure.
|McKenna, an active 4-year-old was originally treated by her primary care physician in June of 2006 in Delphos, Ohio for a fever of 102°, accompanied by shallow breathing, loss of appetite and swollen lymph nodes. He prescribed Cefaclor, which improved her condition for two to three weeks. On July 4 of 2006 McKenna was taken to the Emergency Department at St. Rita’s Medical Center in Lima, Ohio for stomach pain, left shoulder pain, a persistent, nonproductive cough, vomiting, diarrhea, sore throat, ear ache and a temperature of 101°. McKenna’s family requested that she be transferred to Nationwide Children’s Hospital.
Once transferred to Nationwide Children’s, a series of tests were performed on McKenna to determine her diagnosis. On exam, McKenna had significant increased respiratory effort. She had good air movement in her right lung but had no breath sounds from the left lung. McKenna was dependent on lying on her left side to breathe. Her initial CXR showed that her entire left hemi-thorax was replaced with tumor and her heart was pushed into her right side of her chest causing her right lung to be compressed. She underwent a CT scan, (Figure 1), two biopsies by interventional radiology, and a PET scan, which confirmed the large tumor in the left chest was compressing all of her mediastinal organs. The last biopsy on July 12, 2006 confirmed left pleural pulmonary blastoma (PPB). According to Dr. Phillips the chest tumor which was pushing the heart into the right chest, with compression of the right lung, made resection impossible. Based on diagnosis the recommendation, by Dr. Phillips and Mark A. Ranalli, MD, Hematology/Oncology, was made to provide chemotherapy to shrink the tumor to a point where it could be surgically resected.
Dr. Ranalli began administering chemotherapy treatment to McKenna to slow or potentially decrease the mass. Chemotherapy treatment was started based on the treatment protocol from the PPB registry. McKenna was followed with frequent CXRs, CT scans (Figures 2A and B, Figure 3) and PET scans.
She was given five cycles of chemotherapy with a remarkable response. The solid portion of the tumor went away and her medistinum moved back into the midline. From the fourth to fifth cycle there was only slight improvement. At this point it was decided that the mass of tumor was resectable, and this was her best window of opportunity to get a complete resection. She did not receive any radiation therapy.
The pathology revealed that the entire tumor was removed and all margins were negative except one microscopic area which showed tumor going into the margin. She received postoperative chemotherapy and radiation therapy which she has now completed.
McKenna’s surgeries and treatments are proving successful and she has resumed a normal life for a 7-year-old girl, playing with her friends and going to school. She will continue follow-up therapy for the rest of her life. Her prognosis is excellent.
Alistair Phillips, MD, is a cardiothoracic surgeon at The Heart Center at Nationwide Children’s Hospital, and an Assistant Professor of Surgery at The Ohio State University College of Medicine. He is board certified in both surgery and thoracic surgery. Dr. Phillips received his undergraduate degree in biomedical engineering from The Johns Hopkins University and his medical degree from The College of Physicians & Surgeons of Columbia University. He completed fellowships in surgical, cardiothoracic, and pediatric cardiac surgery at Cornell Medical Center and Columbia-New York Presbyterian Medical.
Clinical areas of focus include congenital thoracic disorders, congenital heart disease in children and adults, heart and lung transplantation, electrophysiology and research. Additionally, Dr. Phillips is actively involved in developing new techniques and equipment for use in the hybrid approach to pediatric cardiothoracic diseases.