Chest Wall Malformations

Johns Hopkins National Proton Center opens

Chest Wall Malformations | Johns Hopkins Medicine

By News Release

Pediatric and adult cancer patients in the District of Columbia and elsewhere will now have access to one of the most advanced, lifesaving proton technologies offered in the U.S. at the newly opened Johns Hopkins National Proton Center at Sibley Memorial Hospital in collaboration with Children’s National Hospital

The new Johns Hopkins National Proton Center at Sibley Memorial Hospital is part of the Johns Hopkins Sidney Kimmel Comprehensive Cancer Center in Baltimore, Maryland. U.S.

News & World Report recently ranked the Kimmel Cancer Center 4th in the nation in its annual ranking of best hospitals. Through its collaboration with Children’s National Hospital — rated by U.S.

News & World Report as one of the top ten nationally for pediatric cancer care – Sibley houses the only proton center in the Greater Washington, D.C. region with a dedicated pediatric team. 

Adults and children are now being seen at the 80,000+ square foot proton center, one of the largest and most advanced such facilities in the nation.

The proton collaboration with Children’s National Hospital represents an expansion of the earlier collaboration between Children’s and Johns Hopkins Medicine that established the pediatric radiation oncology program at Sibley, which treats a broad range of children’s cancers.

This project is yet another example of how John Hopkins is moving boldly into the future to ensure that our patients receive the best care possible,” said Paul Rothman, dean and CEO, Johns Hopkins Medicine. “With this center, we can now provide another cutting-edge option in cancer care, an option that will make a real difference in the lives of many patients.” said Paul Rothman, Dean and CEO, Johns Hopkins Medicine.

According to Kevin Sowers, President of the Johns Hopkins Health System and Executive Vice President, Johns Hopkins Medicine, “Our program has leading experts that understand the entire patient and their cancer, not just proton therapy, allowing them to help patients receive the best care possible for their particular needs.”

“This collaboration between the Johns Hopkins Sidney Kimmel Comprehensive Cancer Center, Sibley Memorial Hospital and Children’s National Hospital will transform cancer care throughout D.C., this region and beyond,” said Hasan Zia, MD., interim president and CEO of Sibley Memorial Hospital.

“We’re doing more than just providing the most advanced proton therapy available.

We will be conducting groundbreaking research that will potentially help expand this technology for use in treating other types of cancers while at the same time helping improve the effectiveness of the proton treatments for the cancers currently most amenable to proton therapy.”

The new proton center offers state-of-the-art pencil beam proton therapy equipment, as well as next-generation imaging technologies such as dual energy CT-guided treatment that reduces the range of error, and the latest innovation in biomatrix MRI designed to target moving tumors in places the lung and liver. A large mechanical arm called a gantry can move the beam 360 degrees around the patient, treating the tumor from several angles as it destroys tumor cells layer by layer.

“The opening of the proton therapy center will offer the most advanced cancer treatment to help kids lead better lives,” says Dr. Kurt Newman, president and CEO of Children’s National. “Not only does this allow children in our region to receive proton therapy closer to home, but the center is one of a few nationally that will have a dedicated pediatric team.”

“Proton therapy is an advanced technology that allows radiation to be delivered precisely to cancer tissue,” says Jeffrey Dome, M.D., Ph.D., vice president for Cancer and Blood Disorders at Children’s National Hospital.

 “This provides a significant advantage compared to conventional radiation therapy, especially in children, where sparing the healthy tissue that surrounds the tumor may be critical for normal growth and development.

Proton therapy shows great promise to reduce long-term side effects of radiation treatment.”

The Johns Hopkins National Proton Center at Sibley will also have a fully integrated research room, which will allow clinical, basic science, and medical physics faculty to advance clinical trial research, translational research, and technology development research in proton therapy.

There, experts will lead efforts to study proton outcomes for sarcoma, gynecological tumors, pancreatic and liver tumors, lymph node cancers and tumors located near the heart and major blood vessels such as lung or breast cancers. In addition, the researchers will examine how the cancer cell-killing proton energy interacts with the cells and tissue surrounding the tumors.

New advances in proton therapy will thus be developed and quickly translated into clinical practice.

“Our center is one of only a few academic programs in the country with a team conducting biologic research to improve proton therapy in addition to clinical trials comparing proton and more traditional photon therapy,” says William Nelson, MD.

Director of the Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins. “This research is critical to determining which type of treatment is best for each individual situation.

Through this work, our researchers will provide the knowledge necessary to make cancer treatment more personal, precise and effective.”

 “Proton therapy is advanced radiation treatment that is directed precisely at cancerous tissue in adults and children,” says Akila Viswanathan, MD., interim director of the Department of Radiation Oncology and Molecular Radiation Sciences for Johns Hopkins Medicine.

“This is a significant improvement over conventional radiation treatment for many cancers, especially in children, which has a greater risk of damaging the healthy tissue surrounding the tumor.

Conventional therapy carries the risk of causing health issues that manifest themselves long after treatment, such as cognitive or development impairments or heart disorders.”

“Children with cancer will have access to the highest level of care from specialists dedicated to caring for children,” says Matthew Ladra, M.D.

, Director of Pediatric Radiation Oncology at the Johns Hopkins Kimmel Cancer Center at Sibley Memorial Hospital “This is far more than an investment in expensive pieces of machinery. This is a collaboration that establishes a unique care continuum centered on pediatric oncology patients and their families.

It brings together national and international leaders in academic research and clinical medicine to collaborate on the goal of advancing pediatric oncology treatment.”

Dr. Christina Tsien has been appointed proton center medical director and Dr. Curtiland Deville will serve as the associate proton director, while maintaining his role as the clinical director for the Radiation Oncology Clinic at Sibley Memorial Hospital.

In addition, through a strategic partnership with Howard University, the proton center will serve as an educational and training site for students enrolled in Howard’s medical physics program.  

The Johns Hopkins National Proton Center is opening in phases. The first treatment room opens in October 2019. The second room is scheduled to open in spring 2020, and the third room and fixed beam research room are scheduled to open in fall 2020.

Photo credit: The Sidney Kimmel Comprehensive Cancer

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Source: https://appliedradiationoncology.com/articles/johns-hopkins-national-proton-center-opens

Fetal Balloon Treatment for Lung-Damaging Birth Defect Works Best When Fetal and Maternal Care Are Highly Coordinated

Chest Wall Malformations | Johns Hopkins Medicine

Researchers from The Johns Hopkins Center for Fetal Therapy report new evidence that fetuses with severe congenital diaphragmatic hernia (CDH), a rare but life-threatening, lung-damaging condition, experience a significantly high rate of success for the fetal treatment known as FETO, if they and their mothers receive coordinated and highly experienced care in the same expert setting.

A report on the findings was published online, on Feb. 6, in the journal Obstetrics & Gynecology.

FETO — fetoscopic tracheal balloon occlusion — is a minimally invasive procedure in which a fetoscope is inserted through the abdominal wall into the uterus and then into the mouth of the fetus to place an inflatable balloon, to temporarily block the fetal trachea. The blockage allows lung fluids to build up behind the balloon, encouraging expansion of the airways and lung growth. The procedure improves the odds that fetuses with severe CDH acquire sufficient lung function after birth to lead a normal life.

“The primary cause of death in babies with CDH is that the lungs do not develop properly, and they cannot breathe outside the womb,” says Ahmet Baschat, M.D., director of The Johns Hopkins Center for Fetal Therapy and professor of gynecology and obstetrics at the Johns Hopkins University School of Medicine.

Usually detected through a routine prenatal ultrasound, CDH is a rare condition that impairs lung development, affecting one in 3,000 live births. It is characterized by the partial or complete absence of the diaphragm — the muscle that separates the chest from the abdomen — resulting in a hole.

The gap may permit organs that are usually in the abdomen, such as the bowel, the stomach and the liver, to push into the chest. This causes a hernia or bulge, a displacement that leaves too little space for fetal lungs to develop normally. The degree of lung damage is greatest for large hernias, where the liver herniates into the chest.

After birth, surgical closure of the hole is possible, but the lung damage that has occurred before birth can make this condition fatal.

To assess the feasibility and impact on the health of mothers and babies after FETO therapy, Johns Hopkins researchers enrolled 14 women, all patients at The Johns Hopkins Center for Fetal Therapy, between May 2015 and June 2019. The women were an average of 28 weeks pregnant and an average of 33 years old.

For the study, Baschat and his team performed successful FETO balloon blockages on all 14 fetuses between 26 and 29 weeks of gestation. No procedure-related fetal or maternal complications occurred. The team removed the balloons at about 33 weeks of gestation, after a median 34 days of blockage.

The researchers say that FETO therapy produced favorable outcomes in the 14 infants born to the mothers in the study when performed in a single center setting, where prenatal and postnatal monitoring and care were highly coordinated.

“This is most ly due to prenatal management by a team with experience in fetal interventions, as well as maternal-fetal care in one single institution,” says Baschat.

“From the time of balloon insertion, we had a multidisciplinary team of fetal therapists, neonatologists, pediatric surgeons, pediatric ENTs and obstetric and pediatric anesthesiologists available for any emergency balloon removals and to make sure the fetuses’ airways weren’t obstructed in case of unplanned birth.”

“FETO has been studied in the past, in the United States and abroad, in a randomized trial, a large feasibility study and several smaller studies, and while the overall approach was comparable to our study, we employed a deliberate strategy to minimize potential contributors to preterm birth associated with premature rupture of membranes,” adds Baschat. Some of these strategies included treating the mothers with vaginal progesterone, avoiding the lower part of the uterus when inserting the fetoscope and aggressive treatment of preterm contractions.

A striking difference in the Johns Hopkins study, Baschat notes, is that delivery of the babies was at an average of 37 weeks of gestation, with no deliveries prior to 32 weeks; 7% of deliveries before 34 weeks and 43% prior to 37 weeks of gestation. This allowed all the infants to be candidates for extracorporeal membrane oxygenation (ECMO) — an advanced life-support technique — which may have been an important contributor to the survival of the infants.

Overall, babies were born about 30 days after balloon removal. All cases experienced a sustained increase in lung size, from 23.2% before blockage to 46.6% prior to birth.

All 14 women delivered at The Johns Hopkins Hospital at a median gestational age of 39 weeks (range 33-39). Eight (57%) delivered at term (≥37 weeks of gestation), after scheduled, induced labor.

The majority of mothers (71%) delivered their baby vaginally.

“We’ve been able to achieve a really good safety protocol — not only did the procedure result in lung expansion, but balloon removals were all scheduled; they were not emergency procedures,” says Baschat.

Infant survival on day 28 was 93%, and the overall survival to 6 months or hospital discharge was 86%.

All of the babies had absence of the diaphragm on the side of the hernia and required surgical repair of the CDH using a patch, which was performed within the first week of life. The primary complication after surgery in three of the 14 babies (36%) was recurrence of diaphragmatic herniation, due to areas of the patch detaching from the chest wall as the infants grew in size.

“The study had the highest survival rate ever reported for these types of patients, with the lowest complication rate of FETO in terms of procedure risks, obstetric risks and fetal risks,” says Baschat.

A randomized trial involving U.S. and European fetal therapy centers is underway; however, the researchers say they want to wait to see those results to decide on next steps. “Standardized prenatal and postnatal care appear to be complementary in achieving survival in these infants,” says Baschat.

“Anticipating possible obstetric complications and reactively providing prompt treatment may improve the chance for mothers to deliver at term.

” Meanwhile, the researchers are collaborating with other fetal therapy specialists to investigate how care-setting factors and management strategies can be optimized to apply them across other fetal therapy centers.

Additional authors of the study from Johns Hopkins Medicine include Mara Rosner, M.D.; Sarah E. Millard, R.D.M.S.; Jamie D. Murphy, M.D.; Karin J. Blakemore, M.D.; Amaris M. Keiser, M.D.; Jennifer Kearney, R.N.C.; Janine Bullard, M.D.; Lawrence M. Nogee, M.D.; Melania Bembea, M.D., Ph.D.; Eric B. Jelin, M.D.; and Jena L. Miller, M.D.

Disclosure: Ahmet Baschat received funding from the National Institute for Child Health and Human Development (U10). Amaris Keiser received funding from Guidepoint.

Melania Bembea received funding from the National Institute of Neurological Disorders and Stroke (R01) and from the National Institute of Child Health and Human Development (R21).

Jena Miller received funding from the Fetal Health Foundation and the National Institute for Child Health and Human Development (RO1). The other authors did not report any potential conflicts of interest.

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Source: https://ictr.johnshopkins.edu/news_announce/fetal-balloon-treatment-for-lung-damaging-birth-defect-works-best-when-fetal-and-maternal-care-are-highly-coordinated/

Chest Wall Malformations

Chest Wall Malformations | Johns Hopkins Medicine

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Pectus excavatum (PE), translated literally as “hollowed chest” and also referred to as “sunken chest” or “funnel chest,” is the most common chest wall deformity seen in children.

An overgrowth of the rib cartilages before and after birth causes the characteristic depression of the sternum (breastbone). The cause of PE is not known, but often the tendency to develop PE runs in families.

Depending on the seriousness of the defect, PE may cause poor posture with slumped shoulders and a protruding abdomen or “pot belly,” as well as possible problems with bone growth and alignment later in life.

In severe cases, PE shifts the heart to the left side of the chest and compresses the lungs, limiting the child's ability to take deep breaths. This defect commonly worsens during puberty until age 18 when most of the growth spurt is complete. 

Children with PE usually have no symptoms, but the defect becomes more pronounced with the growth of the chest during puberty. Some teenagers with PE complain of shortness of breath with exertion and pain at the front of the chest and may say that they tire easily.

Diagnosis

The defect is measured using thoracic depth measurements (TDM) to compare the right to left side of the defect using a line created from the nipple to the vertebral column. Differences of less than 1 cm are considered a mild deformity. TDM defects between 1 cm and 2.5 cm are moderate, and a greater than 2.

5 cm defect is considered a severe deformity. Surgical repair is recommended in children with defects that are moderate to severe. Other methods of measurement employ CT scanning to calculate the Haller index — this number compares the depth of the chest cavity beneath the sternum to the width of the chest cavity (from right to left).

The normal ratio of width-to-depth is about 2.5 to 1. 

Treatment

Safe repair of PE is best performed in children over five years of age. The preferred age for repair is at about 14 years of age.

The operation is easier and the recovery is shorter in this age group because in most cases, the majority of the pubertal growth spurt has passed, but the rib bones are still incompletely formed (ossified).

This allows the chest wall to reform into a more normal shape as the child grows after the repair. Older adolescents and adults also report good results with repair.

The operation to repair PE is called a Nuss procedure and involves the placement of one or more stabilizing metal bars just inside the ribcage to move the sternum forward. The bars are shaped to the patient during the operation and remain in place for several years to allow the ribs to adjust to the new shape of the chest. The bars are then removed in a separate operation. 

Recovery

Improvement in the chest wall varies and depends on the severity of the defect. A common problem from this procedure is a pneumothorax (air within the chest cavity but outside the lungs). Your child will have an X-ray the morning after surgery to identify any problems.

Johns Hopkins Children’s Center pediatric surgeon-in-chief David Hackam answers questions about pectus excavatum. He provides information on the surgical procedure to repair pectus excavatum at Johns Hopkins Children’s Center, the appropriate age for surgery and the recovery time.

Source: https://www.hopkinsmedicine.org/health/conditions-and-diseases/chest-wall-malformations

Is Sunken Chest More than a Cosmetic Problem?

Chest Wall Malformations | Johns Hopkins Medicine

Hollow or sunken chest, the most common congenital deformity of the chest wall affecting one in 300 to one in 400 children, is rarely life-threatening and virtually all children can have successful surgical repairs. But the condition is far from purely cosmetic and even the mildest cases require prompt evaluation upon diagnosis, say experts at the Johns Hopkins Children’s Center.

“Surgery for pectus excavatum is rarely done solely for cosmetic reasons. The main reason we do surgery is to improve heart and lung function, not looks, and any cosmetic benefits are secondary,” says Hopkins Children’s pediatric surgeon Fizan Abdullah, M.D. Ph.D.

Abdullah and his colleagues advise early check-ups that would achieve three goals: Rule out serious underlying syndromes, Assess cardio-pulmonary function and Plan surgery or RAP.

  • Rule out more serious disorders. In a small subset of children, sunken chest can herald an underlying syndrome. For example, sunken chest is commonly found among children with Marfan syndrome, a genetic disorder of the connective tissues whose most feared life-threatening complications include growth of arterial aneurysms or stretching and rupture of the heart’s aorta. Experts recommend that pediatricians evaluate all children with sunken chest for other tell-tale Marfan signs, including long slender fingers, arm span exceeding height, long lean skull with downward slanted eyes and a spinal curvature (scoliosis), among others. Any child with three or more of these features should be referred to a Marfan expert, especially if the child has a family history of Marfan or unexplained heart problems.
  • Assess heart and lung function. The sunken chest, especially in more severe cases, can compress the heart and lungs and affect breathing and circulation. Although serious lung and heart problems are rare, even children with milder cases often have reduced cardiovascular endurance, tire quickly, describe a feeling of something sitting on their chest and complain of neck and back pain.
  • Plan the optimal time for surgery. Surgery can alleviate pulmonary and cardiac problems, reduce back and neck pain, improve posture and restore normal appearance to the chest. Minimally invasive alternatives to open-chest surgery have been developed in recent years. Depending on the patient’s age, heart/lung involvement and severity of the malformation, surgeons can choose from several approaches.

The ideal window for surgery is between ages 14 and 16, Abdullah says. If surgery is done too early, the sunken chest may re-emerge because the bones are still growing and taking their final shape.

However, Abdullah says, early surgery is warranted in severe cases where lung and heart function are seriously compromised.

Surgeries in older children and adults tend to be more difficult and more invasive because once the bones start to calcify they may require an open-chest surgery.

“This golden window is not a hard-and-fast rule,” Abdullah says, “but a preferred timeframe, and we can certainly do the surgery in older patients and — more rarely — in younger children with very severe symptoms.”

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Source: https://www.hopkinsmedicine.org/news/articles/is-sunken-chest-more-than-a-cosmetic-problem