“One of the things we are most proud of is our parent-driven approach to our care and research,” said Carlos Medina, MD, Clinical Director of Urology. “We are the only children’s hospital in the state to offer video visits to our patient families. We also have a team of innovators who are finding engaging ways educate our parents on a broad range of urological health issues. The recognition is certainly an honor, but we don’t do it for the accolades. Our focus and priority are the children of Connecticut and region.”

U.S. News & World Report collects data from nearly 200 children’s hospitals nationwide, taking into consideration clinical outcomes, hospital resources, and delivery of healthcare. This in combination with survey results from pediatric specialists across the country is used to identify the top 50 programs for 10 different specialties.

In addition to its urology ranking, Connecticut Children’s was also ranked in the Top 50 programs nationally by U.S. News & World Report in Gastroenterology & GI Surgery, Diabetes and Endocrinology and Neonatal Care.

“The recognition from U.S. News & World Report is a testament to our dedicated physicians, nurses and team members who have continued delivering care during the COVID-19 pandemic while continuing to advance research and clinical outcomes across the health system,” said Jim Shmerling, DHA, FACHE, President & CEO at Connecticut Children’s. “We have made it easier than ever to schedule an appointment with our experts thanks to our comprehensive virtual health program, which includes video visits. We will stop at nothing to keep all children safe and sound as we welcome everyone back for their appointments, surgeries and procedures.”

Technology is an integral part of everyday life, and its use in the provision of healthcare is increasing. Many of the restrictions that delayed or prevented the utilization of telemedicine have been removed during the COVID-19 pandemic to facilitate care during quarantine and social distancing.

Keys to successful implementation are the central tenets of workflow design, team preparation and partnering with patients. Reliable technology infrastructure provides a high-quality audiovisual experience and minimizes gaps in communication during the visit. The training of clinicians and staff in the use of the platform is imperative.

Equally important is working with the patient in advance to ensure their ability to use the platform. Just as one would give directions to a physical office, directions should be provided on how to navigate to and through telehealth. Decisions made in advance regarding which patients and what kind of care are most suitable for telemedicine encourage a safe and efficient process. Lastly, proper documentation of consent is standard of care for all telehealth visits.

Although many patients are experiencing telehealth for the first time due to the pandemic, it is clear that the associated safety and convenience will lead to it being a fixture of healthcare going forward.

All patients undergoing non-emergency surgeries will be tested for COVID-19 prior to their procedure. In addition, there will be a limit on the number of surgeries to allow adequate time between cases for air-turnover and dissipation of aerosolized particles. Visitation rules have been modified to limit the number of individuals in the facility. For inpatients, only one parent or legal guardian is permitted per day, while for outpatients, including the Emergency Department, only one parent or legal guardian is allowed to accompany a child. Parents/guardians must be free of fever and respiratory symptoms. No other visitors, including siblings, are allowed.

In addition, we split our Emergency Department into two different areas: one for patients who have COVID-19 symptoms or known exposure, and one for those who don’t. This system allows us to deliver great care to every child while protecting everyone against the spread of COVID-19. We are also using a “rapid triage” system to put you and your child in your own room right away, rather than having you wait in a common space. Once again, patients who may be at risk of COVID-19 are cared for in a dedicated part of the hospital, including negative-pressure rooms when appropriate.

On the ambulatory side, our clinical operations team implemented an electronic check-in system for patients with in-person appointments, in order to eliminate the need for actual paperwork. Additionally, the team developed a virtual waiting room. This will allow staff to directly room patients from their cars rather than the waiting room, minimizing exposure risk. Lastly, strict cleaning processes are in place to ensure frequent attention to all high touch spots, such as door handles and elevator buttons.

Please be assured that Connecticut Children’s is taking extraordinary steps to keep all your patients and families safe and sound. Learn more about our safety program> 

Made possible by a $1.5 million donation by the Robert R. Rosenheim Foundation, the new center, located on the 4th floor of Connecticut Children’s Hartford campus at 282 Washington Street, includes a variety of features to enhance quality and comfort safety for all patients receiving dialysis care. It’s a facility designed with a child’s unique needs in mind.

“We have worked hard to create a spa-like environment,” said Cynthia Silva, MD, Division Head of Nephrology. “The interactive light wall and mountain imagery is an authentic way to pull the patient’s attention away from dialysis. Our hope is that children will focus on the excitement and joy brought about by our unique environment and let go of any anxiety.”

The new facility includes four private treatment rooms, each equipped with built-in television and seating areas for families as well as color changing lighting, allowing each patient to control his or her own environment. Additional features include light walls, waiting room workstations and a media center. Connecticut Children’s is also among the first to use the Tablo hemodialysis machine for pediatric-specific care.

“We are so grateful to the Robert R. Rosenheim Foundation for their generosity and support in opening the state’s very first outpatient pediatric dialysis center” said Jim Shmerling, President & CEO of Connecticut Children’s. “This new facility will afford families the opportunity to stay close to home for high-quality, life-saving treatments during such a critical time for public health.”

Niyear’s Dialysis Journey

Niyear Perez no longer needs dialysis to live, but he knows what a new pediatric outpatient dialysis center means for kids who do. The 16-year-old Waterbury resident had experienced firsthand the challenges of traveling back and forth to adult dialysis centers before undergoing a kidney transplant on June 5, 2017.

For Niyear, who is in his sophomore year this fall at Wilby High School, the need for dialysis was as unexpected as it was frightening. It all started with severe headaches in 2015 when he was in the sixth grade.

“He kept complaining of bad headaches, and then his blood pressure went through the roof,” Virginia explained. He was referred from their local hospital to Connecticut Children’s, where Dr. Silva diagnosed him with chronic renal failure. “Dr. Silva diagnosed him and immediately took action. She made a decision to remove both his kidneys and that ultimately saved his life.”

Niyear remained in the intensive care unit at Connecticut Children’s for several months and began dialysis in 2016.

“Niyear’s case was difficult because he and his family didn’t realize how little kidney function he had because of his disease,” Dr. Silva explained. “He was very ill when we met him, so he went on dialysis rather quickly for a young teenager. It was hard for him and his family, so having him dialyzed at an adult unit made it even more difficult.”

Virginia, who is Niyear’s guardian, said they first drove to an adult dialysis center in South Windsor, where he underwent dialysis three times a week, for 3-4 hours at a time. He was then transferred to an adult facility in North Haven, where he also went three times a week, 3-4 hours at a time. “It was a rough experience for him, but we had to do what we had to do,” Virginia said. “Then, Dr. Silva suggested we try home dialysis. So, I got trained to do home dialysis and we did home dialysis overnight.”

“It was easier to do it at home, but I couldn’t do a lot of things at night,” said Niyear, who was home-schooled at the time. He was on overnight home dialysis for the next three months.

All of that changed for Niyear when his family found a kidney donor in May 2017. “Actually, it was his art teacher from elementary school who donated one of her kidneys,” Virginia explained. Niyear had been a student at the school for four years, and when an article ran in the local paper, his former teacher came forward to help. “She said she had to do something—and we are grateful,” Virginia said.

Although it took time for him to recover, Niyear—who has two younger brothers and a younger sister—said he feels good today and is looking forward to the future. “I want to go into the NBA or become a therapist,” he said.

Upon presentation to Connecticut Children’s, she was afebrile and tachycardic with a heart rate of 112. Physical examination was pertinent for moderate tenderness across the lower abdomen and pelvis without rebound or guarding. External genitourinary examination revealed normal female genitalia and no abnormalities.

Abdominal X-ray was unremarkable. Doppler ultrasound of the pelvis revealed hematometrocolpos with marked distension of the cervix and vagina and extension of blood products into the myometrium suggesting adenomyosis. Urinalysis showed large ketones and moderate blood but was negative for leukocyte esterase and nitrites. A CBC was within normal limits. She received ibuprofen for pain and ondansetron for nausea with subsequent improvement in these symptoms.

DIAGNOSIS/TREATMENT:

Gynecology was consulted and arrangements were made for outpatient follow-up the next day with Pediatric and Adolescent Gynecology. Because she did not have a bulging hymen on examination, the etiology of her hematometrocolpos was unlikely to be due to an imperforate hymen and concerning for a transverse vaginal septum. An outpatient MRI of the pelvis was performed a few days later and confirmed the presence of a transverse vaginal septum. The distal vagina, however, was not visualized on the MRI which may be due to distal vaginal agenesis or compression from a distended proximal vagina filled with blood. She was started on norethindrone acetate daily to counter estrogenic effects and suppress menstruation. She will undergo further evaluation of her genitourinary anatomy to determine the appropriate definitive surgical correction for her condition.

DISCUSSION:

Hematometrocolpos is a rare finding most often caused by retrograde menstruation secondary to an obstructive reproductive tract anomaly (ORTA). Obstructive defects are often diagnosed in puberty during the first year after menarche.(1) Imperforate hymen is the most common cause of vaginal outflow tract obstruction and occurs in approximately 1/2000.(2) A much more rare obstructive anomaly is a transverse vaginal septum which occurs as a result of failure of the Müllerian ducts and urogenital sinus to fuse or canalize during embryogenesis. The septum can be thin or thick and can be located at any level of the vagina, the most common location is in the upper third of the vagina.(3) Vaginal agenesis occurs in approximately 15% of cases and can be complete or partial.(4)

Adolescent females with an ORTA often go undiagnosed for several months to years. They typically present with primary amenorrhea and abdominal or pelvic pain but those with partially obstructive anomalies may present with menstrual periods and progressive dysmenorrhea.(5) Adolescents will present with worsening cyclic pelvic pain. Since teenagers may have irregular menstruation for the first 1-2 years, these episodes may not occur monthly but will occur with some recurrence over a period of time. Thus, a detailed history and timeline of symptoms are important in reaching this diagnosis.

As blood products build up in the vagina and uterus the pain becomes more severe and constant. The patient may develop urinary retention or difficulty with bowel movements. An abdominal mass may be palpated if the obstruction is long-standing. Retrograde menses may also lead to peritoneal seeding of endometrial tissue resulting in endometriosis.(6) In the case of an imperforate hymen, external genital examination usually reveals a bulging and/or bluish hymen which is diagnostic of the condition. In the absence of a bulging hymen, a transverse vaginal septum should be considered. Pelvic ultrasound should be performed initially to identify the presence of an abnormality. However, MRI is the gold standard imaging modality because it allows for classification of the exact type of Müllerian duct abnormality present which dictates further management.(7)

Imperforate hymen is treated surgically with excision of excess hymenal tissue and removal of menstrual contents. Management of a transverse vaginal septum is much more complicated. Some can be treated with vaginal dilation alone however many require septal excision with anastomosis of the proximal and distal parts of the vagina. This procedure carries a high risk of stenosis for which patients must undergo regular post-operative vaginal dilation. Younger patients may not tolerate vaginal dilation therefore, gynecologists may opt to chemically suppress menstruation and delay surgical repair.(1) Surgical correction has been shown to lead to resolution of sequelae of outflow tract obstruction such as urinary retention, constipation and even endometriosis.(6)

A delay in diagnosis and management of ORTAs can lead to many complications including infertility; thus, pediatricians and emergency medicine providers must consider these when evaluating an adolescent female with worsening recurrent pelvic pain.

REFERENCES:

1. Dietrich JE, Millar DM, Quint EH. Obstructive reproductive tract anomalies. J Pediatr Adolesc Gynecol. 2014;(27):396-402.
2. Breech LL, Laufer MR. Obstructive anomalies of the female reproductive tract. J Reprod Med. 1999; 44:233.
3. Rock JA. Anomalous development of the vagina. Semin Reprod Endocrinol. 1986; 4:13.
4. Laufer MR: Structural abnormalities of the female reproductive tract. In: Emans, Laufer, Goldstein’s Pediatric
and Adolescent Gynecology, 6th ed. Edited by Emans SJ, Laufer MR. 2012, p 334.
5. Sultan C, Gaspari L, Paris F: Adolescent dysmenorrhea. Endocr Dev 2012; 22:171.
6. Breech LL. Disorders of the female pelvis. In: Adolescent Medicine: The Requisites in Pediatrics, 1st ed. 2008,
p175-183.
7. Junqueira LP, Allen LM, Spitzer RF, et al: Mullerian duct anomalies and mimics in children and adolescents: correlative intraoperative assessment with clinical imaging. Radiographics 2009; 29:1085.

Initial symptoms are variable and have significant overlap with common illnesses. Children should be seen by a doctor immediately with the following symptoms:

• High, prolonged fever: temperature of 101 degrees or more for more than three days
• Difficulty feeding (infants) or is too sick to drink fluids
• Severe abdominal pain, diarrhea or vomiting
• Cracked lips
• Reddish eyes (similar to pinkeye)
• Rash
• Swollen glands/lymph nodes
• Change in skin color (becoming pale, patchy and/or blue)
• Racing heart or chest pain
• Decreased amount or frequency of urine
• Lethargy, irritability or confusion

The potential link to COVID-19 comes from the data of children’s testing positive for both this syndrome and coronavirus. A large number showed antibodies for COVID-19, meaning they might previously have been infected. This all suggests that the new inflammatory syndrome may be a delayed reaction to the coronavirus, driven by a child’s immune system response to COVID-19. Doctors are working to learn more. While still seemingly rare, if left untreated, this syndrome can lead to serious effects on the heart resulting from inflammation of coronary arteries and other blood vessels.

While this syndrome was initially similar to Kawasaki disease, doctors are becoming increasingly confident that this is not the same diagnosis. Treatment for this new illness have included high-dose aspirin and antibiotics, steroids, intravenous immunoglobulin (antibodies that help the body fight infection), as well as supportive oxygen through the nose, a mask or, in the most serious cases, a ventilator.

For clinical questions, the Connecticut Children’s Pediatric COVID-19 Hotline is available 24/7 at 833.226.2362 for patients, families and providers.

For more information on COVID-19, please visit our COVID-19 resource center for providers.

The resulting decreased vaccine coverage, may lead to additional outbreaks of preventable diseases, like measles and pertussis. Even small delays leave young children vulnerable. Ultimately, 80% of parents who have initially been hesitant to vaccinate will choose to immunize their child after a discussion with their primary care physician.

Connecticut Children’s Division of Primary Care approached the pandemic by continuing to operate and vaccinate their youngest patients. Despite that, there has been a drop-off in attendance to visits, mirroring the national experience. To address this challenge, Primary Care is using active outreach to patients and families to emphasize the importance of keeping up with vaccinations. These contacts provide the opportunity to let parents know that the clinic is open for both well and sick visits. This reassurance lets families know that Connecticut Children’s has the patient’s entire well-being in mind and is a safe place to receive care. In the community, it is critical to communicate to families what services are still offered in your practices and what has been done from an operational perspective to assure patient safety.

“We are thrilled to have been recognized as a pediatric sedation Center of Excellence,” said Jesse Sturm, MD, an emergency medicine physician and the Director of Sedation Services. “This distinction is confirmation of our dedication to go above and beyond to keep all children safe and sound during any hospital procedure that requires them to be calm and remain still.”

Connecticut Children’s pediatric sedation team is dedicated to helping kids feel less anxious and more comfortable during any procedure or test. The team works to minimize physical discomfort and reduce the negative psychological impact to its patients while helping complete necessary procedures, so that general anesthesia and breathing devices are not needed.

“The sedation service is an invaluable piece of the care we provide to our oncology patients, allowing them to receive sedation for lumbar punctures and bone marrow biopsies in a spacious and organized suite that meets the needs of patients, families and staff. The physicians, nurses, medical assistants and child life specialists are expert both in the medical service they provide and the kind manner in which they provide it,” said Natalie Bezler, MD, oncologist at Connecticut Children’s. “They have truly become part of our care team, getting to know our patients and providing a familiar smile and reassuring presence during an otherwise stressful time.”

Connecticut Children’s Center of Excellence in Pediatric Sedation designation is active for four years. As a Center of Excellence, Connecticut Children’s will now serve as a national example for other hospitals starting a pediatric sedation program.

This role will provide operational support in various practice settings and will be the central resource in addressing: scope of practice, state, federal and medical staff regulations, billing, compliance, APP utilization strategies. They will engage and work with many different partners throughout the organization in accomplishing the strategic goals of the role. These include:

1. Overseeing the development of novel educational strategies for new APPs, as well as professional training and leadership-building opportunities for currently practicing Connecticut Children’s APPs;
2. Maintaining relationships with nurse practitioner and physician assistant training programs to help coordinate student clinical education in Connecticut Children’s facilities and practices, and
3. Developing collaborative practice models and perform research and/or quality initiatives to support innovative and progressive health care delivery systems.

Please join us in congratulating them on this exciting journey as we continue the work of supporting our advanced practice providers.

Dr. Rieger will be in Danbury on Mondays and Tuesdays, Dr. Katsman will be in Danbury on Thursdays and Shelton on Fridays. The two physicians will share the OR on Wednesdays. With expertise in pediatric and adolescent sports medicine, pediatric orthopedics, pediatric trauma and arthroscopic surgery, Dr. Katsman chose Connecticut Children’s because of its commitment and dedication to the care and well-being of children. She will oversee our female athlete program in Fairfield County.

Dr. Rieger has served many leadership roles previously, such as, but not limited to, President of The Orthopedic Center in Pennsylvania and President of the Orthopedic Network Inc., in New Jersey’s statewide IPA. With pediatric orthopedics and sports medicine-specific fellowship experience paired with leadership-style ambition, Connecticut Children’s is thrilled to welcome Drs. Katsman and Rieger to the team to better serve children’s across the state and region.

Importantly for patients, it is a 340B program which allows us to offer medication at a discounted price for patients. In conjunction with other departments, such as genetic counseling, infectious disease, pain medicine, orthopedic surgery, dentistry, social work and physical therapy, the Hemostasis and Thrombosis Program team works together to create individualized coordinated care plans and sees patients at three convenient locations in Hartford, Shelton and Farmington.

Emily joins Connecticut Children’s with over 8 years of experience as a certified pediatric nurse practitioner. She has particular clinical expertise in treating hemophilia, von Willebrand disease, rare factor deficiencies, platelet disorders, management of blood clots, anticoagulation monitoring, and the care of women and girls with blood disorders. Emily and her colleagues work to develop emergency treatment plans and ensure that the patient’s pediatric home remains the primary point of contact.

Nancy Lewis, MD | NICU

Education:

• MD, Duke University School of Medicine
• Residency in Pediatrics, Massachusetts General Hospital
• Fellowship in Neonatal-Perinatal Medicine, Harvard Combined Program in Neonatology

Faculty Appointment

• Associate Professor of Pediatrics, UConn School of Medicine

Clinical Expertise

• Assessment and care of critically ill newborns
• Management of neonatal respiratory and gastrointestinal disorders
• Intestinal inflammation and immunity

2020-2021 EVENING LECTURE SERIES – VIRTUAL

TITLE: Identifying Medically Intractable Epilepsy: Who is a Candidate for Epilepsy Surgery?
DATE: September 10, 2020

TITLE: Autism Management 101
DATE: October 20, 2020

TITLE: Adolescent GYN
DATE: January 14, 2021

TITLE: Ophthalmology
DATE: March 25, 2021

TITLE: Neuropsychology
DATE: April 20, 2021

POSTPONED: 3rd Annual Joint Pediatric Symposium
The Joint Pediatric Symposium originally scheduled for June 5, 2020 in Danbury is postponed until June 2021.

For all CME events, stay up to date by visiting our calendar at cccme.eeds.com

Farmington:

Ophthalmology has moved to 599 Farmington Avenue, Farmington
Optical Shop will be open in August 2020
599 Farmington Avenue, Farmington

Fairfield County:

Hematology/Oncology and the Hemostasis and Thrombosis Program
2 Ivy Brook Road, Shelton

Orthopedics & Sports Medicine available July 6
105A Newtown Road, Danbury
2 Ivy Brook Road, Shelton

Glastonbury:

Orthopedics & Sports Medicine Walk-in/Same Day
310 Western Boulevard, Glastonbury