Tag Archives: Pediatrics

General Surgery, Pediatrics, Print Release, Urology

PUBLISHED: Cloacal Exstrophy Repair

Cloacal Exstrophy Repair
William Remley1; Howard Jen, MD2; Carl-Christian A. Jackson, MD2; Jeremy Wiygul, MD2
1Lake Erie College of Osteopathic Medicine
2Tufts Medical Center

Cloacal exstrophy is congenital malformation marked by an abdominal wall defect with open and exposed hindgut and bladder. It is the most severe birth defect within the exstrophy-epispadias complex, and when spinal defects are also present, it is called the OEIS (omphalocele, exstrophy, imperforate anus, and spinal defect) complex. Cloacal exstrophy is rare, occurring in 1/200,000–400,000 births, but it can be diagnosed on prenatal ultrasound. The defect results in two exstrophied hemibladders separated by an exposed cecal plate, with the distal hindgut being foreshortened and blind-ending, resulting in an imperforate anus. There is diastasis of the pubic symphysis, and the genitalia are separated. In males, the phallus is usually split in half, flattened and shortened, with the inner surface of the urethra exposed. In females, the clitoris is split, the labia are widely separated, and there may be two vaginal openings. Cloacal exstrophy is also highly associated with other birth defects, especially spina bifida, which coexist in up to 75% of cases. Multidisciplinary care followed by surgical management should begin immediately following the baby’s delivery.

Surgical goals in the neonatal period include closure of the meningocele and repair of the exstrophy and omphalocele, resulting in approximation of the bladder halves and repair of the hindgut defect with colostomy creation. Closure of the bladder, with positioning within the pelvis, can either occur at the initial operation or be staged to occur after a period of monitored growth, and is best performed with pelvic osteotomies to protect the closure from tension. Subsequent surgeries over several years will address genital reconstruction and colonic pull-through for fecal continence, if the patient is a candidate. Here, we present a patient diagnosed with OEIS complex by prenatal ultrasound, with a postnatal exam confirming the diagnosis and demonstrating a closed (covered) myelomeningocele. The cloacal exstrophy and omphalocele were repaired in one stage, with primary closure of the involved bowel and the bladder, facilitated by pelvic osteotomies.

General Surgery, Global Surgery, Pediatrics, Print Release

PUBLISHED: Pediatric Infant Bilateral Open Inguinal Hernia Repair – Twin B

Pediatric Infant Bilateral Open Inguinal Hernia Repair – Twin B
Casey L. Meier, RN1; Lissa Henson, MD2; Domingo Alvear, MD3
1Lincoln Memorial University, DeBusk College of Osteopathic Medicine
2Philippine Society of Pediatric Surgeons
3World Surgical Foundation

Indirect inguinal hernia repair is a common procedure for premature infants because of the frequency of a patent processus vaginalis. Prompt surgical correction decreases the risk of incarceration, strangulation, and necrosis in children. There are various techniques for herniorrhaphy. This repair demonstrates an open bilateral indirect inguinal hernia repair in an infant that avoids high ligation by closing the internal inguinal ring, utilizing a purse-string method to keep the hernia sac intact. This approach limits the amount of anesthesia used and prevents excess bleeding, making it safe, effective and efficient.

General Surgery, Global Surgery, Print Release

PUBLISHED: Anal Fistulotomy

Anal Fistulotomy
M. Grant Liska, BS¹; Marcus Lester R. Suntay, MD, FPCS, FPSPS, FPALES²
¹University of Central Florida College of Medicine
²Philippine Children’s Medical Center

Fistula-in-ano is a chronic abnormal communication between the anal canal and, usually, the perianal skin. It can be described as a hollow tract that is lined with granulation tissue and connects a primary opening inside the anal canal to a secondary opening in the perianal skin. It usually originates from the anal glands and is frequently the result of a previous anal abscess. Anal fistulae present with pain, swelling, pruritus, skin irritation, and purulent or bloody drainage. Most anal fistulae are diagnosed based on clinical findings, but complex and deep anal fistulae usually require imaging studies such as CT scan or MRI to delineate the tract.

Currently, there is no medical treatment available and surgery is almost always necessary. A simple intersphincteric fistula can often be treated with fistulotomy or fistulectomy, while trans-sphincteric and suprasphincteric fistulae are treated by placement of a seton to maintain drainage and induce fibrosis. Extrasphincteric fistula treatment depends on the anatomy and etiology of the fistula.

This article presents the case of a 1-year-old male with a history of recurrent perianal infection, which led to the development of an anal fistula. The anal fistula was noted to be superficial, and a fistulotomy was performed.

Announcement, Pediatrics, Preprint Release, Urology

PREPRINT RELEASE: Prophylactic Laparoscopic Bilateral Gonadectomy for Complete Androgen Insensitivity Syndrome

Prophylactic Laparoscopic Bilateral Gonadectomy for Complete Androgen Insensitivity Syndrome
University of Arkansas for Medical Sciences

J. Corbin Norton, MD
Department of Urology
University of Arkansas for Medical Sciences

Stephen J. Canon, MD
Department of Pediatric Urology
Arkansas Children’s Hospital

Amrit Singh, MD
Department of Pathology
University of Arkansas for Medical Sciences / Arkansas Children’s Hospital

Laura L. Hollenbach, MD
Department of Obstetrics and Gynecology
University of Arkansas for Medical Sciences

Georgia Gamble, MD
Department of Obstetrics and Gynecology
University of Arkansas for Medical Sciences

Laura A. Gonzalez-Krellwitz, MD
Department of Pathology
University of Arkansas for Medical Sciences / Arkansas Children’s Hospital

The patient, in this case, is a 15-year-old female who presented with primary amenorrhea and who on work-up was found to have complete androgen insensitivity syndrome. Here, Dr. Canon at the University of Arkansas for Medical Sciences performs a prophylactic laparoscopic bilateral gonadectomy to reduce her future risk for intra-abdominal testicular malignancies. Final pathology results showed a rare case of bilateral germ cell neoplasia in situ and bilateral paratesticular leiomyomas and reinforced the decision to intervene early allowing for the removal of the gonads prior to their conversion to formal germ cell tumors.

General Surgery, Pediatrics, Preprint Release

PREPRINT RELEASE: Cloacal Exstrophy Repair

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0102b-feature-imageCloacal Exstrophy Repair
Jeremy B Wiygul MD
Chief, Pediatric Urology; Assistant Professor Tufts Medical Center

Dr. Wiygul and colleagues take a multidisciplinary approach to treating a cloacal exstrophy, a rare congenital defect that is part of the OEIS complex: omphalocele, exstrophy of the cloaca, imperforate anus, and spine deformities.
Orthopedics, Pediatrics, Preprint Release

PREPRINT RELEASE: Pelvic Osteotomies for Cloacal Exstrophy

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0102A-screenshotPelvic Osteotomies for Cloacal Exstrophy
Purushottam Gholve, MD MBMS MRCS
Pediatric Orthopedic Surgeon
Assistant professor, Tufts University School of Medicine

Dr. Gholve assists pediatric urology team at Tufts in treating a cloacal exstrophy by sequentially closing the pelvic ring with controlled osteotomies.