Online Exclusives
Novel approach to vaginal calculus in a girl with urogenital sinus anomaly
Isolated urogenital sinus can cause distended bladder and/or vagina and may present with an abdominal mass and sepsis during infancy. Older children may present with recurrent urinary tract infections and hematocolpos. We describe a 3-year-old girl with recurrent urinary tract infections thought to be secondary to vesicoureteric reflux. On further investigation, an isolated urogenital sinus anomaly with a calculus inside one of the hemivaginae was noted. She was managed expectantly with a plan to intervene at puberty.Small left colon syndrome in 3 sisters
Neonatal small left colon syndrome is a rare cause of bowel obstruction. Its etiology remains unknown, but a significant association has been noted between maternal diabetes and small left colon. No reported cases within the same family could be found in the literature, excepting 2 sets of twins. We report 3 cases of small left colon syndrome in 3 consecutive sisters born of a nondiabetic mother. This raises the question of a genetic factor in its etiology.Extrahepatic vitelline vein aneurysm: prenatal diagnosis and follow up
Umbilical vein varix is a well-described prenatal anomaly in which the prognosis remains unclear. We describe a very rare venous malformation that mimicked an umbilical vein varix consisting of a persistent vitelline vein. From 2003 to 2010, three patients were referred starting at 20 weeks gestation to our prenatal centers for an umbilical vein varix diagnosis. Fetal follow up was unremarkable, with the exception of the dilated vein size (mean: 35 mm at 33 weeks gestation). After birth, the three children presented with thrombosis from the aneurysmal sac to the portal trunk.Cloacogenic polyps in an adolescent: A rare cause of rectal bleeding
Cloacogenic polyps are rare inflammatory lesions occurring around the anal transitional zone and in the lower rectum which can mimic neoplasia. They should be given diagnostic consideration in patients presenting with symptoms of rectal bleeding, tenesmus, and altered bowel habits.Peliosis hepatis in a child with myotubular myopathy: Successful treatment using hepatic artery embolization
Peliosis hepatis (PH) is a rare condition characterized by multiple blood-filled spaces within the hepatic parenchyma that can lead to fatal hemorrhage. There is no consensus on the best treatment algorithm for PH, and therapy is directed at removing the potential causative agent with operative intervention when necessary. Here we present the first known case of PH in a child with myotubular myopathy who was successfully treated with angiography and hepatic artery embolization as a first line therapy, without the need for operative intervention.Fetal primary small bowel volvulus in a child without intestinal malrotation
Fetal primary small bowel volvulus without atresia or malrotation is an extremely rare but life-threatening surgical emergency. We report a case of primary small bowel volvulus that presented as sudden fetal distress and was diagnosed on the basis of the ‘whirl-pool sign’ of fetal sonography. This diagnosis led to emergency operation after birth at the third trimester with a good outcome. Although the pathogenesis of fetal primary small bowel volvulus is unclear, ganglion cell immaturity may play a role in the etiology.Esophageal atresia, small omphalocele and ileal prolapse through a patent omphalomesenteric duct: A methamizole embryopathy?
Newborns prenatally exposed to methimazole (active metabolite of carbamizole) for maternal hyperthyroidism may present some disorders in common, but the phenotype is not well defined. Choanal atresia is the most frequent, and other anomalies such as esophageal atresia and aplasia cutis were described with this embryopathy. Additionally, patent omphalomesenteric duct or Meckel's diverticulum in similar association was reported in some patients. The predisposed genetic background has to be considered.Ovarian steroid cell tumor, not otherwise specified, associated with congenital adrenal hyperplasia: rare tumors of an endocrine disease
Ovarian steroid cell tumors, not otherwise specified (OSCTs), are extremely rare and present a diagnostic challenge when evaluating an ovarian mass. We present a case of such a tumor in a patient with known Congenital Adrenal Hyperplasia (CAH), secondary to 21-hydroxylase deficiency, who was noncompliant with her medications. The workup, diagnosis, and treatment of this rare condition are described.Radiofrequency ablation of a large hepatic adenoma in a child
Hepatic adenomas are rare benign liver tumors seen most commonly in young women on oral contraceptives. Large hepatic adenomas are at risk of malignant transformation and require treatment in select patients, usually by surgical resection. Radiofrequency ablation (RFA) has become a widely used and accepted tool for the curative treatment of small primary hepatocellular carcinomas in adults; however, its use in the treatment of other liver lesions, such as hepatic adenoma, has only recently been described.Intra-thoracic appendicitis in a child with Down’s syndrome
Morgagni congenital diaphragmatic hernias are rare. They are associated with chromosomal anomalies, especially Down’s syndrome. Acute appendicitis is the most common surgical emergency in childhood, but accurate diagnosis can be a challenge if encountered out of the usual anatomical position. This is the first case report of acute appendicitis within a Morgagni hernia in a child with Down’s syndrome.Staple line ulcers: A cause of chronic GI bleeding following STEP procedure
Intestinal failure (IF) results from a critical reduction in the functional intestinal mass resulting in dependence on total parenteral nutrition (TPN) for growth and survival. Short bowel syndrome (SBS) is the most common cause of intestinal failure in pediatrics. Following resection, the small bowel undergoes adaptation, a process wherein the bowel elongates and dilates in order to increase intestinal absorptive capacity. Small bowel dilatation can lead to dysmotility and small bowel bacterial overgrowth which may further enhance feeding intolerance.Esophageal atresia with proximal tracheoesophageal fistula: A missed diagnosis
This retrospective study was performed to compare the relative incidence of esophageal atresia (EA) with proximal tracheoesophageal fistula (PTEF) at our institution with those reported in literature and to test the hypothesis that our higher relative incidence is caused by the routine use of tracheoscopy.Extrarenal Wilms tumor: a case report and review of the literature
Extrarenal Wilms tumors are extremely rare with only isolated case reports in the pediatric literature. We present the case of a 2-year old boy who presented with a large abdominal mass and constipation. Pathologic diagnosis of the tumor was extrarenal Wilms tumor (ERWT) with favorable histology. We discuss the diagnostic workup, radiologic and operative findings, treatment and review of the literature.Apple-peel intestinal atresia: Enteroplasty for intestinal lengthening and primary anastomosis
Apple-peel atresia (or Type-IIIb intestinal atresia) is an unusual type of jejunoileal atresia. They present with jejunal atresia near the ligament of Treitz and a foreshortened small bowel. Many surgical options have been used, but the optimal method of repair remains unclear. We present a case of a newborn with apple-peel intestinal atresia managed by enteroplasty for intestinal lengthening and primary anastomosis.Bladder calculus formation on the tip of a migrated disused ventriculoperitoneal shunt
Bladder stones in children are rare. They generally occur in children with one or more predisposing risk factors to stone formation. In this unique case report we discuss the formation and successful endoscopic treatment of a large stone in a neuropathic bladder on the tip of a migrated VP shunt.Traumatic hepatic artery laceration managed by transarterial embolization in a pediatric patient
While blunt abdominal trauma with associated liver injury is a common finding in pediatric trauma patients, hepatic artery transection with subsequent treatment by transarterial embolization has rarely been reported. We present a case of a child who suffered from a hepatic artery injury which was successfully managed by supraselective transarterial microcoil embolization, discuss management strategies in these patients, and provide a review of currently available literature.Foregut duplication cyst associated with esophageal atresia and tracheoesophageal fistula: A case report and literature review
A case of esophageal atresia associated with a foregut duplication cyst is reported and the literature reviewed. This is the first documented occurrence in conjunction with Down syndrome and the second case where both anomalies were treated at the initial surgery.Video-assisted surgery in the management of hydatid renal cyst in children
Renal hydatid disease is rare in children. Open surgery is the traditional method of treatment, but minimally invasive techniques are being increasingly used. Herein, we report our experience with laparoscopic management of renal hydatid cyst in four children via a transperitoneal approach in three cases and a retroperitoneoscopy in one. We conclude that transperitoneal laparoscopy can be offered for the management of hydatid renal cyst associated with other intraperitoneal localizations, whereas the retroperitoneoscopy is limited for the treatment of isolated hydatid renal cysts.Isolated caudate lobe (Spiegel lobe) resection for hepatoblastoma. Is it enough to achieve a sufficient resection margin? A case report
Hepatoblastoma arising from and limited to the caudate lobe is an extremely rare clinical entity. The object of this case report is to present a case of isolated caudate lobe resection due to hepatoblastoma originating in the caudate lobe.Staged closure with negative pressure wound therapy for gastroschisis with liver herniation: A case report
Liver herniation with gastroschisis is an uncommon occurrence that is associated with a poor prognosis. This report presents a single case of complex gastroschisis complicated by herniation of the left hepatic lobe. In the subject case, the abdominal wall defect was successfully closed by sequential closure with negative pressure wound therapy after the initial application of a preformed silo. As there are no established standards for the management of gastroschisis with liver herniation, there exists an opportunity for multicenter review to define approaches to optimize clinical outcomes with this complex congenital issue.Pelvic widening to alleviate the mechanical component of constipation in a patient with severe caudal regression and extremely narrow pelvis
Constipation in children is a commonly encountered problem with a broad variety of causes. Constipation caused by a narrow pelvis has, to our knowledge, not been reported in the human literature.A newborn with caudal duplication and duplex imperforate anus
There are case reports of duplication of the colon, rectum, anus, urinary system, lower genital tract, and external genitalia, spinal anomalies, and abdominal wall defects. However, it is rare to encounter a single newborn with all of the mentioned abnormalities, which have been defined as the caudal duplication syndrome (CDS). Herein, we present a newborn with an omphalocele, duplex external genitalia (with duplex labia minora and labia majora), duplex urethral orifices, duplex vaginal orifices, and duplex anal dimple with imperforate anus and rectovestibular fistula on both sides.Pulmonary artery pseudoaneurysm after MRSA septicemia in a pediatric patient
We document a previously unreported case of a pulmonary artery pseudoaneurysm (PAP) that developed in a pediatric patient initially presenting with Methicillin-resistant Staphylococcus aureus hip joint sepsis. This is the first reported case of PAP of infectious origin in a pediatric patient, and this case is unique as the evolution of the PAP was documented by computed tomography.Povidone-iodine for persistent air leak in an extremely low birth weight infant
Chemical pleurodesis with povidone-iodine has been used to treat congenital idiopathic chylothorax in neonates. We report successful use of povidone-iodine in an extremely premature infant to treat a pneumothorax that had persisted for more than one week despite high-frequency ventilation.Retroperitoneal lymphatic malformation and transverse testicular ectopia: A unique clinical presentation
This case report presents a fetal patient diagnosed in utero with a retroperitoneal lymphatic malformation by ultrasound and followed through gestation. At birth the child was noted to have a right inguinal hernia with two palpable testicles. Plan for partial resection and hernia repair with postoperative sclerotherapy was made. At the time of hernia repair, transverse testicular ectopia was diagnosed, and subsequent extraperitoneal transposition orchiopexy was performed following partial resection of the lymphatic malformation.
