Online Exclusives
Gastric outlet obstruction in a neonate because of Peutz-Jeghers syndrome
Neonatal detection of Peutz-Jeghers syndrome is unusual with only 2 cases previously reported in the literature. We describe a neonate presenting with gastric outlet obstruction owing to 2 large Peutz-Jeghers polyps. The child's father and grandmother were known to have Peutz-Jeghers syndrome. On the ninth day of life, the infant underwent colonoscopy, abdominal exploration, and complete surgical resection of 3 polyps. The postoperative course was uneventful, and the patient was discharged home at the age of 3 weeks on full oral feeds.Post meningocele repair urinary ascites in a neonate—a rare presentation
A full-term female neonate with a large lumbosacral meningocele developed sudden abdominal distension and urinary retention after meningocele repair. An erect abdominal radiograph showed homogeneously opacified areas with a paucity of intestinal gas. Abdominal ultrasound revealed ascites with multiple internal echoes. With a provisional diagnosis of hollow viscus perforation, an emergency laparotomy was performed, which failed to reveal any leak from the urinary tract. However, fluid analysis confirmed the diagnosis of urinary ascites.Meconium pseudocyst with particular pathologic findings: a case report and review of the literature
Meconium peritonitis is a sterile chemical peritonitis caused by bowel perforation with intraperitoneal extravasation of the meconium in utero. When the inflamed intestinal loops become fixed, meconium peritonitis leads to a cystic cavity with a fibrous wall, and the result is termed cystic-type meconium peritonitis. On the contrary, a meconium pseudocyst has a muscle layer continuous with the normal intestine and is distinguished from cystic-type meconium peritonitis based on the histopathologic findings.Superior mesenteric artery syndrome: a rare cause of complete intestinal obstruction in neonates
Superior mesenteric artery syndrome (SMAS) is an uncommon cause of high intestinal obstruction in neonates; it is owing to incomplete obstruction in the third part of the duodenum caused by compression between the SMA and abdominal aorta. In neonates, complete intestinal obstruction owing to SMAS has been very rarely reported in the literature .We present a 7-day-old previously healthy male infant with a short history of gastroenteritis and sepsis followed by progressive abdominal distension and persistent bilious vomiting that resulted in hypovolemic shock.Pyloric atresia associated with Dieulafoy lesion and gastric dysmotility in a neonate
In this case study, we report a male infant with pyloric atresia, extreme gastric distension, and a caliber-persistent gastric artery (Dieulafoy lesion) with massive gastric bleeding. After a transverse pyloroplasty and endoscopic hemoclip application to the caliber-persistent gastric artery, very slow gastric emptying developed, which required repeated surgical interventions. Gastroduodenostomy failed to promote gastric emptying. The intraoperative and postmortem histologic examinations of the gastric wall revealed a loss of interstitial cells of Cajal, which possibly explains the extreme motility disorder.Enhancing safety of laparoscopic vascular control for neonatal sacrococcygeal teratoma
Life-threatening bleeding is a hazard of major tumor excision in children. However, fatalities from inadvertent arterial ligation should not be overlooked. Sacrococcygeal teratoma is the commonest neonatal tumor. Laparotomy to ligate the median sacral artery has been used to preempt potentially fatal resectional bleeding. Use of laparoscopy to achieve the same is an evolving technique, with only 7 neonatal cases described. As such, the Idea, Development, Exploration, Assessment, Long-term study (IDEAL) guidelines on surgical innovation recommend case reports addressing proof of concept, technical factors and safety tips.Segmental dilatation of sigmoid colon in a neonate: atypical presentation and histology
Segmental dilatation of the colon is a rare disorder of colonic motility in children, often presenting with severe constipation in older infants, children, and occasionally adults. It may mimic the commoner Hirschsprung disease clinicoradiologically but differs in that the ganglion cell morphology and distribution are typically normal in the colon. We report a neonate with segmental dilatation of the sigmoid colon who had an atypical clinical presentation and describe certain abnormalities in bowel histology (hypertrophied muscularis propria, nerve plexus, and ganglion cells located within the circular layer rather than the normal myenteric location), for the first time in the English literature.Segmental transposition of ileal muscle layers: a rare cause of myopathic pseudoobstruction in a newborn
Gastrointestinal motility disorders are induced by various causes in neonates, some of which are not classified. Generally, these disorders can be classified as a neuropathy or myopathy, each of which can be subdivided into a primary or secondary type. We describe a neonate with a rare form of myopathy related to segmental transposition of the smooth muscle of the ileum and a brief review of the relevant literature.Successful thoracoscopic staged repair of bilateral congenital diaphragmatic hernia
Congenital diaphragmatic hernia (CDH) occurs in 1 of every 2000 to 3000 live births and accounts for 8% of all major congenital anomalies. Bilateral hernias account for no more than 1% of all types of CDH; furthermore, they are usually fatal prenatally. Those that survive are identified through a limited number of case reports. We are presenting an unusual case of bilateral CDH hernia in a neonate that was repaired successfully using a staged thoracoscopic approach. To our knowledge, this represents the first case in the world literature of bilateral CDH that was repaired thoracoscopically.Right congenital diaphragmatic hernia associated with anorectal malformation
We describe a neonate in whom a right congenital diaphragmatic hernia and an anorectal malformation coexisted. Their coexistence in the same patient is rare.Neonatal congenital pancreatic cyst: diagnosis and management
Congenital pancreatic cysts are extremely rare in newborns. The case of a neonatal congenital pancreatic cyst with pancreatitis is reported. The rapid increase in cyst size concomitantly with clinical symptoms prompted surgical management. Intraoperatively, the pancreatic head showed signs of chronic pancreatitis, and in attempts to preserve most of the functional pancreatic tissue, a Roux-en-Y cystojejunostomy was performed. Histology demonstrated a true pancreatic cyst with degenerated epithelial lining.Congenital pouch colon syndrome in a Saudi Arabian neonate
Congenital pouch colon syndrome is partial or complete replacement of the colon by a pouch-like structure and anorectal malformation. Commonly reported from northern India, we believe this is the first report of congenital pouch colon syndrome in a Saudi Arabian neonate. He was referred with an imperforate anus and diagnosed as having a high anorectal malformation. The patient underwent a sigmoid colostomy. Continued abdominal distension after the colostomy prompted sonography and computed tomography, which showed an air- and fluid-containing cystic structure in the abdomen.Congenital true pancreatic cyst detected prenatally in neonate: a case report
True congenital epithelium-lined cysts of the pancreas are rare, and the prenatal diagnosis of a true pancreatic cyst has been reported only rarely. In this article, the authors report a 3-day-old girl with histologically proven true pancreatic cyst that was detected by prenatal ultrasonography. The cystic mass was located in the left upper side of the abdomen, and total cystectomy was performed without complication.Wolff-Chaikoff effect in a newborn: is it an overlooked problem?
Hypothyroidism is a serious endocrine disorder emerging from deficient production of thyroid hormone (thyroid gland agenesis or dysgenesis, or inborn metabolic defects of thyroid hormone production) or a defect in thyroid hormonal receptor activity. Prevention of iodine organification by means of using iodine-containing drugs or solutions is a protective mechanism for the body and is known as the Wolff-Chaikoff effect. This effect blocks thyroid hormone generation and is often transient, with thyroid hormone synthesis recovering in a few days or weeks.Malignant peripheral nerve sheath tumor arising from the colon in a newborn: report of a case and review of the literatures
A malignant peripheral nerve sheath tumor (MPNST) is a rare neoplasm arising from peripheral nerve sheath. Here, we report the first case of MPNST arising in the colon and also the youngest case of MPNST in the gastrointestinal tract. The patient was a 2-day-old neonate with symptoms and signs of intestinal obstruction. The patient had no family history or stigmata of neurofibromatosis type 1. A computed tomographic scan revealed a 5-cm-sized mass in ascending colon causing intestinal obstruction, and emergent right hemicolectomy was performed.Malignant vascular tumors of liver in neonates
Malignant vascular tumors of the liver are rare in children, especially in neonates. We report two cases. One was initially diagnosed as infantile hemangioendothelioma. Rapid growth and appearance of pulmonary metastasis while on aggressive medical treatment suggested malignant transformation. The other neonate presented with intractable ascites and liver biopsy showed histological features of angiosarcoma. Ascites has rarely been reported as a presenting feature of angiosarcoma in literature. Both patients died.Surgical repair of combined gastroschisis and sternal cleft
We present a case of prenatally diagnosed gastroschisis combined with a sternal cleft, as well as the successful surgical management of this unusual condition. Successful management of gastroschisis combined with sternal cleft has not been reported before in the literature.
