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Introduction Neonatal intestinal perforation usually occurs at distal small bowel secondary to distal bowel obstruction. The aim of this report is to describe an unusual case of total colonic aganglionosis with an initial presentation of proximal jejunal perforation. Presentation of case A male newborn presented with jejunal perforation on the fifth day of life and was treated by laparoscopic primary repair. Abdominal distention persisted postoperatively, and radiological examination revealed an obstruction near the terminal ileum. Laparotomy showed a transition zone 30-cm proximal to the ileocecal valve, and diverting ileostomy and appendiceal biopsy was performed. Permanent section demonstrated the complete absence of ganglion cells in the appendix and total colonic aganglionosis was strongly suspected. Discussion Contrary to the classic teaching, proximal bowel perforation can occur in case of far distal obstruction, and careful distal evaluation would direct more appropriate surgical treatment option. Conclusions Total colonic aganglionosis can present as a proximal bowel perforation. Careful distal evaluation can provide diagnostic clues in cases of proximal intestinal perforation. Appendiceal biopsy is a reliable tool for evaluating suspected total colonic aganglionosis, but multiple colonic and rectal biopsies should be obtained to confirm the diagnosis. [ABSTRACT FROM AUTHOR]

Background The sequelae of a central venous cutdown usually include venous deformity causing venous stenosis or stricture. However, the cellular mechanisms causing these deformities have not been elucidated. Methods Silicone 2.7-Fr catheters were placed via the right external jugular vein of 16 rats with the cutdown method. After fixation with formalin at scheduled intervals (1 week, 2 weeks, 4 weeks, and 8 weeks; 4 rats in each group), the vein segment with the catheter in situ was harvested. Histological changes in the vein wall were studied and serially compared with light microscopy; standard hematoxylin-eosin staining, Masson’s trichrome staining, van Gieson’s elastin stain, and immunohistochemical stain against α-actin. Results Pericatheter sleeve formation, circumferential smooth muscle cell proliferation and infiltration into the pericatheter sleeve by direct contact were noted in all 4 rats of 1-week model; this indicated the initiation of neointimal hyperplasia. The neointimal hyperplasia was located inside the elastin layer. At 2 weeks, the SMCs stained faintly but the components of the vein wall were largely replaced by collagen. The proliferation and infiltration of SMCs stabilized at 4 weeks and no SMCs were stained around the catheter. At 8 weeks, luminal narrowing was noted and the venous wall was composed mainly of collagen. Conclusions Circumferential neointimal hyperplasia occurred after surgical cutdown of the external jugular vein in a rat model and was caused by SMC activation, proliferation, and infiltration into the pericatheter sleeve. [ABSTRACT FROM AUTHOR]

Catheter-related or perianal problems are common surgical complications in patients with pediatric malignancies. Acute intestinal obstructions requiring surgical intervention are rare, however. We herein report our experience of 7 patients with separate primary diagnoses who developed acute intestinal obstruction that mandated surgery before or during chemotherapies. The original diseases were hematologic malignancies in 5 patients (3 leukemia, 1 lymphoma, 1 Langerhans cell histiocytosis (LCH)) and solid tumors in 2 patients (1 intra-abdominal desmoplastic small round cell tumor (DSRCT), 1 rhabdomyosarcoma of vagina). The intestinal obstructions were treated by resection of the involved segment with ( N = 2) or without ( N = 3) enterostomy. Two cases of intestinal obstruction were treated with an enterostomy alone. Three patients died due to the progression of the original disease, and 3 patients are currently alive. One patient was lost to follow-up. Although rare, acute intestinal obstructions can occur during the treatment course of pediatric malignancies or be an initial symptom of disease. Operative intervention is mandatory and effective in the treatment of such complications. Overall patient prognosis is dependent on the original disease. [ABSTRACT FROM AUTHOR]

Abstract: Background/Purpose: A silicone central venous catheter (CVC) is usually inserted using a percutaneous technique under general anesthesia. However, there are numerous reports on the postoperative adverse effects of general anesthesia in neonates. The aim of this study is to investigate the feasibility of open surgical cutdown (OSC) for central venous access without general anesthesia. Methods: The medical records of patients who underwent OSC at bedside under sedation and local anesthesia were reviewed. Chloral hydrate (100mg/kg) was given orally for the induction of moderate to deep sedation 15minutes before OSC; then the operative field was infiltrated with 1% lidocaine. When adequate sedation was not achieved, a bolus of phenobarbital (20mg/kg) was given intravenously. Results: Thirteen Broviac lines were inserted into 12 patients. At insertion, the median gestational age was 29weeks, birth weight was 1,140g and age was 33days. No patients required invasive ventilator care; 7 patients received nasal non-invasive ventilator care. Neither intubation nor inotropics were required during the intra- or postoperative period and no perioperative surgical complications occurred. The median catheter duration was 19.5days. Conclusion: OSC at bedside for CVC insertion, using adequate sedation and local anesthesia, is a feasible procedure in neonates. [Copyright &y& Elsevier]

Intestinal obstruction usually occurs as a result of postoperative adhesion; only rarely have there been reports of Treves' field transmesenteric hernia as a cause of intestinal obstruction in patients without preceding abdominal surgery. A 9-year-old girl presented with recurrent episodes of abdominal pain and vomiting. A plain radiograph showed mechanical bowel obstruction, and abdominal CT failed to show a definitive etiology of the obstruction. Laparotomy revealed Treves' field transmesenteric herniation without bowel strangulation. Reduction and closure of the defect cured the patient. Recurrent obstructive symptoms and spontaneous resolution can be a presentation of Treves' field transmesenteric hernia in patients without predisposing factors of intraabdominal adhesion. Diagnosis could be aided by radiological examination, but prompt initiation of surgical exploration on the basis of clinical presentation is mandatory in suspected cases to avoid significant bowel injury. [ABSTRACT FROM AUTHOR]

Dumping syndrome (DS) is common after gastrectomy or gastric bypass surgery, but DS in children has been described almost exclusively as a complication of Nissen fundoplication. DS resolves spontaneously in most patients; however some patients require dietary modification or medications, and surgical treatment is reserved for medically intractable cases. We herein describe an unusual case of DS in a neurologically impaired infant that developed after Nissen fundoplication and pyloroplasty and was treated by reversal of the pyloroplasty. A 12-month-old male infant was referred for medically intractable DS. He suffered from cerebral palsy and gastroesophageal reflux and underwent open Nissen fundoplication, gastrostomy tube placement, truncal vagotomy, and pyloroplasty two months before referral. Gastric emptying scintigraphy confirmed rapid gastric emptying, which was consistent with early DS. On laparotomy, a pyloroplasty scar was identified and opened along the scar. The pyloric ring was identified, reapproximated, and the incision was closed longitudinally. Postoperative gastric emptying scan showed normalized gastric emptying, and the half-emptying time was measured as 110 min. Pyloric reconstruction is a technically easy and less destructive surgery and should be considered as a therapeutic option in medically refractory DS following fundoplication and pyloroplasty. [ABSTRACT FROM AUTHOR]

Intestinal volvulus usually occur secondary to malrotation, and primary segmental volvulus has rarely been reported. A 12-month-old female infant presented with a 3-day history of excessive vomiting. An ultrasonography revealed a “whirlpool sign” in the right upper abdomen, suggesting small bowel volvulus with obstruction. Laparotomy revealed a twisted, viable loop of small bowel in the right upper abdomen, and abnormal adhesions were noted between the distal and mid ileum, with resulting mesenteric narrowing. Attempted mesenteric widening by dissection of the peritoneum overlying the adhesions failed, because of abnormal, taut mesenteric vessels. Subsequent resection of the involved segment cured the patient. Recurrent obstructive symptoms in an infant can be an atypical presentation of segmental volvulus, and segmental volvulus should be included in the differential diagnosis of such cases. [ABSTRACT FROM AUTHOR]

Hypertrophic pyloric stenosis (HPS) is one of the most common gastrointestinal disorders that require surgical intervention in early infancy. Clinical presentation, diagnosis, and treatment have been well established in typical cases. We herein describe an unusual case of early presenting HPS with transient postoperative feeding intolerance after complete myotomy in a preterm newborn. An ex-31-week preterm newborn (birth weight, 1760 g) with persistent feeding intolerance since the first feeding was referred for surgery on the 7th day of life. Abdominal ultrasonography (USG) revealed an elongated pyloric channel (11 mm) with thickened pyloric muscle (2.1 mm). Laparotomy was performed and confirmed suspected HPS. Pylomyotomy was successful, but feeding intolerance persisted until postoperative day 12. USG on postoperative day 12 noted antroduodenal dysmotility, and transpyloric tube feeding was started. Full feeds were reached 8 days after tube feeding, which was then converted to full oral feeding. HPS should be considered in patients who show feeding intolerance shortly after birth. USG criteria for HPS in preterm infants has not yet been established. Transient antroduodenal dysmotility could be a reason for persistent feeding intolerance after complete myotomy in preterm infants, and transpyloric tube feeding could be a treatment in such cases. [ABSTRACT FROM AUTHOR]

Primary intestinal volvulus in a fetus without an underlying cause has been rarely reported. We report a case of fetal primary segmental volvulus of the ileum. A 27-year-old woman was referred at 32 weeks of gestation for evaluation of decreased fetal movement. A fetal ultrasound detected a single, significantly-dilated bowel loop without a vascular supply (coffee bean sign), and a fetal MRI revealed the ‘whirl-pool sign’. An emergency cesarean delivery and laparotomy revealed segmental volvulus of the ileum without any causative factors. Segmental resection resolved the condition without sequelae. Fetal primary segmental volvulus can occur without any underlying pathology, and rapid diagnosis and prompt intervention is mandatory for a good outcome. [ABSTRACT FROM AUTHOR]

Ultrashort-segment Hirschsprung's disease (USHD) is a rare variant of HD that is controversial in terms of diagnostic criteria and acceptable therapy. Idiopathic megarectum, also known as an intractable idiopathic constipation, is a functional disorder, which ordinarily is not surgically treated. Reported herein is a case of USHD complicated by a megarectum. The patient was an 8-year old male who presented with long history of constipation and abdominal distension. Contrast enema revealed massive dilatation of rectum, with no discernible transition zone. Absence of ganglion cells in a full-thickness rectal biopsy taken 2 cm proximal to the dentate line confirmed a diagnosis of USHD. The entire length of dilated bowel (delineated on preoperative contrast enema) was resected, using a Soave type transanal approach, with pull-through of ganglionated normal-caliber colon. In instances of USHD where megarectum prohibits use of technically less rigorous procedures (i.e., Duhamel pull-through or internal sphincter myectomy), a Soave type transanal pull-through may be a reasonable alternative. [ABSTRACT FROM AUTHOR]

Abstract: Ingestion of multiple magnets poses a particular risk for various intraabdominal complications in children. We herein report a case of ingestion of multiple magnets, of which 3 were spontaneously expelled, and the remaining magnets were surgically removed. Since the total amount of ingestion was unknown upon presentation and the remaining intraabdominal magnets failed to pass after 24 h, emergency surgery was performed. Two magnets sandwiched the bowel walls and formed a jejunoileal fistula. There was no peritoneal contamination. We found that not all the ingested multiple magnets attracted each other, and multiple magnets could appear as single material on a plain radiograph. Confirming the exact count of ingested magnets is important; if the count is in doubt or two or more attachments are evident, prompt surgical intervention is warranted. [Copyright &y& Elsevier]

Abstract: Reoperation for a malfunctioning central venous catheter (CVC) is technically demanding as a bedside procedure in premature infants, and general anesthesia and paralysis is usually required for such an operation. Herein, we describe a safe and simple reinsertion technique to treat CVC occlusion using the original tract. Using a previous incision, the subcutaneous tract was identified, and the anterior half circle of the tract was opened. The catheter was transected through the opening, and the distal part was removed. A new catheter was introduced through an empty subcutaneous tunneling pathway and skin exit site, where the old distal part had been recently removed. Using fine 6–0 monofilament suture, traction sutures were placed along the cut edge of the proximal tract. The proximal part was then removed, and a newly placed catheter tip was inserted into the tract opening. When CVC reinsertion is required in premature infants, reinsertion can be accomplished easily and safely using this technique as a bedside procedure without general anesthesia. [Copyright &y& Elsevier]

Abstract: The diagnosis of a thyroglossal duct cyst is often apparent when it presents with typical findings. We report a case of a 6year-old girl with a low-lying thyroglossal duct cyst presenting with recurrent lateral cervical discharge without palpable mass, thus mimicking a second branchial cleft fistula. CT scan differentiated the lesion from a second branchial cleft fistula, and a standard Sistrunk procedure cured the lesion. In patients who show lateral cervical discharge without definite mass, therefore mimicking a second branchial cleft fistula, a low-lying thyroglossal duct cyst should be considered. [Copyright &y& Elsevier]

Abstract: Massive bleeding from the porta hepatis is a rare complication after a Kasai portoenterostomy and usually requires urgent surgical intervention. Among the causes of the porta hepatis bleeding, bleeding from the injured caudate arterial branches has never been reported. Here, we describe 2 cases of caudate arterial branch bleeding after a Kasai portoenterostomy, which were successfully treated with transcatheter arterial embolization without laparotomy. [Copyright &y& Elsevier]

Abstract: Vasoactive intestinal polypeptide (VIP) is known to cause the watery diarrhea, hypokalemia, and achlorhydria syndrome. A 14-year-old girl was admitted with a 4-year history of persistent uncontrollable diarrhea and hypokalemia. Computed tomographic evaluation of the neck, chest, and abdomen were normal. Numerous polyps covering the entire colon and rectum were noted on colonoscopy. The serum VIP level was 143 pg/mL. The patient underwent a total proctocolectomy with an ileal-J-pouch. The pathologic examination revealed ganglioneuromatosis. Postoperatively, the symptoms resolved, and the serum VIP level fell to lower than 5 pg/mL. This is an unusual case of the watery diarrhea, hypokalemia, and achlorhydria syndrome caused by ganglioneuromatosis of the entire colon and rectum. [Copyright &y& Elsevier]

Abstract: Choristoma is a tissue or mass with a normal histology at an abnormal location, and cystic choristoma has rarely been reported in the head and neck region. Cervical cystic masses in neonates are usually diagnosed as cystic lymphangioma. The authors present a case of a congenital laterocervical cystic mass that appeared to be lymphangioma, but which turned out to be a cystic choristoma. [Copyright &y& Elsevier]