Carl-Christian Jackson

Management of pediatric patients with refractory constipation who fail cecostomy

BACKGROUND Antegrade continence enema (ACE) is a recognized therapeutic option in the management of pediatric refractory constipation. Data on the long-term outcome of patients who fail to improve after an ACE-procedure are lacking. PURPOSE To describe the rate of ACE bowel management failure in pediatric refractory constipation, and the management and long term outcome of these patients. METHODS Retrospective analysis of a cohort of patients that underwent ACE-procedure and had at least 3-year-follow-up. Detailed analysis of subsequent treatment and outcome of those patients with a poor functional outcome was performed. RESULTS 76 patients were included. 12 (16%) failed successful bowel management after ACE requiring additional intervention. Mean follow-up was 66.3 (range 35-95 months) after ACE-procedure. Colonic motility studies demonstrated colonic neuropathy in 7 patients (58%); abnormal motility in 4 patients (33%), and abnormal left-sided colonic motility in 1 patient (9%). All 12 patients were ultimately treated surgically. Nine patients (75%) had marked clinical improvement, whereas 3 patients (25%) continued to have poor function issues at long term follow-up. CONCLUSIONS Colonic resection, either segmental or total, led to improvement or resolution of symptoms in the majority of patients who failed cecostomy. However, this is a complex and heterogeneous group and some patients will have continued issues.

Laparoscopic-Assisted Colostomy in Children

INTRODUCTION Colostomy morbidity has been reported to be as high as 50%. Laparoscopic-assisted colostomy (LAC) is associated with decreased colostomy complication. LAC is recommended for stoma formation in adults but has not been previously reported in children. In this paper, we report on our initial experience with LAC in children. MATERIALS AND METHODS Using a two- to four-port (3.5-mm) technique, LAC was performed in a female with an imperforate anus and 2 male patients with complicated Hirschsprungs disease (HD), respectively. Data collected included operative time, time to recover bowel function, and morbidity. Close follow-up was done until stoma closure. RESULTS The operative time was 144 minutes in the HD patients (including concomitant laparoscopic biopsies and a leveling colostomy) and 40 minutes in the imperforate anus patient. Median time to passage of both flatus and stool was 40 hours (range, 24-48). Time to commence feeds postop was 40 hours (range, 24-48). The median time of follow-up was 3 months (range, 2-9) until the stoma was taken down. No complications have occurred to date. CONCLUSIONS LAC is safe and easily performed in neonates and infants. It facilitates accurate stoma placement and orientation. It allows additional bowel mobilization, especially in HD. In accordance with the adult experience, LAC seems to obviate stoma-related complications. Encouraged by our initial low morbidity rate, a prospective evaluation of this technique is planned.

Spontaneous hemothorax in multiple exostoses: a case report and review of literature.

Abstract Multiple hereditary exostoses is a rare autosomal dominant disorder characterized by the growth of multiple osteochondromas. We describe the thoracoscopic remodeling of a spiculated costal exostotic lesion responsible for spontaneous recurrent hemothoraces in a 17-year-old male patient with multiple hereditary exostoses.

An 11-Year-Old Boy With Sudden-Onset Abdominal Pain

Diagnosis: Anisakiasis The pathology of the omental mass showed an inflammatory mass with dense eosinophilic granulomatous inflammation and presence of a helminth in the center with morphology consistent with an Anisakis species larva. More specifically, the crosssections of the larva showed presence of the 2 distinct Y-shaped lateral epidermal cords, a thick muscle layer (polymyarian muscle cells), and ventrally located excretory gland cells (renette cells) (Figures 1 and 2) [1]. Anisakiasis is a zoonotic roundworm infection caused by Anisakis simplex, Anisakis physeteris, or Pseudoterranova species [2]. It is also known as “sushi worm” or “herring worm.” Salmon, herring, cod, mackerel, and squid can transmit Anisakis species. In the United States, the majority of cases are due to ingestion of the Pacific salmon Oncorhynchus species [3, 4]. The incidence of anisakiasis seems to be increasing worldwide, partially due to increased consumption of raw or undercooked fish [4]. It is most commonly seen in Japan. The frequency in the United States is unknown, because the disease often goes undetected and is not reportable [5]. After ingestion, the larva (usually 1 or 2) penetrates the human gastric or intestinal mucosa. The dying parasite induces an inflammatory reaction and a tissue abscess develops with predominance of eosinophils. Gastric anisakiasis usually develops 1–8 hours after ingestion of raw fish and is characterized by acute epigastric pain, nausea, and vomiting [3]. Intestinal anisakiasis usually develops 5–7 days following ingestion of raw fish and may be associated with severe abdominal pain, abdominal distension, and a palpable inflammatory mass that can occasionally cause intestinal obstruction [2]. Diarrhea with blood or mucus may also develop. A syndrome mimicking appendicitis may be observed if the ileocecal region is involved. Anisakis larvae occasionally penetrate into the peritoneal cavity or other visceral organs and cause eosinophilic granulomas [3], as in our case. Anisakiasis is easily misdiagnosed as acute appendicitis, Crohn’s disease, gastric ulcer, or gastrointestinal cancer [4]. Other parasites that can cause similar symptoms include Ascaris, Enterobius, Strongyloides, and T. trichiura or, less commonly, Angiostrongylus costaricensis, Ancylostoma caninum, and Nanophyetus salmincola [2, 6].Peritoneal granulomas have been described in infections by Ascaris, Enterobius, and A. costaricensis [7–9]. Symptoms diminish spontaneously in most patients without specific therapy. In gastric anisakiasis, resolution of symptoms is expedited by endoscopic removal of the larvae. Patients with intestinal or extraintestinal infections often undergo surgery, for possible acute abdomen, as in our case. It seems though, that Figure 1. Pathology of the omental mass (hematoxylin and eosin stain, ×100 magnification). In the center there is presence of a helminth larva with typical morphology of Anisakis, with presence of polymyarian muscle cells (long black arrow), 2 Y-shaped lateral epidermal cords (short black arrows), and intestine (star). There is presence of dense eosinophilic infiltration surrounding the larva. Figure 2. Pathology of the omental mass (hematoxylin and eosin stain, ×200 magnification). There is presence of polymyarian muscle cells (long black arrow) and intestine (star), as well as excretory cells (renette cells), which are banana shaped and ventral to the intestine (short black arrow).

Management of patients with combined tracheoesophageal fistula, esophageal atresia, and duodenal atresia

INTRODUCTION Patients with combined esophageal atresia (EA), tracheoesophageal fistula (TEF), and duodenal atresia (DA) pose a rare management challenge. PRESENTATION OF CASE Three patients with combined esophageal atresia (EA), tracheoesophageal fistula (TEF), and duodenal atresia safely underwent a staged approach inserting a gastrostomy tube and repairing the EA/TEF first followed by a duodenoduodenostomy within one week. None of the patients suffered significant pre- or post-operative complications and our follow-up data (between 12 and 24 months) suggest that all patients eventually outgrow their reflux and respiratory symptoms. DISCUSSION While some authors support repair of all defects in one surgery, we recommend a staged approach. A gastrostomy tube is placed first for gastric decompression before TEF ligation and EA repair can be safely undertaken. The repair of the DA can then be performed within 3-7 days under controlled circumstances. CONCLUSION A staged approach of inserting a gastrostomy tube and repairing the EA/TEF first followed by a duodenoduodenostomy within one week resulted in excellent outcomes.

Aortopexy for Innominate Artery Compression of the Trachea

Tracheomalacia is a rare congenital condition that results from improper formation of the cartilaginous tracheal rings, but is often seen in children who have esophageal atresia with/without trachea-esophageal fistula. This causes the trachea, which is the main airway, to be floppy, and as the child grows, can result in breathing difficulties. These difficulties are usually manifested as breathing noises that may change with position and improve during sleep, or that get worse with coughing, crying, or feeding. Upper respiratory infections can also be more common. While most cases of tracheomalacia resolve by 18 to 24 months of age, a small percentage either continue or cause such severe breathing or feeding issues that surgical intervention is warranted. In cases where the innominate artery is the cause of compression of the weakened trachea, an aortopexy to elevate the vessel up to the sternum and away from the trachea is performed.