Megarectum after surgery for anorectal malformations.

PURPOSE: Megarectum complicating surgery for anorectal malformation (ARM) has implications for long-term continence. Factors influencing continence and defecation include intact rectal reservoir, innervation/proprioception of the anorectal muscle complex, functioning anorectal inhibitory reflex (AIR), and intact perception at the anal margin. We studied outcomes after surgery for ARM with emphasis on megarectum; particularly as to whether altered rectal proprioception from anatomic sacrococcygeal anomalies affect incidence. We also assessed whether an abnormal AIR could trigger passive rectal dilatation without mechanical obstruction. METHODS: Eighty six infants (53 male) with ARM over 20 years were included. Demographics, surgical history, pathology, defecation patterns, imaging, manometry, and morbidity were analyzed. Incidence of sacrococcygeal malformations in children with and without megarectum was compared using Fisher exact test. Manometry results were evaluated for integrity of AIR and correlated to megarectum occurrence. RESULTS: There were 23 high/intermediate and 63 low ARMs. Fourteen (16%) developed a megarectum: 6 of 23 in high and 8 of 63 in low anomalies (P = .33). Twelve patients underwent megarectum resection at a median of 2.6 years (7 months to 10 years); 2 received bowel management protocols. Fifty-seven percent (8/14) of children with and 7% (5/72) without megarectum had sacrovertebral anomalies (P = .0001). Patients with preoperative manometry (n = 5) demonstrated an intact AIR. Colonic manometry demonstrated hyperactive colons (n = 2). Constipation was the predominant preoperative symptom; 3 patients suffered from incontinence after resection. All the specimens showed normal innervation and thickened muscularis on pathology. CONCLUSIONS: Sacral anomalies, which are more prevalent in children who developed megarectum, may result in abnormal rectal proprioception contributing to this pathology. Innervation anomalies may coexist, although preoperative manometries showed normal AIRs. Rectal dysmotility may lead to stool retention with subsequent dilatation, and patients who underwent colonic manometry had diffuse colonic hypermotility. Further physiologic and cellular studies are needed to elucidate the causes of this significant complication after surgical ARM repair in the absence of obstruction.

Anorectal malformation and Down's syndrome in monozygotic twins.

Anorectal malformation (ARM) can be divided in high, intermediate, and low forms according to the level of termination of the rectum in relation to the pubococcygeal and ischiatic lines. Patients with Down's syndrome have a high incidence of gastrointestinal anomalies, such as tracheoesophageal fistula, duodenal obstruction, annular pancreas, Hirschsprung's disease, and ARM. In these children, ARM is generally low with or without a fistula. The mode of inheritance of ARM and its genetic relation with Down's syndrome is not known, even if the association (ARM-Down's syndrome) seems not to be coincidental. We describe here a very rare case of monozygotic twins born with the association of ARM and Down's syndrome.

Obstructive neonatal respiratory distress: infected pyriform sinus cyst.

Infected lateral cervical cysts in newborn are rare. We present the case of a baby born at 41 weeks of gestation. At day 3, persistent cyanosis was noted, and a mass appeared in the left cervical region next to the sternocleidomastoid muscle. No cutaneous sinus was visible. Ultrasound imaging showed no sign of blood flow within the mass and no septae. The mass extended down to the aortic arch and pushed the trachea to the right. A cervical lymphangioma was first suspected. Puncture of the mass evacuated 80 mL of pus, and a drain was put in place. Opacification through the drain showed a tract originating from the left pyriform fossa. Preoperative laryngoscopy and catheterization of the fistula tract confirmed the diagnosis. The cyst was totally excised up to the sinus with the assistance of a guidewire inserted orally through a rigid laryngoscope. This is a rare case of an infected pyriform sinus cyst in the neonatal period.

Efficacy and impact of intravenous morphine before surgical consultation in children with right lower quadrant pain suggestive of appendicitis: a randomized controlled trial.

STUDY OBJECTIVE: The evidence supporting the use of analgesia in children with abdominal pain suggestive of appendicitis is limited. The objectives of the study are to evaluate the efficacy of morphine before surgical consultation in children presenting to the pediatric emergency department (ED) with right lower quadrant pain suggestive of appendicitis and determine whether it has an impact on the time between arrival in the ED and the surgical decision. METHODS: All children between the ages of 8 and 18 years who presented to a pediatric ED with a presumptive diagnosis of appendicitis were eligible to be enrolled in a randomized double-blind placebo-controlled trial if the initial pain was at least 5 of 10 on a verbal numeric scale. Patients received either 0.1 mg/kg of intravenous morphine (maximum 5 mg) or placebo. The primary outcomes were (1) the difference in pain using a visual analog scale at baseline and 30 minutes after the completion of the intervention, analyzed by comparing the mean pain differences for the treatment versus placebo groups; and (2) the time between arrival in the ED and the surgical decision, analyzed by comparing the median delay for the 2 groups. RESULTS: Ninety patients with a suspected diagnosis of appendicitis were randomized to receive morphine or placebo. Both groups were similar in terms of demographics, medical history, physical findings, emergency physician assessment of the probability of appendicitis, and initial pain score. There was no important difference in the decrease of pain between the morphine (n=45) and placebo (n=42) groups 30 minutes after the intervention: 24+/-23 mm and 20+/-18 mm, respectively (delta 4 mm [95% confidence interval [CI] -5 to 12 mm]). There was also no important difference in the time between arrival in the ED and the surgical decision: median 269 minutes (95% CI 240 to 355 minutes) for morphine and 307 minutes (95% CI 239 to 415 minutes) for placebo (delta -34 minutes [95% CI -105 to 40 minutes]). CONCLUSION: The use of morphine in children with a presumptive diagnosis of appendicitis did not delay the surgical decision. In our group of patients, however, morphine at a dose of 0.1 mg/kg was not more effective than placebo in diminishing their pain at 30 minutes.

Accessory liver lobe with mesothelial inclusion cysts in an omphalocele: a new malformative association.

Mesothelial inclusion cysts represent benign lesions that have been reported in a wide variety of locations. Peritoneal cysts are observed and visceral involvement has been described, notably of intraperitoneal organs such as the spleen and the testis. We report the cases of 2 neonates who underwent surgical management of an omphalocele. The hernial sac contained an accessory liver lobe, displaying in both cases multilocular mesothelial inclusion cysts. The hobnail cells lining the cysts exhibited calretinin and cytokeratin immunohistochemical reactivity, as well as focal D2-40 reactivity. One of the cases occurred in the setting of a Beckwith-Wiedemann syndrome (MIM 130650), an overgrowth disorder notably associated with omphalocele and hepatic anomalies and tumors. However, no hepatic mesothelial cyst has been described in this syndrome. In the 2nd case the omphalocele and the hepatic cysts were the sole lesions observed in the neonate. To the best of our knowledge, these 2 cases represent the first description of such an association.

Acute adenitis in children: clinical course and factors predictive of surgical drainage.

OBJECTIVES: To describe clinical course of children hospitalized for a first episode of acute unilateral infectious adenitis and to identify factors predictive of surgical lymph node drainage. METHODS: We reviewed medical records of children from 0 to 17 years of age discharged from a tertiary care pediatric center with a diagnosis of adenitis between 1 April 1996 and 31 March 2001. Patients were included if they had acute (< or = 10 days) unilateral lymph node swelling greater or equal to 2.5 cm on initial physical examination. Exclusion criteria were: bilateral adenitis or adenitis at more than one site; prior adenitis; underlying chronic disease. RESULTS: Two hundred and eighty-four patients were included in this study. The mean age was 4.0 years (3.1 SD). Twenty-three per cent of infected nodes were > 5 cm in size and 92.6% were cervical. Thirteen of 252 blood cultures were positive (5.2%), of which one showed Streptococcus pneumoniae and 12 contaminants. Mean length of stay was 4.2 days (2.2 SD). Surgical node drainage was performed in 60 (21.1%) patients. Factors significantly associated with increased risk of surgical drainage were age < 1 year (adjusted OR: 14.5; 95% CI: 5.0-42.2) and node involvement > 48 h (adjusted OR: 2.9; 95% CI: 1.2-7.2). There were no major complications. Follow-up was documented in 183 patients, of whom 92.3% achieved complete healing. CONCLUSIONS: Children hospitalized for a first episode of acute unilateral infectious adenitis generally do well. Younger patients and those with longer duration of node involvement before admission have an increased risk of surgical node drainage.

A simple technique of laparoscopic full-thickness anterior abdominal wall repair of retrosternal (Morgagni) hernias.

BACKGROUND/PURPOSE: Previous reports of laparoscopic repair of Morgagni hernias in children have involved relatively complex laparoscopic techniques. This report describes a simpler method of repair that we have applied to 4 children. METHODS: Four children with retrosternal (Morgagni) hernias underwent primary laparoscopic repair by placement of interrupted synthetic nonabsorbable sutures through the full-thickness of the anterior abdominal wall, incorporating the posterior rim of the defect and returning back out through the anterior abdominal wall, with the sutures tied in the subcutaneous tissue. RESULTS: The children, ranging in age from 11 to 36 months, underwent laparoscopic repair of their Morgagni hernias and had an uneventful postoperative recovery, apart from a port site hernia in one. CONCLUSIONS: This technique for primary laparoscopic repair of Morgagni hernia is easy to perform, well tolerated by the patient, and gives excellent cosmetic results. Laparoscopic closure of the defect by suturing the posterior rim of the hernia to the full thickness of the anterior abdominal wall would appear to provide a safe and effective means of repairing this type of hernia.