Rare Pediatric Cecal Anomalies: A Lesson in Pediatric Surgery.

BACKGROUND: Rare incidence cases are part of the routine work of pediatric surgeons. Cecal anomalies in children are an example of such cases. Objectives: To describe the presentation, workup, management and outcome of rare cecal anomalies in children and to analyze the skills needed for their successful treatment. METHODS: A retrospective chart review was conducted of all cases of cecal anomalies managed by the pediatric surgical service at a tertiary hospital from June 2017 to January 2020. Data regarding demographics, clinical presentation, radiological studies, surgical treatment, pathology, complications, and outcome were collected. RESULTS: Five cases of cecal anomalies were encountered over a period of 32 months, including a cecal volvulus, cecal duplication, cecal intussusception, and two cecal masses (one ulcerated lipoma and one polyp). All patients, except the patient with cecal duplication, presented acutely and were managed surgically. Long-term follow-up of 17-24 months was unremarkable in all cases. CONCLUSIONS: A wide knowledge base, careful judgment, and creativity enable pediatric surgeons to successfully treat rare conditions such as rare cecal anomalies. These skills should be part of the education of pediatric surgery trainees.

Asymptomatic left isomerism with preduodenal portal vein: computed tomography appearance.

Left isomerism (polysplenia), one of the two major variants of heterotaxia with right isomerism (asplenia), may be rarely diagnosed in adulthood. Most cases are nevertheless asymptomatic and incidentally detected during imaging or surgery performed for unrelated conditions. We hereby report a case of left isomerism fortuitously diagnosed in a 55-year-old man with unrelated tachy-cardiomyopathy. Thoraco-abdominal computed tomography revealed a typical preduodenal portal vein (PDPV) associated with a large series of other occult anatomic variations comprising: polysplenia, agenesis of both pancreatic body and tail, complete non-rotation of the bowel and finally azygous continuation of the inferior vena cava. Subtle but highly specific thoracic features of left isomerism were also found with a bilobed right lung and bilateral long hyparterial main bronchi. The features of adult left isomerism are remembered with special attention to the PDPV.

Galectin-3 in septic acute kidney injury: a translational study.

BACKGROUND: Galectin-3 (Gal-3) is a pleiotropic glycan-binding protein shown to be involved in sepsis and acute kidney injury (AKI). However, its role has never been elucidated in sepsis-associated AKI (S-AKI). We aimed to explore Gal-3's role and its potential utility as a therapeutic target in S-AKI. METHODS: In 57 patients admitted to the intensive care unit (ICU) with sepsis, serum Gal-3 was examined as a predictor of ICU mortality and development of AKI. In a rat model of S-AKI induced by cecal ligation and puncture (CLP), 7-day mortality and serum Gal-3, Interleukin-6 (IL-6), and creatinine were examined at 2, 8, and 24 hours (h) post-CLP. Two experimental groups received the Gal-3 inhibitor modified citrus pectin (P-MCP) at 400 mg/kg/day and 1200 mg/kg/day, while the control group received water only (n = 18 in each group). RESULTS: Among 57 patients, 27 developed AKI and 8 died in the ICU. Serum Gal-3 was an independent predictor of AKI (OR = 1.2 [95% CI 1.1-1.4], p = 0.01) and ICU mortality (OR = 1.4 [95% CI 1.1-2.2], p = 0.04) before and after controlling for age, AKI, and acute physiology and chronic health evaluation (APACHE II) score. In the CLP rat experiment, serum Gal-3 peaked earlier than IL-6. Serum Gal-3 was significantly lower in both P-MCP groups compared to control at 2 h post-CLP (400 mg: p = 0.003; 1200 mg: p = 0.002), and IL-6 was significantly lower in both P-MCP groups at all time points with a maximum difference at 24 h post-CLP (400 mg: p = 0.015; 1200 mg: p = 0.02). In the Gal-3 inhibitor groups, 7-day mortality was significantly reduced from 61% in the control group to 28% (400 mg P-MCP: p = 0.03) and 22% (1200 mg P-MCP: p = 0.001). Rates of AKI per RIFLE criteria were significantly reduced from 89% in the control group to 44% in both P-MCP groups (400 mg: p = 0.007; 1200 mg: p = 0.007). CONCLUSIONS: This translational study demonstrates the importance of Gal-3 in the pathogenesis of S-AKI, and its potential utility as a therapeutic target.

Murine sepsis phenotypes and differential treatment effects in a randomized trial of prompt antibiotics and fluids.

BACKGROUND: Clinical and biologic phenotypes of sepsis are proposed in human studies, yet it is unknown whether prognostic or drug response phenotypes are present in animal models of sepsis. Using a biotelemetry-enhanced, murine cecal ligation and puncture (CLP) model, we determined phenotypes of polymicrobial sepsis prior to physiologic deterioration, and the association between phenotypes and outcome in a randomized trial of prompt or delayed antibiotics and fluids. METHODS: We performed a secondary analysis of male C57BL/6J mice in two observational cohorts and two randomized, laboratory animal experimental trials. In cohort 1, mice (n = 118) underwent biotelemetry-enhanced CLP, and we applied latent class mixed models to determine optimal number of phenotypes using clinical data collected between injury and physiologic deterioration. In cohort 2 (N = 73 mice), inflammatory cytokines measured at 24 h after deterioration were explored by phenotype. In a subset of 46 mice enrolled in two trials from cohort 1, we tested the association of phenotypes with the response to immediate (0 h) vs. delayed (2 to 4 h) antibiotics or fluids initiated after physiologic deterioration. RESULTS: Latent class mixture modeling derived a two-class model in cohort 1. Class 2 (N = 97) demonstrated a shorter time to deterioration (mean SD 7.3 (0.9) vs. 9.7 (3.2) h, p < 0.001) and lower heart rate at 7 h after injury (mean (SD) 564 (55) vs. 626 (35) beats per minute, p < 0.001). Overall mortality was similar between phenotypes (p = 0.75). In cohort 2 used for biomarker measurement, class 2 mice had greater plasma concentrations of IL6 and IL10 at 24 h after CLP (p = 0.05). In pilot randomized trials, the effects of sepsis treatment (immediate vs. delayed antibiotics) differed by phenotype (p = 0.03), with immediate treatment associated with greater survival in class 2 mice only. Similar differential treatment effect by class was observed in the trial of immediate vs. delayed fluids (p = 0.02). CONCLUSIONS: We identified two sepsis phenotypes in a murine cecal ligation and puncture model, one of which is characterized by faster deterioration and more severe inflammation. Response to treatment in a randomized trial of immediate versus delayed antibiotics and fluids differed on the basis of phenotype.

Caecal duplication cyst leading to intussusception in an adult.

Colonic duplication cyst is an uncommon cause of intussusception in adults. We report a case of caecal duplication cyst in a 24-year-old adult with a 3-year history of recurrent abdominal pain mainly in the periumbilical region. CT and MRI of the abdomen showed a lesion suggesting an caecal duplication cyst within an intussuception. The patient underwent diagnostic laparoscopy and was found to have a caecal duplication cyst acting as a lead point for the intusseception for which laparoscopic-assisted resection was performed. The histological examination confirmed the presence of intestinal duplication cyst without any underlying malignancy.

Congenital colonic stenosis with absent caecum and appendix: a rare association.

Congenital colonic stenosis is a rare condition with less than 20 cases reported in the literature since 1966. We report an interesting case of a 7-month-old baby girl who presented with features suggestive of acute intestinal obstruction. On exploration, it was a case of ascending colon stenosis with absence of caecum and appendix. A double barrel ileocolostomy was performed. The histopathology confirmed the diagnosis of colonic stenosis and ruled out the presence of Hirschsprung's disease in the distal colon. The child underwent second stage surgery (stoma closure) after 9 months. In complex cases of congenital colonic stenosis, an early decompressive surgery followed by a delayed second stage closure is recommended in patients with poor general condition.

Meconium peritonitis due to fetal appendiceal perforation: two case reports and a brief review of the literature.

BACKGROUND: Meconium peritonitis is an infrequent congenital disease usually caused by perforation of the fetal digestive tract. Meconium peritonitis resulting from intrauterine appendiceal perforation has been rarely reported and is often overlooked during pregnancy. We herein report two cases of fetal appendiceal perforation. CASE PRESENTATION: Two neonates were found to have intestinal distension and gradually increasing ascites antenatally. After birth, diagnostic abdominal punctures revealed meconium peritonitis. Urgent surgery showed both neonates had developed gangrenous appendicitis in utero. Pathological examination supported the diagnosis of fetal appendiceal perforation in both neonates, and one also had deformity of cecal duplication. In the present report, we also review the presentation, diagnosis, pathology, management, and recent literature of fetal appendiceal perforation. CONCLUSION: Meconium peritonitis due to fetal appendiceal perforation is extremely rare, and preoperative diagnosis is almost impossible. However, clinicians should be aware of abnormal gastrointestinal manifestations in the fetus during the antenatal examination. For neonates with severe meconium peritonitis, an early operation with careful intraoperative exploration is necessary.

A Case of Parasitic Twinning or Caudal Duplication in a Dog.

A 6 mo old 13.5 kg (29.7 lb) male intact American Staffordshire terrier was evaluated for a history of supernumerary pelvic limbs, with malodorous discharge from a supernumerary penis. Imaging (radiographs, abdominal ultrasound, and computed tomography with excretory urogram) showed a supernumerary pelvis with associated pelvic limbs, no osseous continuity with the primary spinal column, a colonic diverticulum extending to the supernumerary pelvis, an enlarged left kidney with a ureter connecting to a single bladder, right renal aplasia, a single descended testicle in the primary scrotum, an intra-abdominal cryptorchid testicle, and two unidentifiable soft tissue masses. At surgery, a single ileum was present with a primary and accessory cecum and colon and the accessory colon entering the supernumerary pelvis. The accessory cecum and colon, right kidney, two unknown soft tissue masses, and the single descended testicle were removed. The right kidney had a ureter that anastomosed with the accessory colon at its entry into the supernumerary pelvis. The supernumerary pelvis and hind limbs were not removed. Five months after surgery, the dog was reported to be doing well clinically. Caudal duplication is extremely rare in veterinary medicine. The appearance of supernumerary external structures may indicate internal connections as well.

Caecal duplication cyst: a rare cause of neonatal intestinal obstruction.

We report a rare case of duplication cyst of the caecum responsible for an intestinal obstruction in a 5-day-old newborn. Preoperatively, we suspected the diagnosis of enteric duplication as ultrasonography demonstrated a cystic mass in the right iliac fossa, and laparotomy confirmed an obstructive caecal mass which was resected and an end-to-end anastomosis was performed. The neonate completely recovered with an uneventful follow-up.

Intracecal appendix: an extremely rare anatomical variation. A case report and review of literature.

Appendicitis is the most common surgical abdominal emergency in the developed world. Most of the surprises encountered during an appendectomy are usually due to the various positions of the appendix tip. Anatomical variations are an extremely rare phenomenon, with only a few case reports scattered among volumes of literature on the vermiform appendix. A new variation is described in which the appendix was intracecal and cause for surprise during surgery. A review of literature of rare anatomical variations of the appendix is also presented.

[A rare case of coecal volvulus after colonoskopy due to a mobile coekum - diagnosis, surgical therapy and postoperative complications]. / Ein seltener Fall eines Volvulus des Coecums nach Koloskopie bei Coecum mobile ­ Darstellung von Diagnostik, operativer Therapie und postoperativen Komplikationen.

Colonoscopy, either performed as screening or as a therapeutic proceedure, is, in general, very safe with only a few cases of serious complications. Most cases of bleeding after endoscopic polypectomy can be safely managed endoscopically. The rare cases of colonic perforations still have to be managed by surgical intervention. The postpolypectomy-coagulation syndrome and a cecal volvulus are very rare complicatoions after colonoscopy. In the current manuscript, we describe a rare case of a cecal volvulus after routine colonoscopy due to an unknown mobile coecum as a predisposition. We discuss the endoscopic, clinical and radiological findings of the patient. Moreover, we describe the performed surgical procedure and the further clinical course of the patient. A cecal volvulus should always be considered as a possible rare, but serious, complication in the differential diagnosis of abdominal pain after colonoscopy. The standard therapy of a cecal volvulus is the right hemicolectomy. As an alternative, a coecopexy without resection could be performed as long as the colonic wall is still vital.

Laparoscopic Management of Mobile Cecum.

BACKGROUND AND OBJECTIVES: The mobile cecum is an embryologic abnormality and has been associated with functional colon disease (chronic constipation and irritable bowel syndrome). However, unlike functional disease, the primary treatment is operative, using laparoscopic cecopexy. We compare the epidemiology and pathophysiology of mobile cecum syndrome and functional colon disease and propose diagnostic and treatment guidelines. METHOD: This study was a case-control series of 15 patients who underwent laparoscopic cecopexy. Age, gender, recurrent abdominal pain, and constipation based on Rome III criteria were assessed. Ileocecal-appendiceal unit displacement was graded as follows: I (cecum retroperitoneal or with little mobility); II (wide mobility, crossing the midline); and III (maximum mobility, reaching the left abdomen). Patients with Grades II and III underwent laparoscopic cecopexy. The clinical outcomes were evaluated according to modified Visick's criteria, and postoperative complications were assessed according to the Clavien-Dindo classification. RESULTS: The mean age was 31.86 ± 12.02 years, and 13 patients (86.7%) were women. Symptoms of constipation and abdominal pain were present in 14 (93.3%) and 11 (73.3%), respectively. Computed tomography was performed in 8 (53.3%) patients. The mean operative time was 41 ± 6.66 min. There were no postoperative infections. One (7.8%) patient was classified as Clavien Dindo IIIb and all patients were classified as Visick 1 or 2. CONCLUSION: Many patients with clinical and epidemiological features of functional colon disease in common in fact have an anatomic anomaly, for which the treatment of choice is laparoscopic cecopexy. New protocols should be developed to support this recommendation.

[Is intestinal malrotation the cause of intussusception? Waugh's syndrome, a case report]. / ¿Es la malrotación intestinal el agente causal de la invaginación intestinal? Síndrome de Waugh, reporte de un caso.

BACKGROUND: Waugh's syndrome is the combination of intussusception and intestinal malrotation. The first case was described in 1911, with few cases being reported since then. CLINICAL CASE: A 7 month-old infant with fever, vomiting and rectal bleeding. Distension and an abdominal mass was found on examination. An exploratory laparotomy was preformed that found a combination of intussusception and intestinal malrotation, which was treated succesfully. CONCLUSION: We consider that this combination is not sufficiently diagnosed very often, leading to its disappearance.

Obstrucción intestinal neonatal secundaria a duplicación cecal / Neonatal intestinal obstruction due to cecal duplication

Introducción. Las duplicaciones de ciego son patologías poco frecuentes, representando el 0,4% de las duplicaciones intestinales, existiendo pocos casos publicados. Caso clínico. Presentamos un caso de una recién nacida de 48 horas de vida con vómitos biliosos y distensión abdominal; en las pruebas de imagen, se observa una masa quística de 30 mm de diámetro en flanco derecho; en la laparotomía se comprueba una tumoración quística localizada en ciego; se realiza resección ileocólica con anastomosis término-terminal. El estudio anatomopatológico confirma la existencia de un quiste de duplicación cecal. Comentarios. En pacientes con obstrucción intestinal, el enema opaco permite realizar el diagnóstico diferencial con otras patologías cólicas del periodo neonatal; cuando el quiste de duplicación cecal no se encuentra comunicado con la luz del colon, se puede observar un defecto de repleción, lo que junto con otras pruebas y la clínica, nos puede hacer sospechar duplicación cecal. Se recomienda realizar una intervención quirúrgica temprana, incluso en pacientes asintomáticos, para disminuir la morbilidad

Cecal duplications are rare, representing 0.4% of all gastrointestinal duplications, with few cases reported in the literature. A 48 hours-old newborn presented with bilious vomiting and abdominal distension; the ultrasound revealed a cystic mass in the right abdomen; ileocolic resection was performed with end to end anastomosis. Microscopy confirmed cecal duplication cyst. In patients with intestinal obstruction, contrast enema allows differential diagnosis with other colonic pathologies in the newborn; when the cecal duplication cyst is connected to the lumen of the colon, enema reveals a filling defect, and cecal duplication can be suspected. Although cystic lesions are asymptomatic, they should be excised when detected to prevent complications

Laparoscopic and Laparoscopy-Assisted Resection of Enteric Duplication Cysts in Children.

INTRODUCTION: Enteric duplication (ED) cysts are rare. The commonness of ultrasonographic investigation contributes to an earlier diagnosis of such a pathology before the onset of the first clinical symptoms. A planned mini-invasive surgical treatment during the infancy is proposed. This study presents the possibility and safety of elective laparoscopic or laparoscopy-assisted mini-invasive resection of ileal (IL) and ileocecal (IC) duplications, thus avoiding bowel resection. MATERIALS AND METHODS: A retrospective review was conducted of medical records of 6 patients at the age from 3 to 22 months with the diagnosis of ED, treated in the Department of Pediatric Surgery, Jagiellonian University Medical College, Krakow, Poland, within the period from January 2012 to September 2014. RESULTS: Excision of cysts without bowel resection was performed in five children with IC and IL duplications. Laparoscopic excision was performed in two children with IC duplication; in the other three children (1 IC and 2 IL duplication), laparoscopy confirmed the diagnosis with consecutive cyst excision without bowel resection after external evacuation of the cyst. The external resection of the cecum and Bauhin's valve was necessary in 1 patient with large IC duplication and malrotation. The postoperative course was satisfactory in all the cases. CONCLUSIONS: The laparoscopic approach allows for confirming the diagnosis and accurately defining the exact site of duplication, as well as for effective and safe mini-invasive treatment. Laparoscopic or laparoscopy-assisted excision of ED without bowel resection is a safe option in a significant number of IL and IC duplications.

Left-sided gastroschisis with caecal agenesis: A rare case report.

Gastroschisis is a congenital anomaly characterised by a defect in the anterior abdominal wall through which the intestinal contents freely protrude. Defect is located almost always to right of umbilicus. To our knowledge very few cases of left-sided gastroschisis have occurred and presented in literature. We report case of left-sided gastroschisis with caecal agenesis, short gut, and malrotation of intestine.

Fryns anophthalmia-plus syndrome: two rare cases.

Fryns anophthalmia-plus syndrome is a rare syndrome with clinical diversity primarily including anophthalmia/microphthalmia, facial clefts, cleft lip/palate, ear and nasal deformities. Here we present two different cases of APS with anopthalmia/microphthalmia, cleft palate, low set ears, ventriculomegaly and one of which had intestinal non-fixation anomaly not described in the literature before.