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Apple peel atresia

Radović V Saša, Lazovic Ranko, Crnogorac Snezana, Banjac Lidija, Suhih Djordje
BACKGROUND: Embryologically, duodenal atresia results from inadequate recanalisation and proliferation of gut epithelius in the 6th week of gestation, while apple-pee atresia of small bowel is a consequence of a vascular accident in subsequent embryonic development, and the two are rather rarely manifested as a joint clinical entity. CASE PRESENTATION: We present here a 29 week preterm boy admitted to the intensive care unit due to breathing difficulties and low birthweight...
2016: BMC Pediatrics
Isabel Filges, Elisabeth Bruder, Kristin Brandal, Stephanie Meier, Dag Erik Undlien, Trine Rygvold Waage, Irene Hoesli, Max Schubach, Tjaart de Beer, Ying Sheng, Sylvia Hoeller, Sven Schulzke, Oddveig Røsby, Peter Miny, Sevgi Tercanli, Truls Oppedal, Peter Meyer, Kaja Kristine Selmer, Petter Strømme
Strømme syndrome was first described by Strømme et al. (1993) in siblings presenting with "apple peel" type intestinal atresia, ocular anomalies and microcephaly. The etiology remains unknown to date. We describe the long-term clinical follow-up data for the original pair of siblings as well as two previously unreported siblings with a severe phenotype overlapping that of the Strømme syndrome including fetal autopsy results. Using family-based whole-exome sequencing, we identified truncating mutations in the centrosome gene CENPF in the two nonconsanguineous Caucasian sibling pairs...
April 2016: Human Mutation
Sunil Jaiman, Sirisha Rao Gundabattula, Chinmayee Ratha
Apple peel deformity is a rare form of upper intestinal atresia of unknown etiology. Umbilical cord ulcers can occur secondary to reflux of gastric juice and bile as a result of the atresia and can cause lethal intrauterine hemorrhage. The authors report 3 instances of congenital apple peel jejunal atresia with helical umbilical cord ulcers afflicting all female offspring in consecutive pregnancies in a single nonconsanguineous family. There was no hemorrhage from the cord ulcers, but all 3 pregnancies resulted in perinatal death...
January 2016: Pediatric and Developmental Pathology
V Insinga, M Pensabene, M Giuffrè, M Busè, M Cimador, G Corsello, F Siracusa
Intestinal atresia type III B (apple peel) and gastroschisis are both congenital malformations who require early surgical correction in neonatal age. Their association is very rare. We present the case of a full term infant with partial apple peel ileal atresia and a small defect of the anterior abdominal wall, complicated by in utero intestinal perforation and subsequent meconial peritonitis. We observed a partial atresia of small intestine, with involvement of terminal ileus savings of jejunum and a large part of the proximal ileum, small anterior abdominal wall defect with herniation of few bowel loops, intestinal malrotation...
May 2014: La Pediatria Medica e Chirurgica: Medical and Surgical Pediatrics
Manish Pathak, Dipanshu Narula
Classically, embryology of duodenal atresia has been linked to defect in recanalization process, while apple-peal atresia of small bowel has been considered as due to vascular accident during embryonic life. We present a 33 week preterm neonate with duodenal atresia with the apple-peal appearance of proximal jejunum for which resection of the jejunum with apple-peal configuration, plication of the duodenum, and duodono-jejunal anastomosis was done. Thus, this rare case of ours questions the embryology of duodenal atresia with the apple-peal appearance suggesting it to be due to a vascular disruption phenomenon during embryonic life...
April 2014: Journal of Clinical Neonatology
I J Broekaert, S van Koningsbruggen-Rietschel, E Rietschel
Apple peel atresia is a special form of intestinal atresia with absence of mesentery. It is most likely due to an intrauterine intestinal vascular accident and has been described with other anomalies. Meconium ileus can compromise blood supply causing intestinal atresia. Therefore, cystic fibrosis needs to be ruled out in apple peel syndrome.
January 2014: Klinische Pädiatrie
Luciano Silveira Onofre, Renato Frota de Albuquerque Maranhão, Elaine Cristina Soares Martins, Camila Girardi Fachin, Jose Luiz Martins
Apple-peel atresia (or Type-IIIb intestinal atresia) is an unusual type of jejunoileal atresia. They present with jejunal atresia near the ligament of Treitz and a foreshortened small bowel. Many surgical options have been used, but the optimal method of repair remains unclear. We present a case of a newborn with apple-peel intestinal atresia managed by enteroplasty for intestinal lengthening and primary anastomosis.
June 2013: Journal of Pediatric Surgery
R Balanescu, L Topor, I Stoica, A Moga
Multiple intestinal atresia (MIA) is a complex congenital defect which represents a challenge for the pediatric surgeon,especially in the rare event of encountering type IIIb or apple peel atresia, which has a high mortality rate. The surgeon’s aim is to preserve as much bowel length as possible, to avoid postoperative sepsis and to prevent long-term complications such as short bowel syndrome. Access to a good neonatal intensive care unit and to parenteral nutritional support is crucial in the survival of these children...
May 2013: Chirurgia
Khalid Khattala, Youssef Bouabdallah
No abstract text is available yet for this article.
2012: Pan African Medical Journal
Nathaly Llore, Sandra Tomita
Congenital mesenteric defects are rare causes of bowel obstruction. Even rarer are mesenteric defects with an apple peel type of deformity, probably described definitively only once previously. We present a case of a 3 year old boy who presented with a septic-like picture of severe metabolic acidosis and lethargy from a bowel obstruction with bowel ischemia. At laparotomy he was found to have bowel infarction due to herniation through a congenital mesenteric defect with an apple peel type of deformity of the bowel without bowel atresia...
January 2013: Journal of Pediatric Surgery
Si Hak Lee, Yong Hoon Cho, Hae Young Kim, Jae Hong Park, Shin Yun Byun
PURPOSE: Small intestinal atresia is relatively common anomaly that causes intestinal obstruction in neonates. Although surgical interventions are usually successful, critical problems could raise in certain cases. This study aimed to identify the distinct clinical characteristics of complex cases of jejunal atresia by retrospective analysis. METHODS: Overall, 91 cases of small intestinal atresia, which occurred in infants between 2001 and 2010 at Pusan National University Children's Hospital, were reviewed retrospectively...
November 2012: Pediatric Surgery International
Ashok Yadavrao Kshirsagar, Sushma Rajaram Desai, Mayur Anant Nagvekar, Gaurav Om Prakash Vasisth
Apple-peel atresia or Type 3 jejuno-ileal atresia (JIA) is an uncommon cause of foetal intestinal obstruction. Bowel obstruction in the foetus is diagnosed on the prenatal ultrasonography only in 50% cases. We report a case in which foetal intestinal obstruction was diagnosed on prenatal ultrasonography. The child showed signs of intestinal obstruction on day one after birth, for which an exploratory laparotomy was performed. Type 3 JIA was found for which resection of atretic segments with jejuno-ascending colon anastomosis was preformed...
May 2011: African Journal of Paediatric Surgery: AJPS
R T Patil, R Gupta, S V Parelkar, S Oak, B Sanghvi, A Prakash, M Bachani, P H Shimoga, S Sahoo
No abstract text is available yet for this article.
October 2011: European Journal of Pediatric Surgery
Roberto Miraglia, Pieralba Catalano, Luigi Maruzzelli, Silvia Riva, Marco Spada, Daniele Alberti, Angelo Luca, Bruno Gridelli
We report successful fluoroscopically guided balloon dilatation of an early postoperative stricture of a jejunoileal anastomosis in a 3-month-old infant who underwent serial transverse enteroplasty for type IIIb apple peel intestinal atresia.
December 2010: Journal of Pediatric Surgery
Marco Castori, Luigi Laino, Vito Briganti, Lucia Pedace, Andrea Zampini, Mario Marconi, Barbara Grammatico, Elsa Buffone, Paola Grammatico
Strømme syndrome is a rare multiple congenital malformation syndrome consisting in apple peel intestinal atresia, ocular anomalies, microcephaly and developmental delay. To date, this condition was described in a couple of sibs and 7 additional sporadic patients. We report on a 11-month-old female, who requested surgical correction for jejunal atresia shortly after birth and also presented with megalocornea and persistence of the pupillary membrane. Microcephaly and developmental delay were absent at last examination...
May 2010: European Journal of Medical Genetics
L Harper, J-L Michel, S de Napoli-Cocci, M-B Aulagne, A Maurel, S Mazouzi, D Ramful
PURPOSE: Apple-peel atresia is a rare form of bowel atresia associating proximal jejunal atresia with a typical coil-shaped distal small bowel. Many of the children suffering from this condition are still managed with initial enterostomy followed by delayed anastomosis. PATIENTS AND METHOD: Between 2000 and 2007, we managed four patients with apple-peel atresia. Three by primary anastomosis and one by initial enterostomy with delayed anastomosis. RESULTS: Total duration of total parenteral nutrition (TPN) was an average 109 days...
November 2009: Acta Chirurgica Belgica
Ashraf Ahmad, Dinesh Sarda, Prashant Joshi, Paras Kothari
According to the current understanding, duodenal atresia is considered to be a primary malformation resulting from the errors in recanalisation in early gestation. We report a rare case of duodenal atresia with apple-peel configuration of remaining small bowel with absent superior mesenteric artery in a preterm child, which indicates that in rare circumstances vascular accidents may be the underlying cause for duodenal atresia, and jejuno-ileal atresia; only second such case to be reported in the English literature...
July 2009: African Journal of Paediatric Surgery: AJPS
Enrico De Grazia, Maria Rita Di Pace, Anna Maria Caruso, Pieralba Catalano, Marcello Cimador
The authors present a previously unreported association of different types of intestinal atresia in identical low-birth-weight twins. Both babies were affected by duodenal atresia, associated in the first case with a complete mucosal duodenal membrane and in the second one with an "apple-peel" jejunal atresia. These occurrences may suggest that they were either the consequence of linkage of 2 genes or a pleiotropic expression of a single gene responsible for such rare conditions.
December 2008: Journal of Pediatric Surgery
T E Herman, M J Siegel
No abstract text is available yet for this article.
May 2008: Journal of Perinatology: Official Journal of the California Perinatal Association
Yolande van Bever, Liselotte van Hest, Roger Wolfs, Dick Tibboel, Thelma L van den Hoonaard, Saskia J Gischler
We describe another patient with the combination of apple peel intestinal atresia, microcephaly, microphthalmia, and anterior eye chamber anomalies. Development so far seems to be normal, although there is major visual impairment due to the corneal clouding. Mutation analysis of the PAX6, FOX1, PITX2, and MYNC genes was normal as was MLPA for these genes. Autosomal recessive inheritance is possible as recurrence in sibs was described, although germ line mosaicism or a microdeletion due to a very small parental translocation cannot be ruled out...
February 15, 2008: American Journal of Medical Genetics. Part A
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