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Wilms tumor is the most common primary malignant renal tumor of childhood which usually presents between 2 and 6 years of age. Its presentation in the neonatal period is extremely rare and presenting with intestinal obstruction is perhaps unknown. We report a 2-day-old baby girl who manifested features of acute upper gastrointestinal obstruction with frequent post-feeding vomiting and abdominal distension. The initial abdominal radiograph showed abnormally displayed small bowel loops to the right hemiabdomen. Subsequent ultrasound and computed tomography scan of the abdomen detected a massive left renal mass. Left-sided nephrectomy was performed, and histopathology demonstrated left-sided Wilms tumor with favorable histology. Post-treatment yearly follow-up for 5 years recorded a disease-free, normally thriving child.

Author(s): Fogam, Michael; Leigh, Natasha; She, Trent | Abstract: Introduction: Adult intestinal malrotation with midgut volvulus is rare and most often diagnosed on abdominal imaging. Once the diagnosis is made, prompt surgical intervention is necessary. A finding suggestive of malrotation with midgut volvulus on abdominal imaging is the “whirlpool” sign where the superior mesenteric vein and superior mesenteric artery twist at the root of the abdominal mesentery. This sign was once thought to be pathognomonic, but recent studies have shown that it can be seen in asymptomatic patients.Case Report: A 20-year-old female presented to our emergency department with diffuse abdominal pain. Computed tomography demonstrated the “whirlpool” sign with a concern for malrotation with midgut volvulus. Surgical consultation was obtained and the patient was rushed to the operating room for an exploratory laparotomy. Normal mesenteric attachments were seen and no significant pathology was identified during the laparotomy. The patient was eventually diagnosed with gastritis and discharged in stable condition.Conclusion: Emergency physicians and surgeons alike should be cautious in confirming malrotation with midgut volvulus solely due to the “whirlpool” sign on abdominal imaging. Premature diagnostic closure can lead to unnecessary procedures and interventions for patients as in the case we report here.

Duodenal inversum is a rare disease not frequently encountered in clinical practice. The diagnosis is usually made late due to its rarity. Many other causes of abdominal pain like ulcer disease, pancreatitis, malrotation are mostly thought of initially and the diagnosis is usually missed. Only a few cases of duodenal inversum present with outlet obstruction. Duodenojejunostomy is perhaps the ideal management for duodenal inversum if the patient presents with outlet obstruction. Our intention is to create awareness of such a rare disease with an available definitive treatment option in the form of minimally invasive surgery. One such case of a 31-year-old man is described that was successfully managed by laparoscopic duodenojejunostomy.

The clinic, diagnosis and treatment of two cases of dolichocolon torsions are represented: a women 31 and a men 34 years old. The represented clinical observations show diagnostic difficulties encountered in patients with congenital malformations of the colon.

The feasibility of liver transplantation (LT) in adult patients with situs inversus (SI) was demonstrated with advances in surgical techniques. However, SI is very rare, and the experience of LT in adult patients with SI is very limited. We present a case of an adult patient with SI who underwent deceased-donor LT and late retransplantation because of chronic rejection. A 42-year-old man with SI totalis who suffered from acute-on-chronic hepatic failure because of hepatitis B virus-associated liver cirrhosis and alcoholic liver disease was referred to our center and underwent successful orthotopic deceased-donor whole-liver transplantation. We used a modified piggy-back technique with cavo-cavostomy and inserted a tissue expander for mechanical support of the unstably located liver graft. The patient recovered uneventfully. At 3 years after the first LT, this patient underwent retransplantation because of chronic rejection. In the second LT, we used similar surgical techniques, but performed splenectomy to make space to accommodate the second liver graft. The patient was discharged after long hospitalization. At 5 years after the second LT, he underwent living-donor kidney transplantation because of chronic renal failure developed after the second LT. Currently, he has done well for 10 years after the first LT. In conclusion, SI is a rare anomalous condition hindering LT. Careful perioperative planning with thorough assessment of the donor and recipient livers and use of patient-tailored surgical techniques can lead to successful LT.

Background Appendectomy for acute appendicitis (AA) is considered one of the most common emergency surgeries. However, emergency appendectomy accompanied with complex lesions such as extensive abscess formation is not recommended in most cases. Therefore, non-operative management followed by interval appendectomy (IA) for AA has been tried. Herein, we present three AA cases with specific etiology that underwent interval appendectomy. Case presentation Case 1: A 68-year-old man was diagnosed AA with intestinal malrotation and intra-abdominal abscesses. He initially treated with conservative therapy and underwent laparoscopic IA after detailed preoperative examination. Case 2: A 22-year-old man had been under treatment for pancolitis-type ulcerative colitis (UC), also bothered by right-lower abdominal pain several times a year. The appendix always appeared swollen on every CT taken during symptoms. He underwent laparoscopic IA; pathological finding revealed typical UC histological features in the resected appendix. After the surgery, he never suffered from terrible right lower abdominal pain. Case 3: A 69-year-old woman complaining a right lower abdominal pain had undergone CT examination, which revealed AA with appendiceal mass, irregular wall thickness of the cecum, and mediastinal and para-aortic lymph node swelling. The operation was carried out after conservative therapy. The pathological diagnosis revealed BRAF mutated colorectal carcinoma. She had received systematic chemotherapy after the surgery, and all metastatic lesions have completely disappeared. Conclusion Interval appendectomy provided us with much clearer anatomical information and precise therapeutic strategies, avoiding technical and general operative complications, and also induced fast recovery and short length of hospital stay. Interval appendectomy is a reasonable procedure and could be recommended in case of AA with some different etiology.

Highlights • Midgut volvulus caused by malrotation. • Midgut volvulus as a rare cause of acute abdomen in adulthood. • Intestinal malrotation presentation in the adulthood., Introduction Acute abdomen due to midgut volvulus with intestinal malrotation is rare event with only only few cases in the literature Butterworth et al. (2018). Intestinal malrotation presented in the adulthood is reported in 0.2−0.5 %, of cases; with only 15 % f them presenting as midgut volvulus Butterworth et al. (2018). Intestinal malrotation is casued by an alteration in embryonic development between 10–12 weeks of gestation. The main alteration is anomalous position of the bowel with the small intestine residing on the right side of the abdomen, while the colon and cecum remain on the left side due to malposition of the Treitz ligament. Additionally, the ascending colon remains attached to the abdominal wall by fibrous peritoneal bands known as Ladd bands, this being a cause of midgut volvulus and intestinal obstruction. Presentation of Case We present a 25-year-old male with failure to thrive who arrives at the ED with clinical signs and symptoms of intestinal occlusion and acute abdomen, initial resuscitation is made in ED and is transferred to OR, an exploratory laparoscopy evidencing intestinal malrotation with cecal volvulus and a Ladd procedure is made openly without PO complications. Discussion The gold standard for diagnosis of intestinal malrotation is the upper gastrointestinal series. However, in patients with acute abdomen associated with this pathology where is suspected intestinal ischemia and hemodynamic instability, it is essential that an emergency laparotomy be performed. Conclusions The diagnosis of intestinal malrotation is difficult, since many patients are asymptomatic in adulthood or present with variable GI symptoms. Therefore, it is imperative to have a high index of suspicion in patients with compatible clinical characteristics in order to perform the best therapy in time and manner.

Malrotation occurs in approximately 1 in 500 live births. However, the true incidence of malrotation is unknown since many asymptomatic patients fail to present. Approximately 90% of patients with malrotation are diagnosed within the 1st year of life. Eighty per cent of them are diagnosed within the 1st month of life. Nevertheless, there are recent reports of manifestations later in life both as emergency conditions and more chronic gastrointestinal symptoms. The relationship between malrotation and horseshoe kidney has not been fully understood, but few case reports have highlighted their occurrence in the same patient. The mode of presentation of this case and its association with a horseshoe kidney is the reason for this report. This was a case of malrotation associated with horseshoe kidney. He had exploratory laparotomy and Ladd's procedure. Malrotation is associated with horseshoe kidney which presented as gastric outlet obstruction. He responded well to treatment after Ladd's procedure.

Thirty five years old women presented to the Accident and Emergency Department with acute onset right upper quadrant abdominal pain with normal vitals. CT images revealed a malrotation causing midgut volvulus and intestinal obstruction. On emergency exploratory laparotomy, the gut loops showed partial ischemia due to a double twist at the root of mesentery and malrotation of the gut along with the caecum which was mobile coming from left iliac fossa and lying in the right iliac fossa. Duodeno-jejunal junction was identified. The coils of the intestine were untwisted and rotation was corrected. The gut color changed back to normal. She was discharged painless after routine post-operative care.

Gastroschisis is defined as a congenital anterior abdominal wall defect which is rarely associated with other congenital anomalies. Gastrointestinal anomalies are the most common associations including malrotation. Other gut anomalies like intestinal duplication and Meckel's diverticulum are very rare. Our report a rare case of gastroschisis who is associated with malrotation, intestinal duplication and Meckel's diverticulum.

The use of intramedullary nail fixation remains the operation of choice for managing unstable and displaced tibia diaphyseal fractures. The literature shows that although commonly performed, there is not a standard approach when performing intramedullary nailing of the tibia; it could be hypothesised that this lack of standardisation may be contributing to the noted complications. This systematic review will look into intramedullary nailing of the tibia in all its parts, from identification of patients through to the surgical procedure techniques and finally the intra- and post-operative complications. A systematic review was conducted using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Search terms included "tibial intramedullary nail" OR "tibial intramedullary rod" OR "tibial IM nail" OR "tibial interlock" AND "malrotation", and "tibial intramedullary nailing" OR "tibial im nail" OR "tibial interlock" OR "tibial rod" AND "malrotation". Two independent reviewers conducted searches in PubMed, OvidSP for Medline and Embase as well as Cochrane Library using the same search strategy. Searches were conducted on 20 January 2021. Any disagreements were resolved by discussion with a third independent reviewer. This systematic review revealed there are gaps in the literature and in the management process of these patients, and suggested that a systematic approach using 'Get It Right First Time' (GIRFT), intraoperative assessment, validated assessment tools, and imaging postoperatively should be used to improve outcomes. Following the use of this framework, it is hoped that the incidence of malrotation post tibia intramedullary nailing will be reduced, however, it is acknowledged that more high evidence studies need to be carried out and further done to optimise the care of these patients.

Situs inversus totalis is the mirror image transposition of the abdominal-thoracic viscera. Approximately one in every 5,000 to 20,000 live births has situs inversus totalis. Most commonly, it is found incidentally and is asymptomatic. A number of malformations, including cardiac, splenic, and gastrointestinal, have been associated with this condition. Coexistence with duodenal atresia is extremely rare, reported in fewer than 30 cases worldwide and one case in Saudi Arabia. We report a preterm neonate who presented with bilious vomiting. Diagnosis of situs inversus totalis with duodenal atresia type III was established and other anomalies were ruled out. The patient was managed surgically by duodenal-duodenostomy and Ladd’s procedure. The report emphasizes the importance of identifying this condition and recognizing the "mirror anatomy" before carrying out an operation. Once the diagnosis is confirmed, surgical intervention must be performed as soon as possible to prevent complications.

Preduodenal portal vein (PDPV) is a rare congenital anomaly commonly associated with other gastrointestinal abnormalities. We report the case of a female neonate with a PDPV, intestinal malrotation and jejunal atresia. This is the second account of this association reported in the literature. The previously reported case underwent a gastroduodenostomy to correct the presumed duodenal obstruction caused by the PDPV. In our case, the PDPV was not corrected and the child remains well and asymptomatic. We propose that in this rare association, the PDPV is not a cause of obstruction and does not need correction.

This is a unique case of prenatal diagnosis of bowel malrotation suspected by an abnormal course of the duodenum. Early detection of volvulus was enabled, leading to timely intervention and a favorable outcome.

Background: Ultrasonography (USG) has been described as an alternative diagnostic tool for malrotation that evaluates the orientation of the superior mesenteric vessels. However, literature concerning the management of patients who do not have abdominal symptoms is limited. We aimed to review the clinical course of infants showing abnormal orientation of the superior mesenteric vessels on USG who were asymptomatic at the time of diagnosis.Methods: Seventy asymptomatic infants with abnormal orientation of the superior mesenteric vessels in a single center between 2014 and 2018 were retrospectively analyzed.Results: The 70 patients, 21 underwent upper gastrointestinal series (UGIS) and 11 underwent abdominal surgery for other surgical conditions. Among the 32 (45.7%) patients who underwent UGIS or abdominal surgery, 11 were proven to have malrotation. Of the 38 (54.3%) patients who did not undergo UGIS or abdominal surgery, six patients were too unstable to undergo UGIS, five died due to cardiac complications, and the remaining patient developed midgut volvulus and died 3 days after emergency surgery. The remaining 32 patients who did not undergo UGIS or abdominal surgery were discharged without additional tests, and all were asymptomatic until their last follow-up. In the multivariate analysis, history of heart surgery and the presence of more than three anomalies were significantly associated with malrotation.Conclusion: A significant number of malrotation were diagnosed in asymptomatic infants with abnormal orientation of the superior mesenteric vessels on USG. Infants with major cardiac or multiple anomalies need special attention and should undergo UGIS in a promptly manner to confirm malrotation.

Acute appendicitis is one of the most frequent causes of acute abdominal pain. Clinical signs, blood tests, and imaging are important to confirm the diagnosis. The classic presentation consists of periumbilical abdominal pain that migrates to the right lower quadrant, guarding, tenderness, and rebound tenderness in the region. We present the case of a 51-year-old male who presented with a one-day history of worsening supraumbilical pain. Abdominal computerized tomography (CT) scan showed intestinal malrotation in which the cecum assumed a midline supraumbilical location with CT features of acute appendicitis. This case highlights that the site of pain in acute appendicitis can be altered depending upon the anatomical location of the appendix, and relying on the pain's location can be misleading. We also suggest that patients with abdominal pain, which is not typical for acute appendicitis, should be investigated by abdominal CT if leukocytosis and inflammatory markers are raised.

Preduodenal portal vein (PDPV) is a rare cause of duodenal obstruction. While half of PDPV cases present with obstructive findings in the neonatal period, the other half are diagnosed incidentally at advanced ages. In half of the obstructed PDPV cases, the cause of obstruction is another congenital anomaly. In our case, the aberrant ventral extension of the pancreas with PDPV caused incomplete duodenal obstruction by compressing the duodenum externally. Özet Preduodenal portal ven (PDPV), duodenal tıkanıklığın nadir bir nedenidir. Yenidoğan döneminde PDPV olgularının yarısı obstrüktif bulgularla ortaya çıkarken, diğer yarısı ileri yaşlarda rastlantısal olarak teşhis edilmektedir. Tıkanmış PDPV vakalarının yarısında, tıkanıklığın nedeni başka bir konjenital anomalidir. Bizim olgumuzda PDPV’ye eşlik eden pankreasın anormal ventral uzantısı duodenuma dıştan bası yaparak inkomplet tıkanıklığa neden olmuştu., Nadir Görülen bir Konjenital Duodenal Obstrüksiyon Nedeni: Preduodenal Portal Ven. [ High resolution figures, metrics, and more: http://lifemedsci.com/a/2022/1/oz/7.htm ] [ This article previously published as: "Somalia Turkey Journal of Medical Science 2020; 1(1): 17-19." Currently, Somalia Turkey Journal of Medical Science was merged with Life and Medical Sciences. ], {"references":["1. Knight HO. An anomalous portal vein with its surgical dangers. Ann Surg 1921; 74(6): 697-9.","2. Georgacopulo P, Vigi V. Duodenal obstruction due to a preduodenal portal vein in a newborn. J Pediatr Surg 1980; 15(3): 339-40.","3. Grosfeld JL, Rescorla FJ. Duodenal atresia and stenosis: reassessment of treatment and outcome based on antenatal diagnosis, pathologic variance, and long-term follow-up. World J Surg 1993; 17(3): 301-9.","4. Rusu S, Zaghal A, Choudhry MS. Surgical Decision Making in Preduodenal Portal Vein: Report of Two Cases in Neonates. European J Pediatr Surg Rep 2018; 6(1): e40-e42.","5. Wabada S, Abubakar MA, Mustapha B, Pius S, Khalil J, Abana AK. Congenital duodenal obstruction due to duodenal atresia with preduodenal portal vein, annular pancreas, and intestinal malrotation associated with situs inversus abdominis: a case report. Journal of Pediatric Surgery Case Reports 2015; 3(12): 545-7.","6. Ohno K, Nakamura T, Azuma T, Yoshida T, Hayashi H, Nakahira M, et al. Evaluation of the portal vein after duodenoduodenostomy for congenital duodenal stenosis associated with the preduodenal superior mesenteric vein, situs inversus, polysplenia, and malrotation. J Pediatr Surg 2007; 42(2): 436-9.","7. Kim SH, Cho YH, Kim HY. Preduodenal portal vein: a 3-case series demonstrating varied presentations in infants. J Korean Surg Soc 2013; 85(4): 195-7.","8. Dinamarco B, Gonzaga E, França WM, Parron PLE, Soares PJM. Preduodenal Portal Vein (PDPV): A Very Rare Cause of Duodenum Obstruction. Surgical Science 2017, 8, 493-8.","9. Bailey PV, Tracy TF Jr, Connors RH, Mooney DP, Lewis JE, Weber TR. Congenital duodenal obstruction: a 32-year review. J Pediatr Surg 1993; 28(1): 92-5.","10. Dalla Vecchia LK, Grosfeld JL, West KW, Rescorla FJ, Scherer LR, Engum SA. Intestinal atresia and stenosis: a 25-year experience with 277 cases. Arch Surg 1998; 133(5): 490-6; discussion 496-7.","11. Escobar MA, Ladd AP, Grosfeld JL, West KW, Rescorla FJ, Scherer LR 3rd , Engum SA, Rouse TM, Billimire DF. Duodenal atresia and stenosis: long term follow up over 30 years. J Pediatr Surg 2004; 39(6): 867-71.","12. Mustafawi AR, Hassan ME. Congenital duodenal obstruction in children: a decade's experience. Eur J Pediatr Surg 2008; 18(2): 93-7.","13. Weber WF, Draus JM Jr. Preduodenal Portal Vein: A Rare Cause of Neonatal Bowel Obstruction. Am Surg 2016; 82(9): 775-6.","14. Esscher T. Preduodenal portal vein: A case of intestinal obstruction? J Pediatr Surg 1980; 15(5): 609-12.","15. Fernandes ET, Burton EM, Hixson SD, Hollabaugh RS. Pre-duodenal portal vein: Surgery and radiographic appearance. J Pedatr Surg 1990; 25(12): 1270-2.","16. Mordehai J, Cohen Z, Kurzbart E, Mares AJ. Preduodenal portal vein causing duodenal obstruction associated with situs inversus, intestinal malrotation, and polysplenia: A case report. J Pediatr Surg 2002; 37(4): E5.","17. Applebaum Harry, Lee Steven L, Puapong Devin P. Duodenal atresia and stenosis- annular pancreas, pp: 1399–405. In: Grosfeld JL, Oneill JA, Fonkalsrud EW, Coran AG (eds), Pediatric surgery. 2006, 6th ed., Mosby Elsevier, Philadelphia.","18. Srivastava P, Shaikh M, Mirza B, Jaiman R, Arshad M. Preduodenal Portal Vein Associated with Duodenal Obstruction of other Etiology: A Case Series. J Neonatal Surg 2016; 5(4): 54.","19. Bansal R, Dhillon KS, Kaushal G. Preduodenal portal vein: A recipe for disaster during laparoscopic cholecystectomy. J Minim Access Surg 2019; 15(1): 63-4.","20. Kato H, Usui M, Iizawa Y, Tanemura A, Murata Y, Kuriyama N, et al. Living Donor Liver Transplantation for Biliary Atresia With Severe Preduodenal Portal Vein Stricture: Success and Pitfall of Portal Vein Reconstruction. Transplant Proc 2016; 48(4): 1218-20."]}

A male infant aged 45 days presented with projectile nonbilious vomiting for 2 weeks. Ultrasound showed picture of idiopathic hypertrophic pyloric stenosis. Laparoscopic pyloromyotomy was done, but postoperative vomiting that was mainly nonbilious continued without improvement. After 4 days of persistent vomiting, laparoscopic exploration was done and complete pyloromyotomy was confirmed and malrotation with complete Ladd's band was found, then case converted to open laparotomy and Ladd's procedure was done. Postoperatively, vomiting stopped completely and baby began gradual feeding till reaching full feed. Despite that the presentation of concurrent Idiopathic Hypertrophic Pyloric Stenosis with malrotation is extremely rare; a formal laparoscopic abdominal exploration should be done as the first step before proceeding to pyloromyotomy.

Highlights • Pseudomyxoma peritonei (PMP) is a devastating consequence of perforated appendicular mucocele. It is considered a rare disease. • The presence of asymptomatic gut malrotation in adults can complicate the clinical picture of acute abdomen. ** (starting a new highlight point): Not considering possible anatomical variations initially during assessment could delay the diagnosis and managment. • The introduction of Cytoreductive Surgery/Heated Intraperitoneal Chemotherapy (CRS/HIPEC) improved the outcome of many patients with PMP. • Preserving part of the colon during challenging colorectal resection to allow a colo-rectal anastomosis offers a superior quality of life., Introduction Pseudomyxoma peritonei (PMP) is a feared complication of appendicular mucocele perforation. Although a rare disease, its major sequel mandates recognition and early intervention. Intestinal malrotation is mostly asymptomatic in adults. Its significance arises when it complicates another coinciding condition by confusing the presentation, leading to delay in diagnosis and treatment. PMP and incidental finding of gut malrotation in adults are two rare events, and the chance of both occurring in the same patient is very slim. This can complicate the clinical picture and lead to devastating outcomes. Presentation of the case We present a case of PMP in a patient with gut malrotation, managed with cytoreductive surgery (CRS) and heated intraperitoneal chemotherapy (HIPEC). Discussion Management with CRS/HIPEC has been found to improve outcomes for patients with PMP. However, the extensive disease and abnormal anatomy of the patient in our report proposed unique intraoperative challenges. Preserving part of the colon was possible with an improvised surgical technique that we used which proved to be safe and effective. Conclusion Early recognition and consideration of uncommon but serious surgical conditions are essential for improved patient outcomes. To our knowledge, this is the first report in the English literature that describes the use of CRS/HIPEC for PMP in a case of intestinal malrotation. This improvised surgical technique was found to be safe and can provide a surgical solution for preserving part of the colon in selected patients.

Introduction: Since the first description by William Ladd, the Ladd's procedure has been the surgery of choice for the correction of malrotation. The laparoscopic Ladd's procedure is becoming popular with the advent of minimal access surgery and is described in the literature. Various techniques of the Ladd's procedure have been described but none of them describes the stepwise technique for derotation of volvulus which is the most difficult and confusing part of the surgery. We describe 'steering wheel' technique for easy derotation of volvulus associated with malrotation. Method: A total of 62 patients were diagnosed to have an intestinal malrotation between 2010 and 2017. All cases which had complete non-rotation with a midgut volvulus were reviewed. Out of these, 48 patients were operated with open technique and 14 patients were subjected to the laparoscopic correction. Technique: Using three-port technique, stepwise derotation of volvulus is done which simulates the rotation of steering of car at an acute turn and has been described in four simple steps. This technique also stresses the importance of the release of Ladd's band before derotation. Results: Of 62 patients diagnosed with malrotation, 14 (22.6%) patients underwent the laparoscopic Ladd's procedure. The mean age was 26 + 8 months, mean weight was 10 + 2 kg and included eight males (57%) and six females (43%). There was only one (7.14%) conversion to open technique, due to a huge dilatation of duodenum causing difficulty in dissection in a patient with malrotation without volvulus. The laparoscopic Ladd's procedure took an average time of 70 ± 15 min. Conclusion: The laparoscopic 'steering wheel' derotation technique is easy and provides a stepwise description of the laparoscopic derotation of volvulus associated with malrotation in children.

We report a rare case of a neonatal girl who presented with coughing and dyspnea immediately after feeds. At birth, she was noted to have an imperforate anus with a posterior fourchette fistula from which she was stooling. Initial imaging with radiography showed a normal bowel gas pattern; however, lumbar vertebral anomalies were noted. An upper GI series was performed and revealed a laryngeal cleft and malrotation. Ultrasound confirmed malrotation with an abnormal SMA-SMV relationship. Since laryngeal cleft is a rare condition and may not be known to most radiologists, its incidence is likely underestimated. It is important to note the association of laryngeal clefts with VACTERL and malrotation. In addition, it is essential not to confuse a laryngeal cleft with a tracheoesophageal fistula since the management differs. Keywords: Laryngeal cleft, Laryngotracheoesophageal cleft, VACTERL, Malrotation

Preduodenal portal vein is a rare congenital vascular disorder that causes duodenal obstruction. In this case report, we discuss late presentation of duodenal obstruction resulting from this anomaly in a preadolescent female with cerebral palsy. The incomplete and late presentation of preduodenal portal vein creates a surgical dilemma. Awareness of this anomaly is critical and should be kept in consideration for all cases of duodenal obstruction.

Gut malrotation is usually a pediatric condition that presents mainly in the first month of life. It rarely presents in adults and is often a significant diagnostic dilemma to the surgeon. Failure to diagnose and treat this condition early may result in dire consequences like small gut gangrene. A previously healthy, 41-year-old female patient presented to the emergency department with a three-day history of lower abdominal pain and constipation. On examination, she had tachycardia, and her abdominal examination was soft, with mild tenderness in the hypogastric region and scanty bowel sounds. Urine pregnancy and dipstick were unremarkable. The patient was diagnosed with small bowel obstruction (malrotation of the small bowel loops with twisting of the mesenteric vessels). The patient was found to have thyrotoxicosis, which was the cause of the intestinal obstruction. She received conservative treatment and specific thyrotoxicosis management (carbimazole, cholestyramine, Lugol’s iodine, and intravenous hydrocortisone). The symptoms resolved entirely after a two-day stay at the intensive care unit. Small bowel malrotation is a congenital anomaly and can present with abdominal pain and obstruction in adulthood. Thyrotoxicosis can cause small bowel obstruction if there is underlying malrotation.

Intestinal malrotation (IM) is a congenital aberrancy of midgut rotation during development, which manifests among neonates more than adults. Older reports have estimated an incidence of one in 6,000 live births, which is now as high as one in 500. This congenital anomaly is generally indolent in the adult population. Recent literature research has failed to reveal any publications regarding the incidence within a Caribbean population. This paper aims to discuss the isolated case of a patient with this rare condition, who presented to a non-paediatric centre in Trinidad. This case highlights the implications of the initial radiological interpretation in conjunction with perioperative and intraoperative decision making. An 18-year-old male presented with a one-day history of abdominal pain, radiating to the epigastrium with nausea and excessive vomiting. Vital signs and blood investigations were normal. Initial CT scan results were interpreted as an internal hernia, which was surgically managed as such. Repeat imaging and a second laparotomy were required to correctly diagnose and perform the appropriate Ladd procedure. IM occurs due to the arrest of rotation of the midgut during fetal maturation. The incomplete rotation variant was seen in this case and is predominantly responsible for the symptomatology and morbidity associated with adult intestinal malrotation (AIM). Stringer has classified these anomalies based on the stage of embryonic development that is disrupted. CT helps with diagnostics in 97.5% of cases. This case highlights the implication of incorrect assessment on imaging and how it may misguide the interpretation of the findings at laparotomy leading to inappropriate surgical procedures. As many as 20% of cases undergo surgery without adult intussusception diagnosed. The incidence of IM seems to have increased but is scarcely encountered in the adult setting. When encountering this condition at a low-volume centre in the Caribbean, the adult specialist may be blindsided, and unknowingly underprepared without a high index of suspicion. Diagnosis at childhood should be discussed with the family and again with the patient on approaching adulthood. Patient education may help with the surgical assessment.

We describe a case of a six-month old male presenting with recurrent midgut volvulus following two previous Ladd procedures. Despite widening of the mesenteric base during each of the previous operations, the base again appeared narrowed, facilitating the development of a recurrent midgut volvulus. The patient underwent a third Ladd procedure with emphasis placed on widening of the mesenteric base, including division of the gastro-colic ligament to further separate the colon from the duodenum with the intent of preventing any future narrowing and subsequent intestinal volvulus.

Background A combination of intestinal malrotation and distal cholangiocarcinoma is considered a rare condition and poses some difficulties in surgical management. We present a case of a patient with asymptomatic nonrotation of the midgut with a concomitant distal cholangiocarcinoma who underwent successful pancreaticoduodenectomy. Case presentation A 52-year-old Sudanese man presented to our hospital with progressive painless jaundice associated with dark urine, pale stool, and itching for the last 2 months. He had no other complaint or significant previous medical history apart from being an ex-smoker. His clinical examination revealed a palpable gallbladder and scratch mark. His other systems were unremarkable. His blood test results showed a normal complete blood count, elevated total bilirubin (mainly direct bilirubin), elevated alkaline phosphatase, and normal cancer antigen 19-9 and carcinoembryonic antigen. Ultrasound, computed tomography of the abdomen, and magnetic resonance cholangiopancreatography showed a dilated intrahepatic and extrahepatic biliary system down to the distal part, where the lumen was obstructed by a periampullary mass measuring 2.4 by 2.1 cm. The patient’s gallbladder was distended. He had no liver metastases or ascites and few lymph nodes. Inversion of the superior mesenteric artery and superior mesenteric vein but no invasion was seen, and malrotation of the bowel was observed with the large bowel on the left side and the small bowel to the right of the abdomen. Endoscopic retrograde cholangiopancreatography showed a fleshy ampulla that was stented. Laparotomy showed malrotation, with the duodenum straight on the right side of the midline, and Ladd’s band crossed the second portion of the duodenum. The vessels were approached from the lateral side meticulously after kocherization of the duodenum and pancreas, dissection along an extended portion of the superior mesenteric artery to assure preservation of the superior mesenteric artery and branches going to the jejunum, Ladd’s procedure, division of the jejunum 10 cm below the uncinate process of pancreas, and modified pancreaticoduodenectomy were performed, and anastomoses were performed in the standard fashion. The patient had an uneventful postoperative course, started oral feeding after 5 days, and discharged to home on day 10 for regular follow-up. Histopathology confirmed distal cholangiocarcinoma, and the patient was referred for further oncological management. Conclusions Pancreaticoduodenectomy can be safely performed in patients with intestinal malrotation with some modifications of the standard approach. Meticulous dissection after preoperative identification of vascular anomaly and a lateral approach are of great help to reduce morbidity.

Congenital absence of the appendix is an extremely rare condition. Often found incidentally during surgical procedures, it poses a surgical dilemma. Surgeons must be well versed in the congenital abnormalities and positional variations of the appendix and must know the steps required during surgery to diagnose this abnormality. In this case report, we discuss the first ever occurrence of congenital absence of appendix in association with malrotation.

Background: The purpose of this study is to analyze the etiology, clinical presentation and outcome of neonatal intestinal obstruction at our institute.Materials and Methods: The medical record of all the patients, presented with intestinal obstruction in neonatal period during 2014 and 2015 was reviewed retrospectively for etiology, clinical features, investigations, management, and outcome.Results: Out of total 53 cases of neonatal intestinal obstruction, 27 were of intestinal atresia (9 cases (17%) were of duodenal atresia, 7 (13%) were of jejunal atresia and 8 (13%) were ileal atresias and 3 cases were found with colonic atresia); 7 were malrotation, 17 were Hirschsprung’s disease (HD). All the patients were investigated with abdominal radiography and sonography. All patients were managed surgically. Overall mortality was 10/53 (18.8%). Out of 27 cases of atresia, 9 patients died (33% mortality). Septicemia was the cause of death in 7 patients (58.3%). Anastomotic leak was present in one mortality case.Conclusion: The most common cause of neonatal intestinal obstruction is atresia. Duodenal atresia was the most common atresia in our study followed by ileal atresia. Postoperative complications like septicemia led to most of deaths in our series. Septicemia, wound infection, hypothermia, prematurity need special attention for survival of neonates.

Intestinal malrotation in neonates is well known with incidence of 1:500 and symptomatic incidence 1:6000 births. Duodenal web as a cause of intestinal obstruction is less common with incidence of 1:10000–1:40000. Intestinal obstruction due to duodenal web associated with malrotation is very rare and only few case reports published in the literature. In this report we present a case of a 2.5 year old female child with duodenal obstruction due to concurrent duodenal web and malrotation and discussed its management.

Background Malrotation is a congenital anomaly during the development of the embryonic intestine. Although it is generally considered a pediatric surgical condition, it can have significant implications for adult surgery in terms of reconstruction. Case presentation The patient was an 85-year-old man with pancreatic cancer and intestinal malrotation. He underwent pancreaticoduodenectomy with modified Child’s reconstruction. Because the ascending colon and efferent loop twisted easily, we fixed the ascending colon to the abdominal wall. Thereafter, right twist and stenosis of the efferent loop occurred. On the 22nd day after the initial surgery, detorsion and Braun anastomosis were performed for efferent loop fixation. Postoperative oral intake was good, and the patient was discharged from our hospital on the 24th day after the reoperation. Conclusions This is a rare case of pancreaticoduodenectomy with malrotation following reoperation due to a complication after Child’s reconstruction. In similar cases of intestinal malrotation, it is important to consider avoiding coaxial positioning of intestinal parts and an upper abdominal space while selecting a reconstruction method.

With an incidence of less than 1%, paraduodenal hernias are very rare but account for ~0.2–5.8% of mechanical small bowel obstruction and carry a mortality rate of 20–50%. Right-sided paraduodenal hernias are three times less frequent than left-sided paraduodenal hernias. We report the case of a 37-year-old man who suffered from colicky abdominal pain accompanied by vomiting. The computed tomography scan showed a mechanical ileus, caused by a presumed paraduodenal hernia, and we chose an elective laparoscopic surgical approach. The patient recovered quickly and was discharged on the second postoperative day. Paraduodenal hernias are a diagnostic challenge as they are typically characterized by long-term non-specific abdominal symptoms and are only detected in the event of acute intestinal obstruction. Until now, laparoscopic therapy has only been described in eight case reports and we review this rare condition and the surgical options.

Midgut malrotation is a congenital anomaly that usually occurs during infancy. The most common type is the “classic” form, which is defined as complete failure of rotation of both proximal (duodenojejunal limb) and distal (cecocolic) small bowel loops (nonrotation). Delayed diagnosis of midgut malrotation anomalies may lead to progression to catastrophic complications including volvulus and ischemic necrosis. Ladd’s procedure is the standard of care. We aimed to report a brief case of a midgut nonrotation in an adult male patient.

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Introduction and importance In this case report from Muhimbili National Hospital, Dar es salaam, Tanzania, we present the unexpected findings of anorectal malformation, colonic atresia, and intestinal malrotation in a 2-day old neonate. This combination is exceedingly rare, with only case reports published in the literature. We describe the challenges in diagnosis and offer our insights based on this experience and review of the literature. Case presentation Our patient was a male born at term, weighing 2600 g, diagnosed clinically with a high anorectal malformation. He was planned for colostomy, and we unexpectedly found a collapsed descending colon. Exploration revealed intestinal malrotation and three segments of type I colonic atresia from the mid transverse colon to the sigmoid colon in addition to the high anorectal malformation. Clinical discussion Creating a colostomy in a high anorectal malformation and failure to identify proximal intestinal atresia would result in potentially devastating consequences. Colonic atresia and anorectal malformation will both present as large bowel obstruction. In the extremely rare situation, when occurring in combination, the obvious clinical diagnosis of anorectal malformation will mask the clinical suspicion of the possibility of colonic atresia. Finding a distal bowel air bubble above the pubococcygeal line on an invertogram is useful in identifying proximal atresia preoperatively. Conclusion The current report emphasizes the importance of maintaining an awareness of possible associated colonic atresia in neonates with anorectal malformation. An invertogram and intraoperative finding of a collapsed descending colon should prompt evaluation for a proximal obstructing lesion., Highlights • A newborn presented with an imperforate anus and no other apparent anomaly • Findings of collapsed descending colon prompted explorative laparotomy. • Intestinal malrotation, colonic atresia and anorectal malformation was found. • Ladd's procedure and colostomy formation was done proximal to atretic segments. • Finding collapsed colon prevented an unnecessary colostomy distal to the atresia.

Introduction Congenital Intestinal malrotation is a complex disorder caused by incomplete or abnormal rotation of the intestine during fetal development. Volvulus of the transverse colon secondary to intestinal malrotation is a rare cause of acute abdomen in adults. It has a high risk of mortality, hence the need for an urgent diagnosis and surgical intervention. Case report We report an unusual case of volvulus of the transverse colon caused by intestinal malrotation. A 21-year-old women presented abdominal pain with nausea and vomiting. On clinical examination, the abdomen was tympanic to percussion with peritoneal sensitivity. The abdominal X-ray revealed a massive obstruction of the distended large intestine with a “U-shaped” loop. He underwent an exploratory laparotomy that revealed the diagnosis of transverse colon volvulus with intestinal malrotation. His condition was treated surgically by transverse colectomy with colostomy. The patient died on the second day following a hemodynamic instability. Conclusion Transverse colonic volvulus is a rare entity with a high mortality, so it requires urgent diagnosis and surgical intervention., Highlights • Volvulus of the transverse colon secondary to intestinal malrotation is a rare cause of an acute abdomen in adults. • Volvulus of the transverse colon has a high risk of mortality, so it requires urgent diagnosis and surgical intervention. • The diagnosis of the volvulus of the transverse colon before surgery is rarely observed, it's most often made intraoperatively. • The choice of a procedure depends on the general condition of the patient.

An association between malrotation and congenital duodenal webbing is rare. We present our experience with four patients at two centers, and a review of published reports. There are currently 94 reported cases of duodenal pathology associated with malrotation. However, only 15 of the 94 cases (15.9%) include patients with malrotation and a duodenal web. We suggest that nonbilious vomiting in a child must prompt the surgeon to consider duodenal pathology even in the presence of malrotation.

ObjectivesMalrotation of the femoral component can result in post-operative complications in total knee arthroplasty (TKA), including patellar maltracking. Therefore, we used computational simulation to investigate the influence of femoral malrotation on contact stresses on the polyethylene (PE) insert and on the patellar button as well as on the forces on the collateral ligaments.Materials and MethodsValidated finite element (FE) models, for internal and external malrotations from 0° to 10° with regard to the neutral position, were developed to evaluate the effect of malrotation on the femoral component in TKA. Femoral malrotation in TKA on the knee joint was simulated in walking stance-phase gait and squat loading conditions.ResultsContact stress on the medial side of the PE insert increased with internal femoral malrotation and decreased with external femoral malrotation in both stance-phase gait and squat loading conditions. There was an opposite trend in the lateral side of the PE insert case. Contact stress on the patellar button increased with internal femoral malrotation and decreased with external femoral malrotation in both stance-phase gait and squat loading conditions. In particular, contact stress on the patellar button increased by 98% with internal malrotation of 10° in the squat loading condition. The force on the medial collateral ligament (MCL) and the lateral collateral ligament (LCL) increased with internal and external femoral malrotations, respectively.ConclusionsThese findings provide support for orthopaedic surgeons to determine a more accurate femoral component alignment in order to reduce post-operative PE problems. Cite this article: K-T. Kang, Y-G. Koh, J. Son, O-R. Kwon, C. Baek, S. H. Jung, K. K. Park. Measuring the effect of femoral malrotation on knee joint biomechanics for total knee arthroplasty using computational simulation. Bone Joint Res 2016;5:552–559. DOI: 10.1302/2046-3758.511.BJR-2016-0107.R1.

Enteric duplication cysts occurring in association with intestinal malrotation are rare, with only a handful of case reports published. We describe a 5-week-old female patient who presented to our institution with a one-day history of bile-stained vomiting with an associated large palpable abdominal mass on clinical examination. Ultrasound evaluation suggested a large cystic lesion arising from the right kidney, however, Intra-operatively we found a jejunal duplication cyst with an associated malrotation and midgut volvulus.

Background Portal venous gas has been considered as a radiological sign requiring urgent operative intervention; however, the reports concerning portal venous gas associated with favorable outcome are recently increasing. Case presentation We describe a 9-month-old boy with acute onset high fever and vomiting. The ultrasonography demonstrated micro-gas bubbles continuously floating in the intrahepatic portal vein. Contrast-enhanced CT, performed 1 h later from echography, revealed a whirlpool sign suggesting an intestinal malrotation with midgut volvulus, but with no signs of residual intrahepatic gas. Operative findings showed a mild volvulus with neither congestion nor ischemic change of the twisted bowel. Detorsion and Ladd’s procedure were completed laparoscopically. Conclusions Transient portal venous gas bubbles may be generated even in the mild intestinal volvulus with no bowel ischemia. Ultrasonography can be a sensitive detector to visualize such small amounts of gas.

A greenstick fracture is an incomplete fracture where the compressive side of the cortex is still intact but plastically deformed. The incidence of poor results following the closed treatment of greenstick fractures in children >10 years of age is seriously underestimated. Therefore, fixing the position of the forearm is important for initial treatment. In cases of greenstick fracture, the possibility of inadequate remodeling of angulated deformities during growth, and in particular, the lack of correction between rotational malalignment and growth when the diaphyseal forearm is involved in the fracture, should be considered. A male, 10-year-old, left hand-dominant, Asian patient fell while playing in the garden and was immediately assessed by an orthopedic doctor at an Emergency Orthopaedic Clinic. Initial examination revealed a deformity of his dominant left forearm and an angulated greenstick fracture of the radius. However, after 3 months, he developed loss of supination of his left forearm and complained of limitation of left forearm supination. Radiography demonstrated a volar angulation of 20°. The patient underwent open reduction, internal fixation and 10° bending with a plate for correction without corrective rotation. At 12 months after injury, the patient did not exhibit pain or limitation of the elbow and wrist. After follow-up for 7 years, the patient was able to perform normal day-to-day activities with no adverse symptoms. The present case indicated that corrective osteotomy is required following the loss of supination after a greenstick fracture of the diaphysis of the radius. The patient of the current study exhibited rotation due to the central band of the interosseous membrane. In the treatment of greenstick fractures, a radius apex angulation of 20° must be corrected via osteotomy due to loss of rotation. The present case indicated that corrective osteotomy of the radius apex alone without rotational correction, in combination with plate bending improved the loss of forearm rotation.

Supernumerary Kidney is a one of the rarest congenital renal anomalies with less than 100 cases reported in the literature. This accessory kidney has its own collecting system, vascular supply and well-defined capsule. We report a case of 18-year-old lady with a history of vague intermittent abdominal pain and has palpable abdominal mass who was found to have malrotated right supernumerary kidney with pelviureteric junction obstruction. Keywords: Supernumerary kidney, Congenital anomaly, Malrotation

Highlights • Intestinal malrotation results from anomalies in embryological midgut rotation. • Malrotation usually presents early in life, with rare cases reported in adulthood. • Ladd procedure remains the mainstay of definitive treatment. • Radiologic findings have a role in early detection and correction of malrotation., Introduction Midgut malrotation results from abnormalities in the 270-degree counterclockwise rotation of the midgut around the axis of the superior mesenteric artery during embryological development, and classically presents early in life with symptoms of intestinal obstruction. Nevertheless, adult cases have occasionally been reported. Presentation of case An 80-year-old female with no surgical history was brought to our emergency department for acutely altered mental status. On exam, her abdomen was distended and diffusely tender to palpation. Computed tomography (CT) scan of the abdomen and pelvis showed a dilated loop of jejunum with evidence of mesenteric twist concerning for closed-loop small bowel obstruction. The patient was taken for exploratory laparotomy and was found to have Ladd bands and other findings suggestive of intestinal malrotation. A Ladd procedure was performed and the patient remained under observation. She experienced intermittent abdominal distension and bilious nasogastric tube output, but subsequent CT scans revealed no evidence of obstruction. She was discharged following clinical improvement and ability to tolerate a diet. Discussion Malrotation of the small bowel exists on a spectrum depending on the embryologic stage during which anomalous rotation occurs. Classic findings on CT imaging (including abnormal mesenteric vasculature, right-sided duodenojejunal junction, whirlpool signs, and left-sided ascending colon) can provide clues to the existence of malrotation. Conclusion Although malrotation is rare in adults, clinical and radiologic findings play an important role in the correct diagnosis of adult malrotation for appropriate and timely intervention.

Introduction Treatment of stone disease in anomalous kidneys can be challenging. As ureteroscopy (URS) has advanced, the number of studies reporting on outcomes of URS for stone disease in anomalous kidneys has increased. Our objective was to perform a systematic review of the literature to evaluate the outcomes of URS for stone disease in this group of patients. Methods A Cochrane style review was performed in accordance with the PRISMA guidelines using Medline, EMBASE, CINAHL, Cochrane Library, Scopus and individual urologic journals for all English language articles between inception and June 2018. Results Fourteen papers (413 patients) with a mean age of 43 years and a male to female ratio of 285:128 were included. The underlying renal anomaly was horseshoe kidney (n = 204), ectopic kidney (n = 117), malrotation (n = 86), cross fused ectopia (n = 2) and others (n = 2). With a mean stone size of 16 mm (range 2–35 mm), the majority of stones were in the lower pole (n = 143, 34.6%) or renal pelvis (n = 128, 31.0%), with 18.9% (n = 78) having stones in multiple locations. Treatment modality included the use of flexible ureteroscope in 90% of patients and ureteral access sheath used in 11 studies. With a mean operative time of 61.3 min (range 14–185 min), the initial and final SFR was 76.6% (n = 322) and 82.3% (n = 340), respectively. The overall complication rate was 17.2% (n = 71), of which 14.8% were Clavien I/II and the remaining 2.4% were Clavien ≥ III complications. Conclusion Although ureteroscopy in patients with anomalous kidneys can be technically challenging, advancements in endourological techniques have made it a safe and effective procedure. In these patients the stone-free rates are good with a low risk of major complications.

Segmental small bowel volvulus is a condition in which a portion of bowel twists upon itself. The most common causes include malrotation and intestinal atresia. Volvulus can present at any age, but typically presents within the first few weeks of life. Presentation of midgut volvulus with normal mesenteric fixation and no malrotation is termed primary midgut volvulus. Unrecognized midgut volvulus leads to bowel ischemia and varying degrees of bowel necrosis. We present a rare case of Segmental small bowel volvulus without malrotation in a preterm neonate. On day of life 53, a preterm female born at 25 weeks gestation developed abdominal distention and respiratory distress. Abdominal x-ray showed distended, gas-filled loops of bowel and upper GI series showed no malrotation. Due to concerns for intestinal obstruction, an exploratory laparotomy was performed, which revealed a segmental small bowel volvulus with extensive necrosis, but no malrotation. A well-known complication of midgut volvulus is bowel ischemia, often leading to necrosis and sepsis. With the low prevalence of primary midgut volvulus, delayed diagnosis is common, which often leads to fatal outcomes. Therefore, primary midgut volvulus should be considered in all patients, including neonates that present with abdominal distention, shock, and signs of intestinal obstruction.

Background: A female neonate with anorectal malformation (ARM) may have one, two, or three openings in the perineum. One opening represents cloaca while three openings usually suggest low ARM. Females with two openings in the perineum may be ARM without any fistula, rectovaginal fistula, or absent vagina. The association of ARM with multiple intestinal atresias and malrotation is rare. Case Presentation: We present here a case of a female neonate with two perineal openings, whose perineum was explored by a general surgeon without any radiological investigation and was later found to have a high ARM, multiple jejunal and ileal atresia with malrotation. Conclusion: ARM associated with small bowel atresia and malrotation are rarely described. Such cases require management in Pediatric surgery settings.

A duodenal obstruction with three separate obstructing duodenal webs is a very rare congenital anomaly, with only one other case reported in the literature. We present a case in a 3-week-old newborn that underwent successful surgical repair.

Intestinal malrotation is a congenital anomaly of the midgut. The incidence of intestinal malrotation is 1 in 500 live births. It is rarely seen in adults. Most of the symptomatic patients present with acute bowel obstruction, intestinal ischemia or chronic vague abdominal pain. We present a 49-year-old male presenting with the findings of acute intestinal obstruction.

Malrotation is an anomaly that occurs when the embryological middle bowel, or ‘midgut’, rotates around the superior mesenteric artery and ends with opposite fixation to the peritoneum. There are many causes of left lower quadrant abdominal pain, the main reasons being diverticulitis, hernias, neoplasms, sigmoid colon perforations, testicular torsions, urinary pathologies, and gynecological pathologies. Although left lower quadrant abdominal pain is associated with the diagnosis of acute appendicitis in patients with malrotation, a known history of malrotation is helpful for diagnosis. In this case report, a patient who admitted with left lower quadrant abdominal pain and was diagnosed as malrotation and acute appendicitis is presented.

Intestinal atresia is a rarely reported congenital anomaly in Haiti. To date there exists little to no published data describing the management of this entity in Haiti. As a low-income country, diagnosis and management of intestinal atresia is a big challenge due to limited resources and limited access to pediatric surgery centers. The purpose of this case report is to describe successful management of a type IV jejunoileal atresia in Haiti. We present a case of a type four intestinal atresia associated with malrotation. Even with a prenatal ultrasound and postnatal plain films concerning for gastrointestinal obstruction, surgery was not performed until seventeen days of life. The clinical course was marked by malnutrition and sepsis. However, the outcome after surgery was good, aided by early enteral feeding through a jejunostomy tube and peripheral parenteral nutrition. Management of intestinal atresia is a large challenge in low-income countries like Haiti. However, acceptable outcomes can be achieved by adapting the surgical technique to permit an early introduction of enteral nutrition.

Congenital short bowel syndrome (CSBS) is an uncommon gastrointestinal disorder in which an unclear aetiology causes considerable intrauterine reduction in small bowel length. As a result of reduced absorptive intestinal length, chronic diarrhoea, vomiting, and consequently, failure to thrive are likely. We report a case of CSBS in a 26-day-old girl who had malrotation and a short bowel with a length of bowel from the pylorus to the ileocecal junction of approximately 40 cm. The patient underwent Ladd’s procedure, but she is still dependent on parenteral nutrition.