Rare Congenital Anomaly Research Articles

Neuroendocrine Tumors in Horseshoe Kidneys: A Review of the Literature With Report of a Novel Finding Expanding Their Morphologic Spectrum

Horseshoe kidney is a rare congenital anomaly with an unusually higher frequency of neuroendocrine tumors. Symptoms are rare, and, in most of the cases, are incidentally diagnosed. The clinical behavior of these tumors is heterogeneous and can be difficult to predict based on histology alone. Necrosis and percentage of Ki-67 may have a role in prognosis. Almost all tumors are carcinoids (well-differentiated neuroendocrine tumors) observed at an early age and with no sex dominance. It is not known the reason for the higher frequency of neuroendocrine tumors in horseshoe kidneys and the histogenesis is unknown. One of the hypotheses supports that renal carcinoid tumors may arise from neuroendocrine cells within foci of metaplastic or teratomatous epithelium within the kidney. With consonance with this hypothesis, there are reports of carcinoids in horseshoe kidneys associated with a cystic lesion lined by the intestinal epithelium, with mucinous differentiation and osseous metaplasia, arising in a mature teratoma of the kidney, arising within mature teratoma and clear cell renal cell carcinoma, with a mucinous cystadenoma element, and arising within mature cystic teratoma synchronous with primary adenocarcinoma. There is only one reported large cell neuroendocrine carcinoma of a horseshoe kidney in a 57-year-old Chinese woman. Herein, we report a patient that to the best of our knowledge is the first case of a combined well-differentiated neuroendocrine tumor and large-cell neuroendocrine carcinoma with rhabdoid features in horseshoe kidney. The histologic component of rhabdoid features expands the morphologic spectrum of neuroendocrine tumors in the horseshoe kidney. We provide a comprehensive review of the literature summarizing pertinent key clinical and pathologic aspects.

A rare case of left transverse testicular ectopia in an adult

Introduction and importance: Transverse testicular ectopia (TTE) is a rare congenital anomaly in which both testes descend into the same inguinal canal and are located in the same hemiscrotum. Diagnosing TTE can be challenging due to its rarity and unusual presentation. Case presentation: The authors present a case of a male in his 50s who was diagnosed with left transverse testicular ectopia after presenting with an empty right hemiscrotum and two testes-like masses in the left hemiscrotum. Clinical discussion: TTE usually presents with a history of an inguinal hernia and a contralateral undescended testis. However, in our case, the patient had both testes in the left hemiscrotum without an inguinal hernia, making this case even rarer. Conclusion: Patients presenting with an empty hemiscrotum on one side and two testes-like masses on the other should be suspected of having TTE. In such cases, it is important to use imaging like USG or MRI, to identify and locate the ectopic site and assess the testicular morphology.

Matching the opposites: liver transplantation from a situs viscerum inversus totalis donor.

Situs viscerum inversus totalis (SIT) is a rare congenital anomaly. Deceased donors with this condition are often declined because of the technical issues in both the organ's procurement and its transplant. Only eight cases of deceased donor organs with SITwere reported to be used for liver transplantation (LT). We herein present a case of LT using a graft from an SIT donor: a modified retroversus piggyback technique was used. A 15year-old female was referred to our institution as a potential donor. An SIT condition was discovered during standard donor evaluation together with the presence of a complex triple arterial pedicle. Procurement operative time was 125min, from skin incision to cross-clamp. Liver extraction occurred 32min after cold flush. The recipient was a 56year-old male affected by recurrent hepatocellular carcinoma (HCC) on hepatitis C related liver cirrhosis. Position and orientation trials of the graft were made and it was decided to implant it with the retroversus technique. Direct duct-to-duct biliary reconstruction was achieved. The postoperative course was uneventful. To our knowledge, this is the first implant with retroversus technique combined to direct biliary reconstruction and the first repetition of that technique. Cases like this highlight how technical complexity can be overcome leading to successful management of difficult scenarios in a safe manner.

VS 08 - HPB Laparoscopic cholecystectomy for left-sided gallbladder – How not to do it?

Abstract Background Left-sided gallbladder (LSG) is a rare congenital anomaly in the gallbladder, which is defined as a gallbladder located on the left side of the falciform ligament and round ligament without situs inversus. (1) The reported prevalence of sinistroposition ranges from 0.2% to 1.1%. (2) There are three recognized variants of LSG: LSG associated with situs viscerum inversus, true LSG, and gallbladder located to the left of abnormally located right-sided round ligament/ ligamentum teres. Method We present a case of a gentleman who was diagnosed as having gall bladder sinistroposition intraoperatively, after all the laparoscopic ports had been placed as per a routine laparoscopic cholecystectomy. Although the gentleman had undergone preoperative MRCP, it was not reported by the radiologists as such. The surgery was carried out using the usual ports used for standard four-port laparoscopic cholecystectomy and demonstrates the difficulty faced by the surgeon with these ports. Results Critical view of safety was demonstrated. Gall bladder was removed in piece meal. Surgery lasted for more than 90 minutes. There were no post-operative complications. Suggested modifications include Right working port from the Palmers point(3) or using a robotic approach with the same standard configuration(4). Our reflection revealed – interchanging of 5 mm and 12 mm working ports could have been helpful for better access. Conclusion The presence of a left sided gall bladder is often associated with various biliary, portal venous and other anomalies that might lead to intra-operative injuries. (5) The diagnosis is usually incidental and is done intraoperatively. Hence surgeons must be aware of these possibilities.

Abstract 4145822: The Enigma of Chest Pain: Rare Presentation of ALCAPA Syndrome in a Young Adult

Introduction: Anomalous Left Coronary Artery Arising from the Pulmonary Artery (ALCAPA), also known as Bland-White-Garland syndrome, is a rare congenital anomaly(incidence:1 in 300,000 live births) typically presenting during infancy. Adult presentations are even rarer due to a high mortality rate of nearly 90% in the first year of life without surgical intervention. This report examines an adult patient with exercise-induced chest pain who received a delayed diagnosis of ALCAPA via multimodal imaging. Case Summary: A 21-year-old male presented to the emergency department(ED) with severe retrosternal chest pain radiating to both shoulders and arms, which began during exercise 30 minutes prior. He had a history of similar, milder episodes during exercise, previously treated as musculoskeletal pain and gastroesophageal reflux disease in the ED. His medical, social, and family histories were unremarkable. Physical examination revealed a soft, continuous murmur along the left sternal border. During this visit, An EKG done showed sinus tachycardia at 110 bpm and deep T-wave inversions in the anterolateral leads, a dynamic ST-T changes from his baseline.Serial Cardiac biomarkers and a lipid profile were within normal ranges. Initially managed for acute coronary syndrome-unstable angina with antithrombotic therapy, beta-blockers, and nitrates, he underwent urgent coronary angiography. This revealed a dilated right coronary artery (RCA) with retrograde flow to the left coronary artery (LCA) into the main pulmonary artery (PA) via collaterals, with no evidence of atherosclerosis (Figure 1).Echocardiography (Figure 2) and coronary CT angiography (Figure 3) confirmed the diagnosis of ALCAPA. The patient underwent successful surgical repair with aortic reimplantation of the LCA and was discharged in good health. Discussion: In adult ALCAPA patients, good collateral circulation may develop; however, myocardial ischemia can occur due to the "coronary steal phenomenon" and poor collateral flow regulation, leading to angina, arrhythmias, and sudden cardiac death. ALCAPA should be considered a differential diagnosis in adults presenting with exercise intolerance, as most sudden cardiac death cases occur by the fourth decade. A high index of clinical suspicion and a multimodality imaging approach is essential for the early diagnosis of this rare coronary anomaly, underscoring the critical role of early surgical intervention in preventing catastrophic, life-threatening events.

Abstract 4134619: A Novel Missense Mutation in TNNT2 Gene in a Lebanese Pedigree With Ebstein Anomaly And Wolf-Parkinson-White Syndrome: A Case Report

Background: Ebstein anomaly (EA) is a rare congenital heart defect occurring in 1.2 to 5 in 100,000 live births, characterized by a downward displacement of the tricuspid valve, thin-walled right ventricle, and tricuspid valve regurgitation. It can present variably from asymptomatic cases to severe symptoms like arrhythmias and right-sided heart failure. EA is often associated with other anomalies such as interatrial communication and mitral valve prolapse. The condition can lead to accessory atrioventricular pathways, frequently resulting in Wolff-Parkinson-White syndrome (WPW), which involves abnormal heart electrical activity and increases the risk of sudden cardiac death. While the genetic basis of EA is not fully understood, it appears to involve multiple genes like FLNA and NKX2-5, MYH6, MYH7 suggesting a complex polygenic inheritance pattern. TNNT2 is known to be associated with Cardiomyopathy but has not been previously associated with EA and WPW. Case: In this report, we present findings from a lebanese family with EA, comprising 2 affected individuals. Whole exome sequencing in the affected individuals identified a pathogenic variant in the TNNT2 gene at coding strain position 260 (a missense mutation: C to T), resulting in the substitution of Proline with Leucine at position 87 in affected individuals (Figure 1). No variants were detected in any other candidate gene examined. Individuals I:1 and II:2 were found to be normal, with no EA findings on echocardiography. Methods: Whole exome sequencing in the patients involved collecting blood samples after obtaining consent. These samples were sent to Centogene lab, where genomic DNA is enzymatically fragmented. The regions of interest are then enriched using DNA capture probes, facilitating detailed genetic analysis. Results: The whole exome sequencing identified a heterozygous missense mutation in both the father and his daughter. This nonsynonymous variant is located on chromosome 1 (GRCh37) in the TNNT2 gene region. The specific variant, NM_001276345.1:c.260C>T p.(Pro87Leu), was found in both individuals. Conclusion: In conclusion, the genetic basis of EA is rather complex and remains poorly understood. It is established that mutations in the TNNT2 gene are linked to several cardiomyopathies, none of them overlap with the described phenotype of our patients. In this report, we therefore confirm the potential role of TNNT2 gene mutation in the genetic basis of familial EA with WPW.

A Stillborn Baby with Tetra-amelia Syndrome in Jember: A Case Report

Tetra-amelia syndrome is a rare congenital disorder characterized by the absence of all four extremities. This extremity disorder occurs in 1 in 1,300 to 2,000 births. This condition is caused by genetic mutations in the WNT3 and RSPO2 genes. Specific risk factors for tetra-amelia syndrome remain unclear. This is also due to the lack of studies that discuss specific risk factors for tetra-amelia syndrome. In this case report, we discuss a stillborn baby with tetra-amelia syndrome to a mother with various pregnancy risk factors in Jember. This case report aims to examine the presence of several risk factors in the mother and their relationship with congenital disorders, especially tetra-amelia syndrome.

A Case Report of Dorsal Pancreatic Agenesis and Uterus Didelphys in a Young Woman: A Seldom- reported Association

Agenesis of the dorsal pancreas is a rare congenital anomaly that often remains asymptomatic and is frequently discovered incidentally during imaging for other conditions. Its association with Müllerian anomalies, such as uterus didelphys, suggests that both conditions may stem from similar embryological disruptions during early fetal development. This report aims to raise awareness of Agenesis of the Dorsal Pancreas (ADP) and its potential associations with Müllerian anomalies. By presenting a detailed case study, we seek to address the diagnostic challenges faced by healthcare professionals and the implications for patient management, emphasizing the importance of early detection and appropriate intervention. We present a case involving a 24-year-old Iranian female who presented with a 10-day history of abdominal pain and vomiting. Initial ultrasound imaging revealed moderate hydroureteronephrosis in the right kidney, prompting further investigation. A contrast-enhanced CT scan was performed, which not only confirmed ureteropelvic junction obstruction but also unexpectedly revealed dorsal pancreatic agenesis. In addition to these findings, the patient was diagnosed with uterus didelphys, highlighting a significant developmental anomaly. This case underscores the necessity for increased awareness of ADP and its associations with Müllerian anomalies among healthcare providers. Early and accurate diagnosis through advanced imaging modalities significantly improves patient outcomes and facilitates better management strategies for associated complications.

Medical Alert Cards for patients with an anorectal malformation: a useful tool to increase awareness.

Anorectal malformations (ARMs) are rare congenital anomalies causing altered anatomy and frequent hospitalizations. Parental awareness and clear communication are crucial in family-centered care. This study assesses the impact of patient-held ARM Medical Alert Cards on healthcare providers and caregivers. Caregivers of children with ARMs, including cloacal anomalies, received Medical Alert Cards with (1) contact details; (2) disease-related information; and (3) patient-specific data. A survey evaluated these cards' effectiveness in enhancing ARM awareness among healthcare professionals and caregivers. Among 33 respondents, 29 (88%) found the cards helpful in increasing ARM awareness. Both caregivers and providers found the content coherent, with a median readability score of 9 (IQR 8-10). The card layout was well-received by 30 out of 32 respondents (94%), and all noted the clarity of the contact details, facilitating patient referrals to specialized care. Most caregivers (13 out of 14, 93%) expressed willingness to use these cards in medical settings. Suggestions for improvement included adding more patient-specific information, enhancing readability, and providing visual anatomy diagram. Medical Alert Cards for patients with ARMs can enhance understanding of ARMs among caregivers and healthcare providers, serving as a vital tool in patient-centered management strategies tailored to individual needs.

A Rare Neck Lesion in Children: Bilateral Chondrocutaneous Branchial Remnant.

Cervical chondrocutaneous branchial remnant is a rare congenital developmental anomaly typically located on the lateral neck. Histologically, it has the appearance of an accessory tragus demonstrating a central cartilaginous core with surrounding fibrosis located in the subcutaneous tissue. In order to address the clinical and therapeutic aspects of cervical chondrocutaneous branchial remnants in children, a 6-year-old boy who presents with bilateral hard cervical masses at the lower region of the neck is presented. Chondrocutaneous branchial remnant is a very rare cervical lesion in children, and surgical excision is the only choice of treatment. Cervical chondrocutaneous branchial remnant has been associated with a variety of congenital anomalies, particularly involving the auditory, cardiovascular, and visual systems. Despite bilateral occurrence of cervical chondrocutaneous branchial remnant, our patient had no cardiac or genitourinary anomalies.

A Review of Sturge–Weber Syndrome Brain Involvement, Cannabidiol Treatment and Molecular Pathways

Sturge–Weber syndrome (SWS) is a rare congenital neurocutaneous disorder typically caused by a somatic mosaic mutation in R183Q GNAQ. At-risk children present at birth with a capillary malformation port-wine birthmark. The primary diagnostic characteristic of the disorder includes leptomeningeal enhancement of the brain, which demonstrates abnormal blood vessels and results in impaired venous drainage and impaired local cerebral perfusion. Impaired cerebral blood flow is complicated by seizures resulting in strokes, hemiparesis and visual field deficits, hormonal deficiencies, behavioral impairments, and intellectual disability. Therefore, anti-seizure medication in combination with low-dose aspirin is a common therapeutic treatment strategy. Recently published data indicate that the underlying mutation in endothelial cells results in the hyperactivation of downstream pathways and impairment of the blood–brain barrier. Cannabidiol (CBD) has been used to treat medically refractory seizures in SWS due to its anti-seizure, anti-inflammatory, and neuroprotective properties. Pilot research suggests that CBD improves cognitive impairment, emotional regulation, and quality of life in patients with SWS. Recent preclinical studies also suggest overlapping molecular pathways in SWS and in CBD, suggesting that CBD may be uniquely effective for SWS brain involvement. This review aims to summarize early data on CBD’s efficacy for preventing and treating epilepsy and neuro-cognitive impairments in patients with SWS, likely molecular pathways impacted, and provide insights for future translational research to improve clinical treatment for patients with SWS.

Correction of Severe Pterygium Colli with a Staged, Double, Posterior and Lateral Approach: A Case Report

AbstractPterygium colli is a rare congenital disorder characterized by excessive skin in the lateral and posterior neck, often associated with genetic syndromes such as Turner's syndrome or Klippel–Feil syndrome. Severe presentations present special challenges for surgeons since its diagnosis is not always evident and its treatment often results in important scaring. Surgical correction of the typical cases mostly involves a lateral approach, but in this “special” case, we used a double, posterior and lateral approach. A 15-month-old girl with prenatal diagnosis of a cystic lesion in the posterior neck presented with massive neck webbing at birth. Genetic studies ruled out chromosomal disorders and RASA 1 mutations. Surgical correction involved a modified posterior approach combined with a lateral approach 1 year later. The cervical tissue redundancy was successfully reduced achieving a good aesthetic outcome with very acceptable scars. At 1 year of follow-up, the patient continues with good neck appearance and mobility. The goal of surgical treatment of pterygium colli is achieving a harmonious neck contour with good function and minimal scarring while minimizing complications and sequelae. No consensus exists on the optimal approach for its correction, emphasizing the need for individualized treatment considering patient-specific characteristics, particularly in the severe cases.

Walking Ability After Microsurgical Reconstruction of Pediatric Popliteal Pterygium Syndrome—A Case Report

Background: Popliteal pterygium syndrome (PPS) is a rare congenital disorder characterized by orofacial, cutaneous, musculoskeletal, and genital anomalies. Surgical interventions are necessary to address the severe knee flexion contracture and equinovarus deformity, but there are no established treatment guidelines. Methods: We present the case of a one-year-old patient with PPS and discuss the challenges in managing the knee deformity. The surgical option chosen for the unilateral knee contracture of 80° consisted of skin management by a large Z-plasty, lengthening of popliteal vessels by grafts, lengthening of the tibial and peroneal nerves by autografts and allografts, capsular releases, and tendon releases to improve mobility and preserve foot sensibility. Results: With a three-year follow-up, the surgical interventions resulted in proper ability to walk freely. Wearing of a foot orthesis was necessary to balance the leg length differences and support the midfoot deformity. Furthermore, sensation of the foot could be restored in terms of touch sensibility and perfusion was always stable during growth. Discussion: The treatment of PPS requires a multidisciplinary approach, considering the rarity and complexity of the syndrome. Surgical interventions aim to release contractures, correct deformities, and preserve foot sensibility. Each treatment option has its advantages and disadvantages, highlighting the need for individualized care.

095. A Rare Case Report: A 10-Years-Old Girl with Ectopic Ureter and Ectopic Hypoplasia Kidney

Background: The urinary tract system develops from an embryological structure in the dorsal part of the embryo, the intermediate mesoderm, which develops between the 4th and 10th week of gestation. During the embryogenesis process, disturbed metanephric mesenchyme can cause congenital abnormalities of the kidneys and urinary tract (CAKUT), one of which is ectopic abnormalities. Ectopic ureter and ectopic kidney had an incidence of 1:2000 and 1:3000 worldwide, respectively. Additionally, this rare case will be even more uncommon with the presence of a hypoplasia kidney. These anomalies contribute to impaired urinary drainage. Case: A 10-year-old girl was referred to the surgical department at Dr. Kariadi Hospital due to continuous urinary incontinence. She can urinate regularly via the urethra, but constantly has vaginal leaks. Previously, she had physiotherapy at the regional hospital but there was no improvement. Contrast-enhanced computed tomography revealed an ectopic kidney image accompanied by right kidney hypoplasia and a cyst on the upper pole of the right kidney measuring ± 0.89 x 0.86 cm. Then, urethrocystoscopy and colposcopy also showed the right ureteral orifice on the superior wall of the vagina, ± 2 cm from the vaginal introitus with the left ureteral orifice in a normal position. The patient underwent ureteral reimplantation, and her symptoms successfully resolved, leading to a favourable prognosis. Conclusion: Complete ectopic ureter and ectopic hypoplasia kidney are rare congenital anomalies that require early recognition and careful management in pediatric patients.

Anomalous Origin of the Right Subclavian Artery from the Right Pulmonary Artery in a Patient with d-Transposition of the Great Arteries: Diagnosis and Surgical Treatment.

Anomalous origin of the right subclavian artery (AORSA) from the right pulmonary artery is a rare congenital anomaly usually diagnosed by computed tomography, magnetic resonance imaging, or angiography. We present a very rare case of diagnosis and surgical correction of AORSA coexisting with d-transposition of the great arteries in a newborn's first hours of life. Due to the critical local hospital and patient conditions, additional cross-sectional imaging was not feasible. The diagnosis was suspected by clinical signs, confirmed by transthoracic echocardiogram, and the patient was urgently taken to the operating room. We discuss clinical and echocardiographic signs of this child with d-transposition of the great arteries and AORSA from the right pulmonary artery.

042. Colonic Duplication with Recto-Vestibular Fistula: A Case Report

Background: Anorectal malformations associated with colonic duplications are a rare condition. Colonic duplication may have different presentations according to its location and size, with an incidence of less than 15% of all gastrointestinal duplications. Colonic duplication is less common than ileal duplication, whether cystic or tubular. Duplications are considered congenital malformations because they are thought to arise from disturbances in embryonic development. This report aims to present and analyze the clinical presentation, diagnostic approach, and management of anorectal malformations associated with colonic duplications in an 11-month-old child at our hospital, highlighting the challenges and treatment strategies for such a rare case. Case: We present a case of an 11-month-old girl, referred from a network hospital with a post-colostomy at 4 months of age for recto-vestibular fistula with findings of colonic duplications at colostomy. Exploratory laparotomy was performed until the colonic duplications were identified and completely resected. Conclusion: Colonic duplication is a rare congenital disorder, and its association with anorectal abnormalities is even rarer. The surgical treatment includes resection of the duplicated colon. Surgical intervention is the treatment of choice.

Type 1a Duodenojejunal Tubular Duplication Cyst with Complex Rotational Anomaly Masquerading as Chronic Anemia

ABSTRACT Enteric duplication cysts and reversed intestinal rotation (RIR) are rare congenital anomalies, with their coexistence being exceptionally uncommon. We report a 4-year-old girl who presented with chronic anemia and intermittent abdominal symptoms since infancy. Detailed workup for medical causes of anemia was inconclusive. Computed tomography of the abdomen revealed intestinal malrotation with a grossly dilated small bowel loop. Intraoperative findings revealed a long duodenojejunal tubular duplication with a separate mesentery (Type 1a) and RIR. The patient underwent a Ladd’s procedure, resection of the duplication cyst, and end-to-end anastomosis. This case underscores the anatomical rarity, varied clinical presentation, and challenges in making an accurate and timely diagnosis in such a case.

ACUTE RESPIRATORY DISTRESS REVEALING A COMPLETE ATRIOVENTRICULAR CANAL IN A 22-MONTH INFANT

The atrioventricular canal (AVC) is a complex and rare congenital cardiac anomaly. It is potentially severe due to the complexity of its lesions and associated fixed pulmonary arterial hypertension. We report the case of a 22-month-old infant girl with Trisomy 21 syndrome, admitted for acute respiratory distress. TTE showed a complete AVC with pulmonary arterial hypertension. Prognosis depends on the promptness of diagnosis and therapeutic management.

009. Case Report: Cloacal Exstrophy a Rare Case of Abdominal Defect on Pediatrics

Background: Cloacal Exstrophy (CE) is a rare congenital disorder. CE caused by rupture of the cloacal membrane before the urogenital septum is formed, then causes herniation. CE has incidence of 1: 200,000 to 1: 400,000. Embryologically, CE is considered to be the result of a defect in the caudal mesoderm, around 29 developmental days, or failure of mesodermal migration during the primitive streak period resulting in failure of cloacal septation. The aims and objectives of this case report are to explain the case in depth with existing literature and its suitability. Case: The method used is prospective direct observation. The case, a female 2-days-old patient, presented with omphalocele, ileal prolapse, incompletely formed hindgut, open bladder, abdominal wall defect, and urine come from the hemiblader and meconium from the cecal plate. The patient was born via cesarean because the mother with fetal distress. The patient underwent a Dual Stage Pathway (DSP), first the abdominal wall defect was closed with a colostomy, then bladder reconstruction was carried out. The reconstruction procedure in CE cases can be carried out with Dual Stage Pathway (DSP), which consists of omphalocele reconstruction, bladder closure, gastrointestinal diversion and closure of the abdominal wall. Conclusion: CE is a congenital disorder in many organ systems including neurological, gastrointestinal, musculoskeletal, renal and genitourinary. Surgical management of CE patients requires multidisciplinary care with a multi-stage approach.

002. Case Report: High Jejunal Atresia Type 1 Treated by Side to Side Anastomosis Jejuno-Jejunostomy

Background: Jejunal atresia characterized by complete occlusion lumen of the intestinal, is a rare congenital abnormality occurring in 1 out of 12.000 live births. The most recent hypothesis is that it caused by vascular accidents in the uterus during embryonic development. jejunal atresia Type I is characterized by mucosal, stenosis of the intestine. Some literature explains that side to side anastomosis surgery cannot be used in cases of jejunal atresia. The purpose of this case report is to present a case of high jejunal atresia type 1 managed with side to side anastomosis. Case: A 6 days old female with abdominal distension and billous vomiting since birth. the babygrams obtained a triple bubble sign. On emergency exploratory laparotomy, jejunum atresia was found 5 cm from the treitz ligament, no mesenteric defect, a web was found, the ratio of the diameter of the proximal and distal jejunum lumen was 7:1. The patient was decided to do Kimura procedure. The remaining bowel was normal. We initiated enteral feeding on day 7 post operatively and then achieve full enteral feeding on day 25. Patient discharged without complication. Conclusion: High jejunal atresia type 1 can be managed with side to side anastomosis (jejuno-jejunostomy) surgery technique and the outcome was good.