{"title":"3D-printed model for surgical planning in congenital porto-systemic shunt: A case report","authors":"Sergio Assia-Zamora, Miriam Cortés-Cerisuelo, Athanasios Tyraskis, Nigel Heaton","doi":"10.1016/j.epsc.2024.102926","DOIUrl":"10.1016/j.epsc.2024.102926","url":null,"abstract":"<div><h3>Introduction</h3><div>Hepatobiliary surgery can be challenging due to the complex nature of variations in liver anatomy, particularly in small children. Congenital portosystemic shunts (CPS – Abernethy syndrome) are increasingly recognized with a range of clinical presentations and associated complications and can present anatomical challenges for operative shunt closure. Three-dimensional printing (3DP) has gained popularity due to its ability to produce accurate models with the specific anatomical characteristics for the individual patient. We used a 3DP model from original image sources for planning of surgical shunt closure in a child with CPS and for educating the family and surgical team preoperatively.</div></div><div><h3>Case presentation</h3><div>A 5-year-old male with autism and hyperammonemia of 80 μmol/L and was found to have an intrahepatic CPS. No tumors were seen within the liver. Physical examination was unremarkable. After treatment with Rifaximin to lower blood ammonia levels, his ability to concentrate and achieve milestones improved. Angiography with shunt occlusion showed an intrahepatic shunt between the left portal and the middle hepatic vein into the IVC<em>.</em> Intrahepatic portal branches were demonstrated with the implication that a one stage closure could be performed. A 3DP model was printed from the cross sectional imaging which allowed for more detailed planning of shunt ligation and to explain the surgical details to the parents<em>.</em> Surgical closure was performed with no complications.</div></div><div><h3>Conclusion</h3><div>3DP models can be a useful tool for planning complex congenital porto-systemic shunt closure surgery.</div></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"112 ","pages":"Article 102926"},"PeriodicalIF":0.2,"publicationDate":"2024-11-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142705151","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Spigelian hernia diagnosed in a newborn: A case report","authors":"Nicole Chicoine , Frederick Rescorla","doi":"10.1016/j.epsc.2024.102922","DOIUrl":"10.1016/j.epsc.2024.102922","url":null,"abstract":"<div><h3>Introduction</h3><div>Spigelian hernias represent only 0.1–0.2 % of all abdominal wall hernias and are infrequently encountered in pediatric patients. Limited literature surrounding pediatric Spigelian hernias exists, and there is no uniform or optimal surgical repair technique.</div></div><div><h3>Case presentation</h3><div>A term female with unremarkable prenatal history presented a left-sided Spigelian hernia at birth that contained loops of bowel, by physical examination and ultrasound. The hernia was easily reducible, so she was discharged with a plan for an elective repair at a later point. At 8 months of age, she underwent an abdominal wall ultrasound that showed a defect of 6 cm in length. She underwent elective repair at 9 months of age due to parental preference in the setting of an enlarging defect size. The hernia repair was done through a combination of laparoscopic and open techniques. The laparoscopy part, which consisted in one port placed in the umbilicus for a camera, enabled visualization of the suspected hernia site, confirm the proper approximation of the lateral and medial borders of the hernia, and helped avoid any intra-abdominal injuries during the repair. The repair itself was completed in an open manner with interrupted sutures in a top to bottom approach, in a transverse orientation. The patient recovered well from the operation. At 1 year of follow up she has shown no signs of recurrence.</div></div><div><h3>Conclusion</h3><div>Congenital pediatric Spigelian hernias can be successfully repaired using a combination of laparoscopy and open approach. The addition of a laparoscopy allows proper visualization of the intra-abdominal side of the repair.</div></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"112 ","pages":"Article 102922"},"PeriodicalIF":0.2,"publicationDate":"2024-11-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142705153","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Fereshteh Moshfegh , Zahra Hosseinzade , Seyed Hamid Salehi , Mohammad Javanbakht , Ali Manafi Anari , Behnam Sobouti
{"title":"Rapidly progressing necrotizing fasciitis with chickenpox infection: A case series","authors":"Fereshteh Moshfegh , Zahra Hosseinzade , Seyed Hamid Salehi , Mohammad Javanbakht , Ali Manafi Anari , Behnam Sobouti","doi":"10.1016/j.epsc.2024.102925","DOIUrl":"10.1016/j.epsc.2024.102925","url":null,"abstract":"<div><h3>Introduction</h3><div>Varicella usually resolves without complications. However, there is a risk of necrotizing soft tissue infections such as necrotizing fasciitis (NF) that can complicate the management.</div></div><div><h3>Case series</h3><div>Case 1 was a 5-year-old male who developed widespread vesicular lesions and fever, diagnosed as varicella. His condition worsened as he presented with abdominal distension and pain, leading to hospitalization for suspected toxic shock syndrome. Computed tomography (CT) of the chest and abdomen revealed features consistent with NF, prompting extensive debridement and subsequent vacuum-assisted closure (VAC) therapy. After several surgical revisions he underwent skin grafting and was eventually discharged home. Case 2 was a 4.5-year-old male with no significant medical history, who presented with generalized fever, neck pain, and rapidly spreading skin rashes. He subsequently developed severe acute idiopathic scrotal edema and extensive ecchymosis, requiring immediate hospitalization. Broad-spectrum antibiotics were started. He underwent emergent wide debridement of the ecchymotic areas. The pathology analysis confirmed necrotic tissue, and cultures were positive for bacteria and fungi. He underwent repeated debridement. The last debridement was followed by uncontrollable hemorrhage, and he passed away.</div></div><div><h3>Conclusion</h3><div>While varicella is generally a mild disease, life-threatening complications such as necrotizing fasciitis can occur.</div></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"112 ","pages":"Article 102925"},"PeriodicalIF":0.2,"publicationDate":"2024-11-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142658599","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Mashael AlSiddiqi , Safaa Najar , Sami Taha , Hassan Baghazal , Noora Alshahwani
{"title":"Recurrent spontaneous gastric perforations in a neonate with renal tubular dysgenesis: A case report","authors":"Mashael AlSiddiqi , Safaa Najar , Sami Taha , Hassan Baghazal , Noora Alshahwani","doi":"10.1016/j.epsc.2024.102923","DOIUrl":"10.1016/j.epsc.2024.102923","url":null,"abstract":"<div><h3>Introduction</h3><div>Neonatal gastric perforation (NGP) is a very rare, life-threatening condition. Management can be complicated in patients with co-morbidities. Recurrent gastric perforation is even more rare.</div></div><div><h3>Case presentation</h3><div>A 33 6/7 male was born by cesarean section and had a birth weight of 2300g. His mother was gravida 3/para 1, otherwise healthy. The parents were third-degree cousins. Prenatal ultrasounds showed severe oligohydramnios with bilateral hyperechogenic kidneys. On physical exam, he had subtle facial dysmorphism, rocker bottom feet, stiff joints, and long slender fingers. He developed respiratory distress shortly after birth, which required intubation and mechanical ventilation. He was made nil per os (NPO), and antibiotics were started. At 20 hours of life, he had a sudden deterioration with desaturations, hypotension, and severe abdominal distension. Abdominal X-ray showed free air in the peritoneal cavity. We placed a peritoneal drain. One day later he reaccumulated air. We suspected that he could have renal tubular dysgenesis (RTD) given that he had no urine output. One day later he was taken to the operating room for an exploratory laparotomy. We found a large perforation in the lesser curve of the stomach. We repaired the perforation and placed a peritoneal dialysis catheter. Four days later he was re-explored for pneumoperitoneum. We found a perforation in the anterior wall of the stomach and repaired it in 2 layers. There was no bowel obstruction distal to the stomach. Three days later, we re-explored him for pneumoperitoneum and found a perforation on the posterior wall. The two other repairs were intact. We closed the perforation and placed a gastrostomy. Thirteen days later he was re-explored for pneumoperitoneum, and we found a second perforation in the posterior wall. Feedings were slowly started. His renal function improved, and he was discharged home at 3 months of age.</div></div><div><h3>Conclusion</h3><div>The association of RTD and spontaneous gastric perforation has not been previously reported. We do not know if there is a causality between the two conditions but based on our case, we recommend that patients with RTD should be closely watched for signs of gastric perforation.</div></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"112 ","pages":"Article 102923"},"PeriodicalIF":0.2,"publicationDate":"2024-11-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142658535","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Evance Salvatory Rwomurushaka , Patrick Amsi , Jay Lodhia
{"title":"Ovarian dysgerminoma in a pre-pubertal girl: A case report","authors":"Evance Salvatory Rwomurushaka , Patrick Amsi , Jay Lodhia","doi":"10.1016/j.epsc.2024.102920","DOIUrl":"10.1016/j.epsc.2024.102920","url":null,"abstract":"<div><h3>Introduction</h3><div>Ovarian dysgerminoma is a rare malignant germ cell tumor that rarely occurs in pre-puberal girls.</div></div><div><h3>Case presentation</h3><div>An eight-year-old girl presented with a year-long history of progressive, painless abdominal distension and significant weight loss but without obstructive symptoms. Physical examination revealed peripheral subcentric lymphadenopathy and signs of cachexia. Her abdomen was globally distended with a large, irregular mass occupying most of the abdominal cavity. Imaging with CT of the chest and abdomen revealed a large intraperitoneal mass with regional lymphadenopathy and pulmonary metastases, initially raising suspicion for peritoneal Burkitt's lymphoma. However, an ultrasound-guided biopsy was inconclusive, prompting an exploratory laparotomy. During surgery, a large mass measuring 20 by 30 cm was excised, originating from the right ovary and fed by mesenteric vessels. The uterus, left ovary, and fallopian tube were intact and unaffected, and the peritoneum, liver, and omentum appeared smooth and without nodules.</div><div>The initial postoperative course was uneventful, and histopathology confirmed the mass as an ovarian dysgerminoma. Adjuvant chemotherapy with Etoposide, Carboplatin, and Bleomycin was initiated. Unfortunately, the patient succumbed nine weeks post-surgery, following two cycles of chemotherapy, likely due to complications from hospital-acquired pneumonia and pre-existing pulmonary metastases.</div></div><div><h3>Conclusion</h3><div>Ovarian dysgerminoma must be included in the differential diagnosis of pre-pubertal females who present with a pelvic mass.</div></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"112 ","pages":"Article 102920"},"PeriodicalIF":0.2,"publicationDate":"2024-11-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142658584","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Multifocal hepatic adenoma in an adolescent female requiring right hepatic lobectomy: A case report","authors":"Prabhath Mannam , Patricia Repollet-Otero , Oluwadamilola Egbewole , Nicolle Burgwardt , Christine Finck","doi":"10.1016/j.epsc.2024.102924","DOIUrl":"10.1016/j.epsc.2024.102924","url":null,"abstract":"<div><h3>Introduction</h3><div>Hepatic adenomas are benign tumors that are extremely rare in adolescents. Proper and early management is essential to prevent associated hemorrhage and potential malignant transformation.</div></div><div><h3>Case presentation</h3><div>We herein present a case of a 17-year-old female patient with obesity, precocious puberty, and oral contraceptive use that presented with abdominal pain, emesis, elevated liver enzymes, and leukocytosis. Magnetic resonance imaging identified two lesions within the right hepatic lobe: a 2.0× 6.8 × 2.0 cm T1-hyperintense semilunar component and a 5.9 x 7.2 × 7.2 cm T1-hypointense component suspicious for intralesional hemorrhage. A diagnosis of hepatic adenoma was made based on imaging characteristics and the patient's oral contraceptives were discontinued to prevent lesion expansion. The patient was discharged following improvements in her clinical presentation and was scheduled for follow up imaging. Three weeks later, the patient presented again with similar symptoms, suggestive of an acute exacerbation of her hepatic lesions. Repeat computed tomography imaging revealed free fluid in the pelvis and expansion of the larger lesion to 10.9 x 6.5 × 6.4 cm that was now bilobed with irregular borders. The lesions were unsuitable candidates for embolectomy and the patient ultimately underwent a right hepatic lobectomy and wedge biopsy of segment II for another lesion discovered intraoperatively. Pathological analysis identified three distinct masses in the right hepatic lobe and a single mass within segment II, all of which were consistent with a diagnosis of a benign multifocal hepatic adenoma. No residual tumor or recurrence was identified at 3-month follow-up imaging.</div></div><div><h3>Conclusion</h3><div>Despite being benign, hepatic adenomas can present with rapid growth and aggressive clinical deterioration that may require urgent surgical resection.</div></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"112 ","pages":"Article 102924"},"PeriodicalIF":0.2,"publicationDate":"2024-11-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142658598","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Umar Mahmood, Muhammad Usama Aziz, Maria Munawar, Laraib Amjad, Chaudhary Ehtsham Azmat, Sunaina Siddique
{"title":"Uterine rupture due to pyometra and imperforate hymen in a 7-year-old girl: A case report","authors":"Umar Mahmood, Muhammad Usama Aziz, Maria Munawar, Laraib Amjad, Chaudhary Ehtsham Azmat, Sunaina Siddique","doi":"10.1016/j.epsc.2024.102921","DOIUrl":"10.1016/j.epsc.2024.102921","url":null,"abstract":"<div><h3>Introduction</h3><div>Pyometra is a rare condition characterized by pus accumulation in the uterine cavity primarily affecting postmenopausal women, with only a few cases reported in the pediatric population.</div></div><div><h3>Case presentation</h3><div>A 7-year-old female with developmental delay was brought to the hospital in shock and having clinical signs of peritonitis. Her medical history included abdominal pain and pyuria for one week prior to presentation. The only other positive physical finding was that the hymen was imperforate. An erect abdominal X-ray revealed air under the diaphragm and an ultrasound (US) of the abdomen showed moderate ascites. She was taken to the operating room for an exploratory laparotomy. We found approximately 700 mL of purulent material. The examination of all abdominal organs revealed a perforation at the fundus of the uterus and extensive adhesions. We repaired the uterine wall and left a trans-abdominal drain in the uterine cavity. We also left a drain in the pelvis. We then closed the abdomen and did a hymenoplasty during which we drained 200 additional milliliters of purulent fluid. Cultures were positive for Acinetobacter species. She remained on tobramycin for 10 days. The drains were removed on the 2nd and 5th postoperative days. She was discharged home after an uneventful recovery and has been healthy on all postoperative visits.</div></div><div><h3>Conclusion</h3><div>Female children with imperforate hymen are at risk for pyometra and uterine perforation. A perineal exam should be included in the work up of premenarchal females who present with peritonitis.</div></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"112 ","pages":"Article 102921"},"PeriodicalIF":0.2,"publicationDate":"2024-11-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142658585","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Appendiceal hydatid cyst in a 5-year-old child: A case report","authors":"Mobina Taghva Nakhjiri, Jafar Soltani, Maryam Ghavami Adel, Hojatollah Raji","doi":"10.1016/j.epsc.2024.102916","DOIUrl":"10.1016/j.epsc.2024.102916","url":null,"abstract":"<div><h3>Introduction</h3><div>Hydatidosis is the formation of cysts caused by the parasitic infection of Echinococcus granulosus. This disease predominantly affects the liver and lungs. The involvement of other organs is quite rare.</div></div><div><h3>Case presentation</h3><div>A 5-year-old Iranian boy presented with abdominal pain, anorexia, and hepatomegaly. The pain had been present for about 1 year. He had developed low-grade fever during the previous two months. On physical exam he had abdominal distention and a palpable mass on the right lower quadrant. Basic blood work revealed leukocytosis and eosinophilia. An abdominal ultrasound revealed a 5 × 6 × 8 cm cystic lesion in the pelvis, and two cystic lesions located in the right lobe of the liver. A computerized tomography (CT) scan of the abdomen confirmed the findings. CT of the chest and brain ruled out further cystic lesions. The patient had a positive Indirect hemagglutination assay (IHA), confirming the diagnosis of hydatidosis. A regimen of an albendazole was initiated. He was taken to the operating room for the resection of all cysts. The hepatic cysts were drained and re-filled with hypertonic saline solution, which was left in place for several minutes. Next, the hypertonic solution was drained and all hepatic cysts removed. We turned our attention to the pelvic cyst. It had a thick wall and was indistinguishable from the surrounding tissues. After dissecting it we found that it was arising from the wall of the appendix. We surrounded it with gauze embedded in hypertonic solution and resected the majority of it, leaving a small aspect of the external layer that was densely adherent to the bowel. He recovered well from the operation, remained on albendazole for 6 months, and has had no recurrences this far.</div></div><div><h3>Conclusion</h3><div>Appendiceal hydatid cysts should be included in the differential diagnosis of patients with hydatid disease and pelvic cysts.</div></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"111 ","pages":"Article 102916"},"PeriodicalIF":0.2,"publicationDate":"2024-10-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142552036","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Humaid Alzaabi, Maithah Alkaabi, Hiba Ahmad, Iftikhar Jan
{"title":"Laparoscopic repair of a sliding right inguinal hernia containing both fallopian tubes, both ovaries and the uterus in an infant: A case report","authors":"Humaid Alzaabi, Maithah Alkaabi, Hiba Ahmad, Iftikhar Jan","doi":"10.1016/j.epsc.2024.102914","DOIUrl":"10.1016/j.epsc.2024.102914","url":null,"abstract":"<div><h3>Introduction</h3><div>Sliding inguinal hernias containing the uterus, fallopian tubes, and adnexa are rare and can be challenging to repair, especially in infants.</div></div><div><h3>Case presentation</h3><div>A 4-month-old female infant, born prematurely from a triplet pregnancy, had been managed in the Neonatal Intensive Care Unit (NICU) for several months before discharge. She was referred to us with swelling in the right inguinal area. The other two triplets had inguinal hernias and had undergone surgical repair at the same hospital. On physical exam, the patient had a sizable, reducible hernia with no signs of obstruction or strangulation. She was taken to the operating room for a laparoscopic exploration, which was done with one 5-mm port for a camera and two 3-mm working ports. We found a sliding right inguinal hernia that contained both ovaries, both fallopian tubes, and part of the uterus in the hernial sac. The contents were carefully reduced, the hernial sac was carefully dissected, and the defect was repaired laparoscopically using a purse-string suture. No hernia was found on the left side. The postoperative course was uneventful. At six months of follow-up, she is doing well and has no signs of recurrence.</div></div><div><h3>Conclusion</h3><div>Sliding inguinal hernias containing bilateral ovaries, fallopian tubes, and the uterus are rare. Laparoscopy can help with the diagnosis, the reduction of the contents, and allows to assess the condition of the reduced organs.</div></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"111 ","pages":"Article 102914"},"PeriodicalIF":0.2,"publicationDate":"2024-10-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142571442","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Carolina Pinzon-Guzman , Alexander Kevorkov , Karl Grenier , Maeve O'Neill Trudeau , Pramod Puligandla , Etienne St-Louis
{"title":"Segmental absence of intestinal musculature increasingly recognized in premature infants with perforated viscus: A case series","authors":"Carolina Pinzon-Guzman , Alexander Kevorkov , Karl Grenier , Maeve O'Neill Trudeau , Pramod Puligandla , Etienne St-Louis","doi":"10.1016/j.epsc.2024.102915","DOIUrl":"10.1016/j.epsc.2024.102915","url":null,"abstract":"<div><h3>Introduction</h3><div>Segmental absence of intestinal musculature (SAIM) is a pathological diagnosis, denoting focal absence of the muscularis propria in the presence of intact surrounding structures. This condition often presents as spontaneous intestinal perforation (SIP) and can be difficult to distinguish clinically from necrotizing enterocolitis (NEC).</div></div><div><h3>Case presentations</h3><div>We present 5 cases of premature babies with gestational age ranging from 24 + 3 weeks up to 32 + 1 weeks, who all presented with intestinal perforation before the 15th day of life. Three patients presented with a distended abdomen and discoloration and were subsequently found to have pneumoperitoneum on abdominal x-ray (AXR). The 2 other patients presented with clinical deterioration but without signs of intestinal perforation and were subsequently found to have pneumoperitoneum on AXR. All underwent bowel resection of perforated bowel with either ileostomy with mucous fistula or primary end-to-end anastomosis. Two patients required repeat exploratory laparotomies within a week of their initial surgery due to pneumoperitoneum in subsequent AXR caused by new perforations in other parts of the small bowel. One patient sustained an iatrogenic liver injury intra-operatively and passed away 1 day post-operatively. All patients were found to have SAIM on histopathological examination.</div></div><div><h3>Conclusion</h3><div>SAIM is a pathological diagnosis that can manifest as SIP and may denote a risk for recurrent peritonitis in premature infants who underwent bowel resection for perforated viscus without a clear etiology.</div></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"111 ","pages":"Article 102915"},"PeriodicalIF":0.2,"publicationDate":"2024-10-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142571443","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}