Journal of Pediatric Surgery Case Reports最新文献

Introduction

We report a case of fetal volvulus presenting with meconium-stained amniotic fluid, illustrating how meconium can mask in utero bilious emesis.

Case presentation

The male neonate was born at 37 + 1 weeks gestation via c-section for maternal indications and admitted to the Neonatal Intensive Care Unit for respiratory distress presumably due to meconium aspiration. However, an initial x-ray was not consistent with meconium aspiration syndrome. Overnight, the orogastric tube drained 173 mL of dark green-tinged fluid and the bilirubin was elevated at 8.2 (threshold 7.4), prompting phototherapy. Orogastric feeds were attempted but unsuccessful. The orogastric output was originally attributed to swallowed meconium. However, the large volume, bilious color, persistent output, unexplained respiratory distress, and elevated bilirubin prompted an upper GI series (UGIS). The UGIS was consistent with a proximal obstruction that was likely present from birth. The patient underwent exploratory laparotomy which revealed a volvulus from malrotation that was repaired with detorsion and Ladd procedure. Despite a dusky bowel suggestive of ischemic changes, the bowel was fully pink after untwisting and no bowel needed to be resected. After untwisting, Ladd's bands were lysed, patency was demonstrated, and an appendectomy was performed. The baby was discharged on day-of-life 16 with full oral feeds and no complications.

Conclusion

Persistent bilious gastric output in the setting of extensive meconium-stained amniotic fluid is an uncommon presenting sign of fetal volvulus from malrotation. Prompt recognition is important to prevent bowel necrosis.

Introduction

Imperforate hymen and transverse vaginal septum are conditions characterized by obstructive defects, typically leading to hematometrocolpos detected around the time of puberty. We encountered a patient who had undergone hymenotomy in infancy to treat pyocolpos due to an imperforate hymen and later developed hematometrocolpos in puberty due to a transverse vaginal septum.

Case presentation

A 13-year-old female presented with hypomenorrhea and hematometrocolpos-induced dysmenorrhea. She had a history of pyocolpos with a urinary tract infection at 3 months of age. At that time, no vaginal opening was found on perineal examination, and imperforate hymen was diagnosed. When hymenotomy was performed, the external cervical os was not detected by intravaginal bronchoscopic examination. However, this abnormal finding was not fully investigated. The postoperative course after hymenotomy was free of complications, and the patient remained asymptomatic until menarche. When she presented 3 months after menarche, genital examination revealed a bulging transverse septum inside the vagina, 5 cm from the hymen. Ultrasound and magnetic resonance imaging revealed a distended uterus, distended upper vagina, and collapsed lower vagina. Transverse vaginal septum was considered, and vaginoscopy was performed to exclude vaginal reclosure or adhesion caused by the previous surgery. A diagnosis of transverse vaginal septum was confirmed, and total excision of the septum was performed. The patient underwent regular postoperative follow-ups for stenosis prevention.

Conclusion

Considering the rarity of these concurrent conditions, clinicians must always consider the possibility of several complex anomalies. Vaginoscopy using a hysteroscope may be useful for definitive diagnosis and determining appropriate treatments.

Introduction

Although extrapulmonary complications can occur in cases of severe Respiratory Syncytial Virus (RSV) infections, abdominal compartment syndrome (ACS) has not previously been described in the context of RSV. In this case series we describe two infants who presented with severe RSV and subsequently developed ACS.

Case presentations

Case 1: A 7-week-old term infant with severe RSV requiring intubation and care in the ICU. Shortly after admission, she became septic with abdominal distention and erythema, concerning for an intra-abdominal source. In addition, she had increased peak airway and bladder pressures suggestive of ACS. An abdominal ultrasound revealed significant ascites and dilated loops of bowel, but no suitable window for drainage. Given the possibility of intra-abdominal sepsis and ACS, an exploratory and decompressive laparotomy was performed revealing colitis and tense ascites. After relief of ACS, she quickly recovered with successful closure of her abdomen and eventual resolution of her ascites.

Case 2: A 6-month-old preterm infant who presented similarly to Case 1 with symptoms of severe RSV. A few days after ICU admission, she developed abdominal distention with hemodynamic instability, acute kidney injury, and rising bladder pressures, suggestive of ACS. She then urgently underwent a decompressive laparotomy with similar findings of tense ascites and colitis. Despite surgical decompression, however, her sepsis worsened post-operatively with evidence of multisystem organ failure, including hemodynamic instability, anasarca, and coagulopathy. Ultimately, she succumbed to her illness.

Conclusion

Our case series shows that infants with severe RSV are at risk for ACS, which can be deadly despite surgical intervention.

Introduction

Innominate Artery Compression of the Trachea (IACT) is rare but can cause stridor in infancy. IACT involves anterior compression of the trachea as the innominate (brachiocephalic) artery originates later along the transverse portion of the aortic arch. We present a case of IACT in a previously healthy 9-month-old male that failed to respond to medical therapy. This case demonstrates an unusual, delayed presentation of IACT.

Case presentation

Our patient is a 9-month-old previously healthy male presenting to the outpatient pulmonology clinic with a one-month history of stridor. Patient initially presented to outside providers and prescribed systemic steroid courses, which failed to improve the stridor. On exam, patient demonstrated respiratory distress and biphasic stridor that worsened when feeding from a bottle. Patient was admitted from clinic for further evaluation by ENT. Initial chest radiographs showed findings consistent with viral lower respiratory infection versus reactive airway disease and no radiopaque foreign body. Bedside scope was performed by ENT, which did not show obstruction or foreign body above the level of the glottis. Computed tomography (CT) without contrast was performed per ENT recommendations, which showed focal tracheal narrowing but significant movement artifact. To provide further clarification on the tracheal narrowing, a direct bronchoscopy was performed by ENT, which showed tracheal compression at the level of the thoracic inlet. Computed Tomographic Angiography (CTA) of the chest confirmed the diagnosis of IACT. Patient underwent arterial relocation and reimplantation, and the tracheal compression was markedly improved on repeat bronchoscopy. Patient recovered from the procedure well with improvement in stridor and respiratory distress. Patient was discharged on POD3 and followed up in pulmonology clinic with resolution of stridor and no residual respiratory symptoms.

Conclusion

This case particularly highlights the importance of continued investigation into persistent stridor that is refractory to medications, with special consideration for congenital causes.

Introduction

Symptomatic incomplete vascular rings resulting from aberrant right subclavian arteries (ARSA) are usually repaired with a subclavian to carotid transposition via a right thoracotomy or median sternotomy approach. We describe the successful use and long-term outcomes of a less invasive approach via a right collar incision for ARSA repair in a pediatric case series.

Case presentations

Between January 2020 and December 2022, four pediatric patients with symptomatic ARSAs underwent operative repair using a right collar incision on the pediatric general and thoracic surgery service at a quaternary pediatric medical center. Demographic information, clinical characteristics and intra-operative complications were obtained from retrospective review of the medical record. Structured telephone interviews using a survey tool adapted from the Self Evaluation of Breathing Questionnaire and the PEDI-EAT 10 collected data on long-term symptomatic improvement.

Two patients were male, and two were female. Age at time of operation ranged from 9 to 38 months. In all patients, the operative indication was dysphagia in the setting of an ARSA with no additional symptom etiology of symptoms identified. No patients required postoperative intensive care unit admission. Hospital length of stay was 2 days for all patients. There were no intra-operative complications. All patients experienced long-term symptomatic improvement following their operation with 100 % reporting symptomatic improvement and none reporting symptom recurrence.

Conclusion

A right collar incision is a feasible and effective approach for subclavian to carotid transposition of ARSA in pediatric patients and should be considered for operative intervention of symptomatic aberrant right subclavian arteries.

Introduction

Phyllodes tumors are rare fibroepithelial neoplasms. They represent less than 0.5–1% of all primary breast tumors and their presentation is even rarer in children.

Case description

A 15-year-old female with Autism Spectrum Disorder (ASD) was seen in the outpatient surgery clinic due to bilateral breast masses. She underwent core-needle aspiration. biopsy of both masses and the results were suspicious of fibroadenomas. Two days later the patient presented to the emergency room with ulceration and bleeding from the left breast mass. She was scheduled for an urgent nipple-sparing mastectomy, which was uneventful. The pathology revealed a multifocal, aggressive type tumor with moderate stromal cellularity, moderate stromal atypia and high mitotic index, corresponding to a borderline phyllodes tumor. Based on this result, she underwent an elective right nipple-sparing mastectomy. The pathology of the right breast mass was similar to the left breast mass.

Conclusion

Core-needle aspiration biopsy is not reliable for the diagnosis of phyllodes tumor. If a phyllodes tumor is diagnoses in one breast, a contralateral breast mass should be considered a phyllodes tumor until proven otherwise.

Introduction

Duodenum inversum is a rare congenital anatomical variant and presents a myriad of clinical challenges. This case presentation highlights a unique case of symptomatic duodenum inversum diagnosed in the second decade of life requiring operative intervention.

Case presentation

We present the case of a thirteen-year-old male who presented with one year of intermittent abdominal pain with associated bilious emesis. Diagnostic work-up included multiple abdominal ultrasounds and abdominal radiographs which only revealed constipation. Despite adequate bowel regimen, patient's symptoms persisted. Duodenum inversum was diagnosed on a fluoroscopic upper gastrointestinal study and the patient was subsequently taken to the operating room to evaluate for concurrent midgut volvulus and malrotation. A diagnostic laparoscopy revealed fibrotic adhesive bands contributing to partial duodenal obstruction. Surgical resection of these bands alleviated the obstruction and resolved the patient's symptoms with no recurrence noted on post-operative follow-up.

Conclusion

In cases of duodenum inversum, recognition through diagnostic tools like fluoroscopic upper gastrointestinal studies is critical. While initial medical management may suffice for mild cases, surgical intervention remains the cornerstone for severe cases, particularly when obstruction is evident, emphasizing the necessity of tailored approaches based on clinical severity.

Introduction

Thoracoscopic superior-segment (S6) segmentectomy is a simple procedure. However, it is applicable only when an interlobar fissure approach is applied, which can sometimes be challenging in cases with a fused interlobar fissure. Herein, we discuss our experience in performing thoracoscopic left S6 segmentectomy in a pediatric patient with congenital pulmonary airway malformation (CPAM) involving a fused interlobar fissure.

Case presentation

A 5-year-old girl was prenatally diagnosed with a cystic mass in her left thorax and suspected to have a congenital pulmonary airway malformation. No postnatal respiratory symptoms were observed; elective surgery was performed after reaching the age when differential lung ventilation became feasible. Multiportal complete thoracoscopic surgery was performed under general anesthesia. A large cyst was identified in S6, and the interlobar fissure was fused; thus, we performed a fissure-last S6 segmentectomy using a posterior approach. We first manipulated the vein by retracting the lower lobe anteriorly thus enabling us to identify the segmental veins (V6), and subsequently divide V6a and V6c. We divided segmental arterial branches and separated the intersegmental plane between the S6 and basal segment along V6b using staplers. Lastly, the fused interlobar fissure was divided using a stapler. No postoperative leakage was observed, and the chest tube was removed on the evening of the day of surgery. Pathological examination revealed CPAM type 1 with no malignant findings. The patient survived and is living without any complications 16 months postoperatively.

Conclusion

The fissure-last method may be an alternative treatment for patients with incomplete interlobar fissures.

Introduction

Neonatal sacrococcygeal teratoma (SCT) results in poor outcomes if it is associated with preterm delivery and an immature histology. Furthermore, some patients experience recurrence, generally one to two years later. We herein present a preterm infant case of an immature SCT with metastatic recurrence within two months of the initial resection.

Case presentation

A neonate with prenatally diagnosed SCT was born at 28 weeks’ gestation with a birth weight of 2337 g. The tumor measured 18 × 9 × 8 cm in diameter, and it was predominantly solid and hypervascular. Postnatally, the patient presented with anemia and coagulopathy requiring a massive transfusion presumably due to intratumoral hemorrhage. Surgical resection was performed at 12 days of age. The tumor was histologically diagnosed to be an immature teratoma. The alpha-fetoprotein (AFP) levels decreased but increased thereafter on postoperative day 68. Computed tomography revealed both local recurrence and bilateral lung metastases. During chemotherapy, all secondary lesions were enlarged. We resected the lesion in the right lung, which was histologically diagnosed as mature teratoma. Even after switching chemotherapy protocols, the residual lesions were progressive. We resected the lesion from the left lung. Following another course of chemotherapy, we resected the presacral recurrent tumor, which was histologically confirmed as a mature teratoma. The patient has not shown any signs of a re-recurrence for 6 years.

Conclusion

This case highlights the fact that immature sacrococcygeal teratomas can recur and metastasize as early as two months after the initial resection.

Introduction

Superior mesenteric artery syndrome (SMAS) is a complete or incomplete obstruction of the third portion of the duodenum caused by compression between the aorta and the superior mesenteric artery (SMA). Some diagnostic strategies and treatments exist for adults; however, in neonates, these are controversial because the condition is uncommon.

Case presentation

A 4-day-old male infant with a birth weight of 2630 g was referred to our hospital with a 2-day history of bilious vomiting. Ultrasonography and contrast enema revealed no malrotation or midgut volvulus. On day 7 of life, an upper gastrointestinal series with contrast revealed obstruction of passage in the third portion of the duodenum. Congenital duodenal stenosis was suspected, and exploratory laparotomy was performed on day 9 of life. The SMA compressed and narrowed the third portion of the duodenum. The duodenum was mobilized and the ligament of Treitz was divided using the technique described by Strong, which released the duodenal compression. A feeding tube was successfully inserted through the duodenum. No duodenal web was observed and SMAS was suspected. The patient's postoperative course was uneventful and SMAS was diagnosed. Complete oral intake was achieved on postoperative day 20, and the patient was discharged on postoperative day 30.

Conclusions

Neonatal SMAS is a rare but possible cause of duodenal obstruction. The procedure reported by Strong is useful in neonatal cases.