Seminars in Neonatology

Abstract Background Diagnosis and management of pediatric cancer develop a major life event that might impact psychosocial functioning and quality of life (QoL) even long after the initial therapy has been completed. Treatment outcomes have been measured in terms of survival time, but they also significantly impact survivors’ quality of life. The pediatric differentiated thyroid carcinoma survivors’ QoL has rarely been evaluated. This study aims to translate and validate the Indonesian version of the ThYCA-QoL questionnaire. Results The median age of 105 eligible survivors was 20.88 years old. Eighty percent of the survivors were female, were married or in a relationship (86.7%), and had paid jobs or were full-time students (71.7%). The median follow-up time was 64.82 months. Cronbach-α co-efficient was > 0.70 for psychological, concentration, throat, and mouth problems. For sympathetic, neuromuscular, voice, and sensory, the scores were < 0.70, where a multi-trait scaling analysis showed that all item correlations were > 0.40. Validity was assessed using the Pearson correlation coefficient for y-QoL with r > 0.60 and p < 0.01. Conclusions The Indonesian ThYCA-QoL questionnaire is a reliable and valid tool to evaluate pediatric patients’ QoL after treatment. This simple assessment tool can be used to evaluate and manage pediatric thyroid cancer patients’ HRQoL.

Abstract Background Non-hypertrophic gastric outlet obstruction (GOO) in children is rare and usually presents beyond infancy. Aetiology is variable, and management is not well established due to its rarity. Methods We aimed to conduct a descriptive study of these cases within our geographic area. It is a retrospective chart review of all children with non-hypertrophic gastric outlet obstruction presenting to three hospitals in northwest Syria during the period 2019–2023. Demographics, presentation, and management data were extracted. Results Twelve cases were identified, eight males, with age range 1–12 years and follow-up range 3–42 months. Eight patients had a history drinking battery acid and one with ingestion of high-dose ibuprofen. The other three had no clear aetiology. Five patients improved by endoscopic balloon dilatations (EBD), and seven needed surgeries. All those who had successful EBD were treated within 3 weeks of ingestion. Conclusion Non-hypertrophic GOO in children is rare and mostly related to caustic material ingestion. Treatment by endoscopic balloon dilatation is likely to be successful if done within 3 weeks from caustic ingestion. After that period, often surgery is needed.

Abstract Background This study reviews a university pediatric surgery practice for the incidence of stuck subcutaneous port catheter (SSPC) removal and the efficacy of a guidewire extraction technique. Methods We reviewed all central catheter removals between 2018 and 2020. A SSPC was defined as resistance to removal after dissection of the subcutaneous tissue with signs of impending device fracture. Details of the catheter duration, initial diagnosis, and medications administered through the device were all recorded. Results One hundred eight patients underwent catheter removal and six were defined as SSPCs. Catheter in situ time ranged from 35 to 96 months. All six patients underwent the guidewire extraction technique and were removed in their entirety without the need for endovascular intervention or venotomy. Conclusions Pediatric patients who have a SSPC should undergo the guidewire extraction technique.

Abstract Background Neonatal bowel perforations pose a significant disease burden for pediatric surgeons around the world. However, very little is known about these perforations in low-income settings. This study aims to investigate the epidemiology of neonatal perforations at a tertiary hospital in Uganda. Results Twenty neonates with bowel perforation who were admitted to a single national referral hospital from May 2020 to April 2021 were included. Fifty-five percent (n = 11) of the neonates in this cohort were male, and 16 were term with birth weight above 2.5 kg. Thirteen were below 1 week of age and all maternal ages were less than 40 years. Pneumoperitoneum was the most common finding on erect abdominal X-ray and colon was the frequent site of perforation. Forty percent of the babies in this cohort had blood group O+. Fifty-five percent of our patients died before discharge. Conclusion Outcomes for neonatal bowel perforations are still dismal. Health workers taking care of neonates should have a high index of suspicion for neonatal gastrointestinal perforations.

Abstract Background Double congenital tracheoesophageal fistula (TEF) without esophageal atresia, known as double H-type fistula, is an extremely uncommon malformation. Due to its nonspecific symptoms and the small size of the fistula in infancy, H-type tracheoesophageal fistula is rarely diagnosed in the neonatal period and is also challenging to repair. Case presentation We report a rare case of double H-type tracheoesophageal fistula in a 2-day-old newborn, identified with a time-lapse and repaired separately. The diagnosis was established through repeated bronchoscopy, with methylene blue injected through an esophagoscope. A right thoracotomy was performed to undertake the surgical repair after cannulating the fistula with a vascular guide wire. Five months later, the infant, who had a history of recurrent episodes of coughing and shortness of breath, was diagnosed with acute respiratory distress, and a second TEF was detected by bronchoscopy. A cervical incision was used to repair the second H-type fistula. Conclusions Double H-type tracheoesophageal fistula should be considered while correcting a tracheoesophageal fistula, especially if the symptoms continue after the first operation. Repeated radiological contrast studies and bronchoscopy with methylene blue injected through an esophagoscope help the early diagnosis.

Abstract Background Extrahepatic bile duct tumor in neonates and infants is extremely rare. Fibroma in neonates and infants has been reported in different body parts but not in the bile duct. We report a 2-month-old girl with common bile duct (CBD) fibroma. Case presentation A 2-month-old girl presented with jaundice and pale stool for 15 days. Her perinatal period was uneventful. On examination, we found the girl deeply icteric and having hepatomegaly but no palpable lump. Laboratory investigations revealed direct hyperbilirubinemia with elevated liver enzymes. Ultrasonography revealed hepatomegaly with focal dilatation of the extrahepatic bile duct (2.1 × 0.5 cm). Our preliminary diagnosis was a type I choledochal cyst. Considering the clinical scenario, we planned for exploration without further imaging. On exploration, we found the liver blackish and a hard mass involving the common CBD and cystic duct. The gall bladder was filled with clear mucus. After excising the mass, the gall bladder, and CBD, we reconstructed the anatomy with Roux-N-Y hepaticojejunostomy. The postoperative period was uneventful except for minor bile leakage during the first four postoperative days, which stopped spontaneously. Histopathology revealed spindle-shaped fibrous tissue admixed with collagenous tissue, compatible with a fibroma. A liver biopsy showed the features of cirrhosis. However, liver function tests were normal 2 weeks after surgery. The baby was thriving at 6 months of follow-up, hepatomegaly was resolved, and there was no clinical or radiological sign of recurrence. Conclusions CBD fibroma is a rare cause of obstructive jaundice in neonates and infants. The earliest possible excision of the tumor and bile drainage restoration are necessary to halt the progression of liver damage.

Abstract Background Heteropagus twinning is a rare birth defect in 1 in million live births. Epigastric heteropagus is the most frequent type, in which a smaller dependent (parasite twin) is attached to the body of the host (autosite twin) to the thorax and umbilicus. Several congenital anomalies have been previously described in the host twin. We describe the first case of epigastric heteropagus twinning with dextrocardia and situs inversus in which a successful surgical separation is performed. Case presentation A 48-h-old female sex twins were presented to Bahawal Victoria Hospital. A careful anatomic study using contrast X-ray, magnetic resonance imaging, and echocardiogram showed epigastric heteropagus twinning and situs inversus in autosite twin. A successful surgical separation was performed, and the parasite twin was removed from the body of the autosite twin. Conclusions Antepartum screening detects congenital malformations; however, several pregnancies are unsupervised in underdeveloped countries. In the case of heteropagus twinning, careful surgical separation in the absence of complications leads to the favorable survival of the autosite twin.

Abstract Background Dermoid cysts are benign germ cell tumors with ectodermal and mesodermal components. It is a slow-growing lesion that commonly arises in the midline of the head and neck. The last location represented 7%, and the periorbital region was the most frequently arising area. True lateral neck dermoid cysts are rare. We present an unusual location of a dermoid cyst in a newborn. Case presentation We report an unusual location of a 10-day newborn giant dermoid cyst with multiple implantation sites, namely the shoulder, scapula, cervical, temporal, and occipital scalp. Successful surgical resection was achieved, and the patient was discharged after the removal of the stitches. Conclusion Dermoid cysts are unusual neoplasms with rare diagnoses at birth, but often in childhood. Concomitant implantation locations on the shoulder, neck, temporal, and occipital scalps are rare.

Abstract Background Hypospadias is the most common congenital abnormality of the urethra affecting live male births. The incidence is rising with the increasing pollution. During the last 50 years, developments in surgical instrumentation and optical systems allowed surgeons to increasingly undertake microsurgical procedures. Optical magnification is essential in pediatric surgery. Magnifying loupes are the most frequently used, although they often cause neck pain to the surgeon. Recent advances led to the development of a compact video microscope (VITOM; Karl Storz Endoscopy GmbH, Tuttlingen, Germany) that displays high-definition magnified images on a flat screen. Objective Evaluate VITOM as a potential substitute for loupes in complex open pediatric procedures and explore VITOM as an effective intraoperative teaching modality for open surgery. Methods Fifty-two patients enrolled in our study were divided into two groups: Group A (26): high magnification was used either (VITOM Karl Storz Endoscopy GmbH, Tuttlingen, Germany) or (Surgical microscope, Carl Zeiss Microscopy GmbH), along with microsurgical instruments. Vicryl 8-0 suture was used for urethroplasty. Group B (26): patients were operated upon by conventional instruments (fine tip but not microsurgical instruments), sutures, and magnification (3.5×). Vicryl 6-0 suture was used for urethroplasty, with loupes-assisted magnification. Results More complications were found in group B. Using VITOM was accompanied by less neck pain, enhanced visualization, and an enhanced teaching environment. Conclusion Operating with high magnification tools minimizes post-operative complications in hypospadias surgery. They enhanced identification of the penile anatomy and topography which facilitated reconstruction techniques as well as enhanced the comfort of the surgeon. Level of evidence I.

Abstract Background It is not often written in medical journals that preduodenal portal vein, biliary atresia, intestinal malrotation, and situs inversus totalis are all related. This is a rare association. Case reports A 2-month-old female infant had biliary atresia type III, situs inversus totalis, midgut malrotation, and a preduodenal portal vein. She had been operated on by the Kasai procedure (hepato-portoenterostomy). Discussion It is important to carefully look into the relationship between the preduodenal portal vein and biliary atresia because the patient is at risk of injury from this aberrant vein during operative intervention. Conclusion The relationship between biliary atresia and other congenital anomalies like preduodenal portal vein, intestinal malrotation, and situs inversus must be taken into consideration to avoid other diverse effects during surgical intervention.

Abstract Background Extralobar pulmonary sequestration–a congenital lung malformation characterized by nonfunctional lung tissue with its own visceral pleura and without a connection to the normal tracheobronchial tree–is often surgically resected given the potential for infectious complications. We report a case of a child with extralobar pulmonary sequestration in a rare and challenging intradiaphragmatic location, which made preoperative identification and planning difficult. Case presentation A 2-year-old boy presented for follow-up of a large left extralobar pulmonary sequestration initially diagnosed on an antenatal ultrasound. Follow-up imaging, including prenatal MRI and postnatal CT angiography, was inconclusive on the precise location of the extralobar pulmonary sequestration in relation to the diaphragm but did reveal a subdiaphragmatic arterial supply from a branch off the celiac trunk. Planned resection with diagnostic thoracoscopy revealed the mass to seemingly be below the diaphragm; however, subsequent abdominal laparoscopy identified it to be within the diaphragm. Once the supplying vessel off the celiac trunk was controlled and divided, the mass was circumferentially excised from the edges of the diaphragmatic muscle. The remaining diaphragmatic defect was then closed, and the patient did well postoperatively. Conclusions This case demonstrates the difficulty of making a definitive diagnosis of intradiaphragmatic extralobar pulmonary sequestration without operative intervention. CT angiography and identification of the sequestration’s arterial supply may not be conclusive in determining the precise location if in close proximity to the diaphragm.

Abstract Introduction Amyand hernia (AH) is a rare disease, so there are no standard strategies and there are many different aspects at each stage of its management. Based on our own experience, we encountered these differences even in a small number of cases and therefore sought to review the literature to highlight the diversity of approaches to this pathology. This review of the literature was not intended to describe the statistical findings found in the clinical case series, but rather to highlight the clinical and surgical difficulties of AH in children. Nevertheless, we conducted an introductory statistical study based on data from PubMed and Google Scholar to understand the global prevalence of AH. Materials and methods The search for the key terms Amyand hernia, Amyand’s hernia, and “children” between 2003 and 2023 resulted in 52 PubMed and 548 Google Scholar articles. Results After the exclusion of irrelevant studies, 101 articles were found. A total of 83 case reports describing 182 pediatric patients were used to understand the demographic distribution of this pathology. Given the impossibility of further comprehensive statistical analysis (due to heterogeneous data), a narrative design was used to describe the remaining aspects of AH management. Finally, three clinical cases demonstrated the mentioned aspects. Discussion As a result of the search, conclusions were drawn about the main difficulties in the management of AH in children, which were discussed.

Abstract Background Congenital megalourethra is a rare male genital malformation, with less than 100 cases reported in literature. Urethrocutaneous fistula, in turn, has only 65 cases described, which what makes the association between these two anomalies an even rarer condition. Case presentation The patient was male and born at 37 weeks of gestational age. At birth, the testicles were impalpable, and he had a penile malformation compatible with scaphoid megalourethra associated with congenital urethrocutaneous fistula, with urine output exclusively through a small orifice in the ventral region of the penis. When the child completed 7 months and 22 days, underwent the first surgery, laparoscopically. The first Fowler-Stephens was performed, and the gonadal vessels were ligated, with reconstruction of the penis. Nine months later, the second Fowler-Stephens procedure was performed, with bilateral orchidopexy and penoplasty to improve penis aesthetics. The urethra region was not approached. The postoperative period of the second surgery progressed uneventfully. Nowadays, the patient presents adequate urinary stream. Conclusion The scaphoid megalourethra associated with congenital urethrocutaneous fistula is an extremely rare pathology, with very few cases reported in the literature to date. The obstetric USG is important to increasing the chances of early diagnosis and treatment.

Abstract Background Chylolymphatic cysts are benign pathology in lymphatic vessels due to obstruction in the lymphatic system. Their incidence is still unknown. However, they account for approximately 3 to 9% of all pediatric lymphangiomas. Most of these lesions are asymptomatic and are discovered accidentally. They can be presented with nonspecific abdominal pain, vomiting, and changes in bowel habits. Rarely, chylolymphatic cysts can as intestinal obstruction, volvulus or even torsion. Case presentation A 3-month-old male presented to our unit with manifestations of acute intestinal obstruction. Abdominal examination revealed signs of generalized peritonitis and intestinal obstruction. Abdominal ultrasound revealed minimal turbid intraperitoneal collections and no motility of bowel loops. Plain erect abdominal radiograph revealed multiple small bowel air fluid levels. Abdominal exploration revealed a large chylolymphatic cyst causing acute intestinal obstruction. Excision of the cysts was done with preservation of adjacent bowel loop. The patient was discharged in good health on the 6th day postoperative. A chylolymphatic cyst with a diameter of 5 cm was identified by histological investigation. Conclusions Most chylolymphatic cysts in infants are asymptomatic, but they can cause fatal complications such as intestinal obstruction, volvulus, or even torsion.

Abstract Background Importance of helix valgus lies in the psychological and social effect it could have on the patient. Our objective was to assess the impact of otoplasty on the quality of life of children with prominent ears and to establish possible predictors of post-surgical outcomes. Material and methods A retrospective review of children with prominent ears who underwent otoplasty between 2016 and 2018 was conducted. Demographical information, medical history, characteristics of helix valgus, motivations for surgery, complications, and post-surgical satisfaction were collected. To assess changes in parent-reported quality of life and patient satisfaction following surgery, the validated Glasgow Children’s Benefit Inventory (GCBI) and Patient Outcomes of Surgery-Head/Neck (POS-Head/Neck) surveys were used, respectively. Results Fifty-three patients responded to the survey (64.6%). From those, 90.6% of parents reported an improvement in health-related quality of life after otoplasty, with a 24.6 ± 20.6 average score on the global GCBI scale. The post-surgical score on the POS-Head/Neck surveys was significantly higher than the pre-surgical score (p < 0.0001), suggesting an improvement in patient-reported aesthetic appearance. Analysis of possible predictive factors showed significant differences regarding age. Children over 10 years of age obtained significantly higher scores in both questionnaires. Conclusions Otoplasty has a clear positive impact on the quality of life of children with prominent ears. Age at the time of surgery appears to be a predictor of post-surgical outcomes. Our results would justify delaying surgery until 10 years old.