Surgical management of neonatal severe hyperparathyroidism.

BACKGROUND: Neonatal severe hyperparathyroidism (NSHPT) is a rare disease that can be lethal. Most patients require parathyroidectomy. OBJECTIVE: Report experience in managing this severe disease. DESIGN: Retrospective chart review of case series. SETTING: Tertiary health care center. PATIENTS AND METHODS: We reviewed data on patients managed for NSHPT from June 2001 to January 2023. Demographic, clinical, and follow-up data were collected, and descriptive data were generated. MAIN OUTCOME MEASURES: Pre- and postoperative levels of parathyroid hormone (PTH) and serum calcium, and effect of autotransplantation. SAMPLE SIZE: 19. RESULTS: The 13 males and 6 females had a a mean age of 46 days at referral. The mean preoperative parathyroid hormone (PTH) and serum calcium levels were 996 ng/L and 4.54 mmol/L, respectively. Twelve patients underwent ultrasonography preoperatively. Of these, six had prominent glands, while no glands were seen in the other six. A Sestamibi scan was done for 15 patients, of which nine showed negative results and six showed positive results, with three glands observed in the neck and three in the sublingual area. Nineteen patients underwent renal ultrasonography, with nine showing nephrocalcinosis. The mean age at surgery was 5.2 months. Total parathyroidectomy (four glands) was performed in 17 patients, and 15 underwent concurrent auto-transplantation. One patient had three glands removed, in addition to auto-transplantation. Another underwent single gland excision as a redo-surgery after previous surgery elsewhere. The mean postoperative follow-up duration was 6 years. The mean postoperative PTH and calcium levels were 25 ng/L and 1.64 mmol/L, respectively. Ultimately, all the patients were required to initiate calcium and vitamin D supplements, except for two patients who had undergone auto-transplantation. Molecular genetic screening of the calcium-sensing receptor gene reported likely pathogenic/pathogenic mutations in 16 of 19 patients (13 were homozygous, two were heterozygous, one was negative, and data was unavailable for the remaining three patients). CONCLUSIONS: Surgical treatment of NSHPT is effective. Preoperative radiological localization studies did not impact the treatment plan. Auto-transplantation proved ineffective in maintaining independence from medical supplements. LIMITATIONS: The retrospective nature of the study may imply inaccuracybut since the data are gathered from electronic medical records, we believe it is highly accurate. The small sample size limits generalizability.

Implementation of Enhanced Recovery After Surgery protocols for gastrostomy tube insertion in patients younger than 14 years of age: a retrospective cohort study.

BACKGROUND: Enhanced recovery after surgery (ERAS) protocols have improved treatment outcomes and have standardized patient care. OBJECTIVES: Identify the benefit of introducing the ERAS protocol for feeding after gastrostomy insertion with or without Nissen fundoplication, the effects on the time of reaching the full feeds the length of stay single-center experience, and complications associated with early feeding protocols. DESIGN: Retrospective cohort study SETTING: Tertiary hospital METHODS: The study review included cases performed between 2015 and 2021 by four surgeons, and cases performed in 2022 by all surgeons using ERAS feeding protocol (P) in a tertiary hospital. MAIN OUTCOME MEASURES: Comparison the mean and mode of the length of stay (LOS) and the time until the patient reached full feed (TFF). SAMPLE SIZE: 224 patients; 181 by the four surgeons and 43 cases by the ERAS protocol group. RESULTS: The difference in the ERAS protocol from the four surgeons in TFF and LOS was statistically significant (P<.001). There was no noticeable difference in postoperative complications after introducing the ERAS protocol. CONCLUSION: ERAS improved the TFF and decreased the LOS without any increase in procedure complications. Increasing bed utilization and reducing costs were two benefits of reducing LOS at our hospital. LIMITATIONS: Single-center study, which may not be generalizable. Multiple comorbidities. Travel time from different parts of the country could impact LOS. Retrospective and thus dependent on the accuracy of the information in file notes. CONFLICT OF INTEREST: None.

Management of twin reversed arterial perfusion sequence: eight cases over 13 years.

BACKGROUND: Twin reversed arterial perfusion (TRAP) sequence is a rare condition that affects primarily monozygotic monochorionic twin pregnancies in which a normal twin acts as a pump (donor) for an acardiac recipient (perfuse) twin. OBJECTIVE: We report our experience over the last 13 years at a tertiary health care center. DESIGN: Descriptive, retrospective case series SETTING: Tertiary health care center PATIENTS AND METHODS: All TRAP cases managed between the years 2009 and 2022 at our Fetal Diagnosis and Therapy Center were included. Data recorded included demographic and clinical information which was used to generate descriptive data. Patients were managed by a multidisciplinary team with variable interventions. MAIN OUTCOME MEASURE: Survival of normal twin SAMPLE SIZE: Eight RESULTS: Eight pregnant women with TRAP syndrome were managed at our center during that period. One was monozygotic monochorionic and the others were monochorionic diamniotic. Median maternal age at presentation was 27 years and median gestational age at diagnosis was 23 weeks. All were diagnosed with ultrasound (US) imaging. Three were managed with bipolar ligation of the cord of the acardiac twin under general anesthesia, one US-guided (single port) and 2 fetoscopic (2 ports) with a median operative time of 39 minutes. The last five cases were managed with US-guided radiofrequency ablation (RFA) under local anesthesia, one needed 2 sessions, 1 week apart. The median duration of the RFA procedure was 23 minutes. There were no complications and all had viable normal babies born at a median of 32 weeks of gestation (6 C-section, 2 spontaneous membrane rupture). CONCLUSIONS: Acardiac twin cord ligation and RFA are feasible and safe options with excellent outcome for TRAP syndrome. RFA may be preferable owing to its less invasiveness under local anesthesia. LIMITATIONS: None, given the rarity of the disease and the study design. CONFLICT OF INTEREST: None.

Renal lymphangiectasia in pediatric population: case series and review of literature.

Renal lymphangiectasia is a rare disorder where perirenal, parapelvic, and/or intra-renal lymphatics are dilated. The clinical presentation of renal lymphangiectasia can range from asymptomatic to renal failure. Ultrasound, computed tomography and magnetic resonance imaging have been used for the diagnosis. Management of such cases varies from conservative to nephrectomy. We report three cases of pediatric renal lymphangiectasia that were managed by recurrent sclerothera-pies and medical supportive treatment at our institution. A literature review is also presented. To the best of our knowledge, there are 83 reported cases in the literature, 60 adults and 23 pediatrics.

Thyroid surgery in 103 children in a single institution from 2000-2014.

BACKGROUND: Data on thyroid surgery in children are scarce. OBJECTIVE: Analyze outcome data on thyroid surgery in a pediatric population. DESIGN: Medical record review. SETTING: Tertiary health care institution. PATIENTS AND METHODS: We collected demographic and clinical data on patients 18 years or younger who had thyroid surgery in the period 2000 to 2014. Descriptive data are presented. MAIN OUTCOME MEASURES: Indications for thyroidectomy, thyroid pathology, complications, length of stay, and radioactive iodine treatment and recurrences. SAMPLE SIZE: 103. RESULTS: Of 103 patients who underwent 112 thyroidectomy procedures, 80 (78%) were females and the mean age at operation was 13.2 years. and 17 (16%) were associated with multiple endocrine neoplasia type 2. There was no history of radiation exposure. Eighty-one patients (78%) had fine needle aspiration (FNA) which correlated with the final histopathology in 94% of cases. Sixty-six patients (64%) had malignant cancer (61 papillary), 44 (74.6%) of 59 patients who had neck dissection had lymph node metastasis and 7 (11%) had distant metastases to the lung. Procedures included total thyroidectomy (50%), hemithyroidectomy (17%), completion (31%), and subtotal thyroidectomy (2%). Twenty-three patients (22%) developed hypocalcemia (3 permanent) and 6 (5.8%) had unilateral recurrent laryngeal nerve injury (3 permanent). Patients were followed up for a mean duration of 71.7 months (median 60 months). Of 66 patients with thyroid cancer, 43 (65%) received radioactive iodine, and 10 (15%) had recurrence. CONCLUSION: Malignancy is the commonest indication for thyroid surgery in children and FNA is highly diagnostic. Hypocalcemia and recurrent laryngeal nerve injury are significant complications. The recurrence rate in thyroid cancer is 15%. LIMITATIONS: Retrospective. CONFLICT OF INTEREST: None.

Ventriculoperitoneal shunt peritoneal catheter knot formation.

The ventriculoperitoneal (VP) shunt is a common procedure in pediatric neurosurgery that carries a risk of complications at cranial and abdominal sites. We report on the case of a child with shunt infection and malfunction. The peritoneal catheter was tethered within the abdominal cavity, precluding its removal. Subsequently, laparoscopic exploration identified a knot at the distal end of the peritoneal catheter around the omentum. A new VP shunt was inserted after the infection was healed. This type of complication occurs rarely, so there are a limited number of case reports in the literature. This report is complemented by a literature review.