Pituitary gigantism: a retrospective case series

Author(s):  
Ana L. Creo ◽  
Aida N. Lteif

AbstractPituitary gigantism (PG) is a rare pediatric disease with poorly defined long-term outcomes. Our aim is to describe the longitudinal clinical course in PG patients using a single-center, retrospective cohort study.Patients younger than 19 years diagnosed with PG were identified. Thirteen cases were confirmed based on histopathology of a GH secreting adenoma or hyperplasia and a height >2 SD for age and gender. Laboratory studies, initial pathology, and imaging were abstracted.Average age at diagnosis was 13 years with an average initial tumor size of 7.4×3.8 mm. Initial transsphenoidal surgery was curative in 3/12 patients. Four of the nine patients who failed the initial surgery required a repeat procedure. Octreotide successfully normalized GH levels in 1/6 patients with disease refractory to surgery (1/6). Two out of five patients received pegvisomant after failing octreotide but only one patient responded to treatment. Five patients were ultimately treated with radiosurgery or radiation patients were followed for an average of 10 years.PG is difficult to treat. In most patients, the initial transsphenoidal surgery failed to normalize GH levels. If the initial surgery was unsuccessful, repeat surgery was unlikely to control GH secretion. Treatment with octreotide or pegvisomant was successful in less than half the patients failing surgery. Radiosurgery was curative, but is not an optimal treatment for pediatric patients. Despite the small sample, our study suggests that the treatment outcome of pediatric PG may be different than adults.

2016 ◽  
Vol 17 (3) ◽  
pp. 242-253
Author(s):  
M. Greenham ◽  
V. Anderson ◽  
J. Campbell ◽  
P. Monagle ◽  
M.H. Beauchamp

Previous studies investigating long-term outcomes in children following vitamin B12 deficiency during infancy have been limited to IQ or clinical observation. This paper seeks to describe comprehensive neuropsychological profiles in a case series of school-aged children who were treated for infantile vitamin B12 deficiency. This was a retrospective case series of seven children who were treated for vitamin B12 deficiency during infancy and aged 5 to 16 years at the time of testing. While most children had age-expected intellectual performance, the distribution of the sample was skewed to the lower end of the normal range. Furthermore, children were found to have impairments in a number of neuropsychological domains, most common were attention and memory, followed by executive function. These results suggest that while neurological symptoms quickly resolve following treatment, these effects on early brain development may disrupt brain maturation and have the potential to impact on later development.


2020 ◽  
Vol 220 (3) ◽  
pp. 736-740 ◽  
Author(s):  
Julia F. Kohn ◽  
Alexander Trenk ◽  
Woody Denham ◽  
John G. Linn ◽  
Stephen Haggerty ◽  
...  

2020 ◽  
pp. 112067212092645
Author(s):  
Tina Felfeli ◽  
Kamiar Mireskandari ◽  
Asim Ali

Purpose To report the characteristics and longitudinal visual outcomes of traumatic cataracts and retinal detachments in children with self-inflicted injury. Methods A retrospective case series of pediatric patients at a tertiary care center who sustained ocular trauma due to self-inflicted injury between 2000 and 2014. Results A total of 11 children, all with an intellectual disability and a mean age of 10 ± 4.8 years (range, 2.8–16.2), were identified with traumatic cataract secondary to ocular self-inflicted injury over the study period. Five eyes had a concurrent unilateral retinal detachment. Over the course of follow-up, one additional eye developed a cataract and five eyes developed a retinal detachment. Patients underwent an average of 2.5 ± 2.3 procedures and 17.1 ± 10.7 eye examinations over 11.7 ± 4.2 years of follow-up; 36% required general anesthesia for examination. Visual acuity was improved or preserved in 77% of the eyes that underwent surgical interventions, but was worse in the remaining cases due to development of traumatic retinal detachment. The use of protective helmets, eye shields, and immobilizing orthoses were essential in management of active self-inflicted injury. Conclusion Traumatic cataracts and retinal detachments due to self-inflicted injury may cause severe visual loss. Visual prognosis although poor in children with severe intellectual disabilities may be optimized with risk awareness for early detection and customized interventions.


2018 ◽  
Vol 21 (1) ◽  
pp. 49-53 ◽  
Author(s):  
Derek Yecies ◽  
Paul Graham Fisher ◽  
Samuel Cheshier ◽  
Michael Edwards ◽  
Gerald Grant

OBJECTIVEPrimarily metastatic juvenile pilocytic astrocytoma (JPA) is rare, likely representing 2%–3% of all cases of JPA. Due to the rarity of primarily metastatic JPA, there is currently no standard treatment paradigm and the long-term outcomes are not fully known. The goal of this case series was to add to the current understanding of this disease process.METHODSThe authors searched a comprehensive database of pediatric patients with brain and spinal cord tumors treated at Lucile Packard Children’s Hospital from 1997 to 2016 and identified 5 patients with primarily metastatic JPA. A retrospective chart review was performed and details of the patients’ treatment and clinical course were recorded for further analysis.RESULTSFor the 5 patients with primarily metastatic JPA, the mean follow-up period was 12.3 years. All patients in our series had biopsies or subtotal resections and upfront treatment. Three patients were treated with chemotherapy alone, one was treated with chemotherapy and radiotherapy, and one was treated with radiotherapy alone. Four patients had stable disease after initial treatment, and one patient had multiple episodes of progressive disease but underwent successful salvage therapy and has had stable disease for 19 years. One patient died of an intracerebral hemorrhage 10 years following initial radiation treatment believed to be secondary to radiation vasculopathy.CONCLUSIONSEvaluation of the entire neuraxis should be performed in all instances of initial JPA diagnosis to properly assess for primarily metastatic disease. Many patients with primarily metastatic JPA will have stable disease after upfront treatment, although the higher rate of stable disease found in this series relative to other reports is likely secondary to the small sample size.


Medicina ◽  
2021 ◽  
Vol 58 (1) ◽  
pp. 19
Author(s):  
Moushmi Patil ◽  
Jodhbir S. Mehta

Background and Objectives: To report the long-term outcomes of patients with refractory Vernal Keratoconjunctivitis (VKC) who underwent surgical excision of giant papillae (GP) with mitomycin C (MMC) 0.02% and amniotic membrane transplantation (AMT). Materials and Methods: This is a retrospective interventional single-center case series including five eyes of four patients who had refractory, symptomatic VKC with GP, along with corneal shield ulcers and/or punctate epithelial erosions. They underwent surgical excision of GP with MMC 0.02% alone (1 eye) or with MMC 0.02% and AMT (4 eyes). Their long-term visual and surgical outcomes were studied. Results: All subjects were male with bilateral involvement and mean age of presentation 9.8 years. The surgery was uneventful in all cases. Amongst the four eyes which underwent MMC with AMT, only one eye demonstrated papillary regrowth requiring repeat surgery. Postoperative follow-up ranged from 59 to 77 months (median 66 months). Four patients had the best corrected visual acuity (BCVA) >/= 6/9.5. One patient had BCVA 6/15 at the final follow-up due to the presence of anterior corneal stromal scar and poor ocular surface. Conclusions: Surgical excision of GP in combination with MMC and AMT, in refractory VKC, is a good treatment option with better clinical outcomes over a longer follow-up.


2020 ◽  
Vol 19 (2) ◽  
pp. 134-142
Author(s):  
Albert M Isaacs ◽  
Yarema B Bezchlibnyk ◽  
Jarred Dronyk ◽  
Geberth Urbaneja ◽  
Heather Yong ◽  
...  

Abstract BACKGROUND Endoscopic resection of colloid cysts has gained recent widespread practice. However, reported complication and recurrence rates are variable, possibly, in part, because of a lack of consistency with reporting of the extent of cyst capsule removal. OBJECTIVE To present the long-term outcomes of endoscopic resection of third ventricle colloid cysts without complete capsule removal and propose a grading system to allow consistent description of surgical outcomes. METHODS A retrospective review of 74 patients who underwent endoscopic resection of symptomatic third ventricle colloid cysts between 1995 and 2018 was performed. Kaplan-Meier analyses were used to assess recurrence-free survival rates. RESULTS Median patient age and cyst diameter were 48.0 (13.0-80.0) yr and 12.0 (5.0-27.0) mm, respectively. Complete emptying of cyst contents with capsule coagulation was achieved in 73 (98.6%) patients. All patients improved or remained stable postoperatively, with a median follow-up duration of 10.3 (0.3-23.7) yr. Radiographic recurrence occurred in 6 (8.1%) patients after their initial surgery, 5 (6.8%) of whom underwent redo endoscopic resection. No major complications or mortality was encountered at primary or recurrence surgery. CONCLUSION Endoscopic resection of third ventricle colloid cysts without emphasizing complete capsule removal is a viable option for successfully treating colloid cysts of the third ventricle. Long-term follow-up demonstrates that it is associated with low risks of complications, morbidity, mortality, and recurrence. The proposed extent of the resection grading scheme will permit comparison between the different surgical approaches and facilitate the establishment of treatment guidelines for colloid cysts.


Gut ◽  
2014 ◽  
Vol 63 (Suppl 1) ◽  
pp. A162.3-A163
Author(s):  
P Harrow ◽  
H Kwok ◽  
G Parkes ◽  
P Premchand

2020 ◽  
Author(s):  
Satoru Hashimoto ◽  
Hiroki Sato ◽  
Ken-ichi Mizuno ◽  
Kazuya Takahashi ◽  
Masafumi Takatsuna ◽  
...  

Abstract Background: Gastric tube carcinoma (GTC) after esophagectomy has been observed increasingly in recent years after advanced treatment of esophageal carcinoma. Surgical removal of the reconstructed gastric tube is not usually preferred; however, endoscopic submucosal dissection (ESD) is greatly anticipated to be a promising option. This study investigated the efficacy and safety of ESD for GTC. Methods: This study was designed based on a retrospective survey performed at our single center between 2003 and 2018. Patients with GTC were treated with ESD and followed-up according to the policy of our institution. The curability of ESD was evaluated histologically according to the Japanese gastric cancer treatment guidelines. Patient characteristics, outcomes of ESD procedures, and long-term outcomes were analyzed. Results: A total of 31 patients (2 females; median age, 73 years) with 45 GTC cases were treated with ESD. The mean period from esophagectomy to the diagnosis of GTC was 10.6 years. The only adverse event due to ESD was bleeding (2 patients: 6.5%). No fatal events, such as perforation, occurred. The rates of complete resections and curative resections were 80.6% and 48.4%, respectively. The 3-year and 5-year overall survival rates were 67.6% and 47.7%, respectively. The 3-year and 5-year disease-specific survival rates were 100% and 92.9%, respectively. One patient died of GTC and 14 patients died of other diseases, including five primary carcinomas. Conclusions: ESD for GTC can be a safe treatment and contribute to positive long-term outcomes. Long-term regular gastroscopy is required for early detection. Moreover, additional care should be given to GTC patients who are at high risk for other primary carcinomas or comorbidities.


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