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Laparoscopic vascular surgery: four case reports.

PURPOSE: We report four cases of laparoscopic vascular procedures (two iliofemoral bypasses, one aortobifemoral bypass, and one aortoiliac endarterectomy) performed with a technique that does not require insufflation of the peritoneal cavity with gas. METHODS: Initially in the porcine model and later in patients, we developed a laparoscopic technique with a mechanical arm used to mechanically elevate the abdominal wall, creating a working cavity. Conventional vascular instruments were used in combination with laparoscopic devices. Five to seven ports were needed for the procedures (four to six ports of 0.5 to 1.5 cm and one incision of 4.0 cm). Exposure was maintained by use of roticulating fan retractors and laparotomy sponges. End-to-side technique was used for all graft anastamoses (cross-clamp time of 40 to 70 minutes). Distal anastamoses were performed through an open femoral incision. Operative time was 5 hours (iliac procedure) and 7 hours (aortic procedure). RESULTS: All patients were ambulating and tolerating a regular diet within 24 hours (iliac procedures) or 48 hours (aortic procedures) after operation. They were discharged home on the second (iliac procedures) or third (aortic procedures) postoperative day. Within 1 week they had resumed normal daily activities. No complications occurred, and no blood bank products were transfused. When compared with the standard surgical approach, gasless laparoscopic technique gave a faster postoperative recovery with less pain and decreased the risk of wound herniation, dehiscence, and infection. From a laparoscopic standpoint, the use of disposable instruments was minimized and the theoretical risk of CO2 embolism eliminated. CONCLUSION: These four cases demonstrate that laparoscopic vascular bypass procedures are feasible and that the accepted advantages of laparoscopy can be extended to patients undergoing vascular surgery.

Aged↗

Patch augmentation of the pulmonary artery with bioabsorbable polymers and autologous cell seeding.

OBJECTIVE: In recent years bioabsorbable synthetic or biologic materials have been used to augment the pulmonary artery or the right ventricular outflow tract. However, each of these polymers has one or more shortcomings. None of these patch materials has been seeded with cells. Thus, we have tested a fast-absorbing biopolymer, poly-4-hydroxybutyric acid, with autologous cell seeding for patch augmentation of the pulmonary artery in a juvenile sheep model. METHODS: Vascular cells were isolated from ovine peripheral veins (n = 6). Bioabsorbable porous poly-4-hydroxybutyric acid patches (porosity > 95%) were seeded on 3 consecutive days with a mixed vascular cell suspension (21.3 +/- 1.3 x 10(6) cells). Forty-five (+/- 2) days after the vessel harvest, 1 unseeded and 6 autologously seeded control patches were implanted into the proximal pulmonary artery. The animals received no postoperative anticoagulation. Follow-up was performed with echocardiography after 1 week and before explantation after 1, 7, and 24 weeks (2 animals each) for the seeded control patches and after 20 weeks for the nonseeded control patch. RESULTS: All animals survived the procedure. Postoperative echocardiography of the seeded patches demonstrated a smooth surface without dilatation or stenosis. Macroscopic appearance showed a smooth internal surface with increasing tissue formation. Histology at 169 days demonstrated a near-complete resorption of the polymer and formation of organized and functional tissue. Biochemical assays revealed increasing cellular and extracellular matrix contents. The control patch showed a slight bulging, indicating a beginning dilatation. CONCLUSION: This experiment showed that poly-4-hydroxybutyric acid is a feasible patch material in the pulmonary circulation.

Absorbable Implants↗

Laparoscopic colorectal surgery in the elderly.

BACKGROUND: The efficacy and safety of laparoscopic colorectal surgery have been demonstrated in several well designed trials in recent years. At the same time the experience in the field of laparoscopy in the elderly has increased. The aim of this study was to evaluate retrospectively the experience of laparoscopic colorectal surgery in patients over 75 years in our unit. DESIGN: Retrospective study. SETTING: Surgical Department, San Lorenzo Hospital, Valdagno (VI). SUBJECTS AND METHODS: All the patients over 75 years, out of a total number of 121 unselected patients, undergoing laparoscopic colectomy or laparoscopic abdomino-perineal resection between June 2002 and May 2005. MAIN OUTCOME MEASURES: Postoperative mortality, operating time, conversion rate, morbidity, time of discharge from hospital. RESULTS: Six patients were converted to open surgery. 87 patients over 75 years underwent laparoscopic colorectal surgery. There was no postoperative death, in three cases a reoperation was done, because of postoperative intestinal obstruction in one case (over 75) and for anastomotic fistula in two (one over 75, after low anterior resection). Mean operative time was was 180 minutes (range 150-300). CONCLUSIONS: Laparoscopic colorectal surgery in elderly patients is a safe group of procedures for benign and malignant lesions of large bowel, resulting in a low rate of complications even if the time of operation is increased compared with open procedures. Postoperative pain and hospital stay, with return to normal life, are decreased compared with open surgery.

Aged↗

Modified posterior exenteration for ovarian cancer.

The operative description of a modified posterior exenteration along with operative findings, other operative procedures, postoperative course, and follow-up information are presented for 47 patients (37 primary cytoreduction, ten secondary cytoreduction). All had stage IIIC or IV epithelial ovarian cancer with pelvic disease encasing the reproductive organs, pelvic peritoneum, cul-de-sac, and sigmoid colon. In addition to modified posterior exenteration, all patients had multiple other procedures performed as part of the cytoreductive efforts. Forty-five (95.7%) had optimal (less than 2 cm) cytoreduction and 18 (38.3%) had complete cytoreductive surgery. Thirty-four patients were ultimately rendered continent of feces (25 primarily and nine after colostomy reversal). Nine patients (19.1%) had serious morbidity and one (2.1%) died postoperatively. The median follow-up for those undergoing primary cytoreduction was 13.3 months (6-84). Nineteen (51.4%) were alive at the time of writing, 16 (43.2%) were dead, and two (5.4%) were lost to follow-up. Modified posterior exenteration effectively removes all visible pelvic disease with acceptable mortality. Hence, even patients with the most advanced cases of ovarian cancer may attain optimal cytoreduction and become ideal candidates for adjunctive therapy, with improved survival or a chance for cure.

Adult↗

Clinical results in 87 patients treated for asymmetrical breasts. A follow-up study.

A retrospective study was made of 87 patients operated on between 1984 and 1990 for asymmetrical breasts. Thirty-two patients had 36 implants (13 also had breast reduction or mastopexy of the contralateral breast). Capsular contraction, Baker grade II-IV, was found in a third of the implants. The remaining 54 patients underwent reduction mammaplasty or mastopexy alone. 90% of the patients were satisfied with the result. There was no significant difference in the overall satisfaction between the patients who did or did not have implants. We nevertheless think that mammary implants should be avoided whenever possible in the correction of breast asymmetry. Even though there is no difference in patients' satisfaction, it is our experience that those with implants have more frequent follow-up consultations and are subject to more postoperative procedures than those corrected without implants.

Adolescent↗

Symptomatic nonparasitic hepatic cysts: options for and results of surgical management.

BACKGROUND: Management options for symptomatic nonparasitic hepatic cysts (SNHC) lack verification through comparative studies with respect to safety and long-term effectiveness. HYPOTHESIS: Open cystectomy is the treatment of choice for patients with SNHC. SETTING: University hospital department of surgery. PATIENTS: Data were retrospectively analyzed from the clinical charts of 34 patients (26 women and 8 men) undergoing surgery for SNHC from January 1, 1975, through January 1, 1999. Charts were obtained from the original hospital referral. MAIN OUTCOME MEASURES: Morbidity rates and long-term recurrence. We considered the following variables for analysis: age, sex, hepatic cyst location, diameter of the cyst at primary surgery, symptoms, surgical procedure, postoperative morbidity and mortality, length of postoperative hospital stay, and long-term outcome. RESULTS: The 34 patients underwent 47 operations for SNHC (mean diameter, 15.0 cm), with a mean follow-up of 50.0 months. Ten patients underwent open and 8, laparoscopic deroofing of the cyst. Enucleation of the cyst and hepatic resections were performed as primary procedures in 4 and 2 patients, respectively, and as secondary procedures in 6 and 7 patients, respectively. Two recurrences (25%) were found after laparoscopic deroofing and 3 (30%) after open deroofing. Two (50%) and 6 (100%) recurrences were found after cystojejunostomy and needle aspiration, respectively. No symptomatic recurrences occurred after 10 cystectomies and 9 hepatectomies. One operative death (3%) occurred; however, morbidity rates were 18% (6/34) and 15% (2/13) after primary and secondary surgery, respectively. CONCLUSIONS: These results support our policy of performing open radical procedures in the treatment of SNHC; cystectomy is performed for primary surgery and hepatic resections for recurrences and complications. Conservative procedures have shown higher rates of recurrence and the need for further surgery. Only further technological improvements will allow a systematic and safe use of laparoscopy for radical surgery for SNHC.

Adult↗

Enterocele is correctable using the Ripstein rectopexy.

PURPOSE: About one-third of the patients with rectal prolapse or rectal intussusception have concurrent enterocele at defecography. The purpose of this study was to evaluate the effect of the Ripstein procedure on the concurrent enterocele and to study the outcome of the procedure with respect to the patients' symptoms. METHODS: Twenty-two patients with enterocele and either rectal prolapse or rectal intussusception at defecography were treated using the Ripstein procedure. Postoperatively, the patients were evaluated with clinical examination (22 patients) and defecography (16 patients). RESULTS: None of the patients had recurrence of enterocele, rectal prolapse, or intussusception at postoperative follow-up. Continence was improved in 15 of 16 incontinent patients. Emptying difficulties were unchanged in eight patients, improved in five patients, and had deteriorated in four patients. CONCLUSIONS: Enterocele is corrected by using the Ripstein rectopexy. Persisting defecation difficulties after the Ripstein procedure are unlikely to be secondary to enterocele. The Ripstein procedure can be an alternative in the treatment of enterocele, as a majority of these patients also have rectal prolapse or rectal intussusception.

Defecation↗

[Endotracheal intubation in thoracic catheter peridural anesthesia].

After standard preparation, the catheter is preferably inserted in a sitting position. A test dose of 4 ml etidocaine 1% is injected, initially followed by 1 ml per segment of the total calculated analgesic dose. Analgesic spread, blood pressure, and pulse are checked every 2 min. If a sufficient effect is not reached within 20 min, a further 2 ml per segment is injected. The patient is sedated in the operating room with diazepam, droperidol, or chlorpromazine and receives 6-8 l O2 during spontaneous respiration via a Hudson mask. The epidural catheter is left in place for postoperative analgesia. Intubation is used only as required. Of a total of 52 patients, 31 (60%) did not require intubation, 24 (46%) were age 70 or older, and 31 (60%) were classified as ASA risk group III-IV. Thirty-three of all the procedures (63%) involved upper abdominal surgery (liver, gallbladder) and the majority of these received thoracic (Th 9-10) punctures, with the catheter inserted 5 cm in the epidural space; 88% were punctured medially and 12% laterally. The most frequent complications were bradycardia and hypotension. Respiratory arrest and aspiration were not seen. Thoracic epidural catheter anesthesia can be carried out with or without intubation and with a minimum of complications in selected procedures. Postoperative analgesia is an important advantage.

Adult↗

Lung biopsy with frozen section as a diagnostic aid in patients with congenital heart defects.

In eight patients with congenital heart defects and equivocal preoperative hemodynamic data, lung biopsy with frozen section was performed and quantitative morphometric analysis carried out to help select a palliative or corrective surgical procedure. During catheterization it had been possible in only six of the eight patients to enter the pulmonary artery and obtain pressure measurements; four patients had moderate to severe elevation of pulmonary vascular resistance whereas the other two had only mild elevation of pulmonary arterial pressure, but they were being considered for a Fontan procedure. The lung biopsy tissue was taken inflated and fixed inflated in hot glutaraldehyde. From cryostat sections arterial concentration was assessed relative to alveolar concentration, and the degree of arterial muscularity was assessed by wall thickness and extension of muscle; the more advanced Health-Edwards arterial changes were also evaluated. On the basis of the morphologic assessment, six of the eight patients underwent corrective surgery and two underwent a palliative procedure. Postoperative hemodynamic data available in four of the patients who underwent corrective surgery revealed either a significant reduction in pulmonary vascular resistance or return to a normal level of pulmonary arterial pressure, whereas no change has occurred in the one patient studied who underwent a palliative procedure. Assessment of pulmonary arterial development and structure on lung biopsy with frozen section proved helpful in deciding between a palliative and corrective surgical procedure.

Adolescent↗

Reconstruction of finger and elbow function after complete avulsion of the brachial plexus.

Simultaneous reconstruction of elbow and finger function with free muscle and nerve transfers after complete avulsion of the brachial plexus (nerve roots C5 to T1) and its long-term results are presented. The basic procedure combined free or vascular pedicle latissimus dorsi muscle transfer with reinnervation by the spinal accessory nerve to obtain elbow and finger flexion, intercostal nerve transfer of the radial nerve to activate elbow and wrist extensors, and suture of the supraclavicular nerve or intercostal sensory rami to the median nerve to restore hand sensibility. Six patients had some or all of these procedures. Postoperative follow-up ranged from 2 to 5 years. Elbow function was restored completely, and some finger flexion was achieved in all cases, although a dynamic splint was necessary to straighten the digits. Patients have continued to improve in grasp power and finger control. This procedure appears to be promising for the restoration of basic hand function in severely handicapped patients.

Accessory Nerve↗

Bilateral video-assisted thoracoscopic surgery for bilateral spontaneous pneumothorax.

OBJECTIVE: To review our experience with bilateral video-assisted thoracoscopic surgery (VATS) for the treatment of bilateral spontaneous pneumothorax (SP). DESIGN: Retrospective study followed by a telephone interview for follow-up. SETTING: Thoracic Surgery Department, Chest Diseases Hospital, Kuwait. PATIENTS AND INTERVENTIONS: Fifteen patients undergoing bilateral VATS for bilateral SP from 1994 to 1999. RESULTS: The mean age of the patients was 22.9 years (range, 17 to 34 years), and 14 were men. All patients were successfully treated using the bilateral video-assisted technique. Operative indications included simultaneous bilateral pneumothorax (n = 7) and contralateral recurrence of SP (n = 8). Twelve patients had primary SP. In the three remaining patients, simultaneous bilateral SP was secondary to sarcoidosis in two patients and histiocytosis X in one patient. Eleven patients had multiple blebs or bullae located in the upper lobes, and 4 patients had no blebs. All blebs or bullae were resected. All patients had gauze pleurodesis. The mean +/- SD operative time was 133.6 +/- 9.1 min. There were no perioperative complications and no deaths attributable to the procedure. Postoperative prolonged air leak occurred in three patients (20%). The mean drainage time was 3 days (range, 2 to 8 days). The mean postoperative hospital stay was 5 +/- 1.7 days. Mean follow-up was 3.3 years (range, 2 to 5 years) for all patients. Pneumothorax recurred in one patient with histiocytosis X after 1 month and required a reoperation on the right side. CONCLUSIONS: Bilateral VATS is a safe procedure in the treatment of simultaneous and nonsimultaneous bilateral SP. This avoids the need for subsequent operations.

Adolescent↗

Prophylactic minitracheotomy in lung resections. A randomized controlled study.

Thirty consecutive patients undergoing lung resections were randomized into two groups: Group A (n = 15) received minitracheotomy postoperatively and group B (n = 15) were control patients. Postoperative respiratory course was monitored by serial clinical assessments, chest x-ray examination, arterial blood gases, sputa bacterial cultures, and the patient's requirement and response to chest physiotherpy. The two groups were similarly matched in age (mean 58.5 years), smoking habits, pulmonary functions, and surgical procedures. Postoperative pulmonary complications of collapse/consolidation developed in 11 patients (two in group A and nine in group B) (p less than 0.03). Four patients (all in group B) required nimitracheotomy in addition to antibiotics and chest physiotherapy to treat their pneumonia. Chest physiotherapy requirement was less in group A than in group B, with a mean number of sessions of seven in group A and eight in group B and a mean total time of 92 minutes in group A and 112 minutes in group B. The mean duration of minitracheotomy was 4.13 days. Minor temporary symptoms resulted from the minitracheotomy in eight patients (42%) and included discomfort, voice changes, subcutaneous emphysema, and stridor. There was one case of long-term morbidity (5%)-skin scarring from wound infection at the site of the minitracheotomy. No postoperative deaths resulted. We conclude that the prophylactic use of minitracheotomy is safe and effective in decreasing postoperative respiratory complications in patients undergoing lung resections.

Adult↗

Surgical management of severe cocontraction, globe retraction, and pseudo-ptosis in Duane syndrome.

BACKGROUND: Correction of severe cocontraction and pseudo-ptosis present unique surgical challenges in patients with Duane syndrome. METHODS: We report four Duane syndrome patients with esotropia in primary position, poor abduction, and severe cocontraction causing limitation to adduction, globe retraction, and pseudo-ptosis. All were treated with partial tendon transposition of the vertical rectus muscles augmented with Foster fixation sutures and surgical weakening of the ipsilateral lateral rectus muscle. One patient had a large recession of the lateral rectus muscle, and in three patients, the lateral rectus muscle was inactivated by removing from the globe and attaching its insertion to the lateral orbital wall. RESULTS: Postoperatively, all patients were aligned within eight prisms diopters of orthotropia, had no face turn, and improved adduction and abduction. The two patients who had restriction to abduction on intraoperative forced ductions also had residual esotropia in primary position and underwent recession of the ipsilateral medial rectus muscle as a second procedure. Postoperative binocular single visual field was enlarged by 56 to 500% in the three patients who were tested preoperatively and postoperatively. Globe retraction and cocontraction were markedly relieved. Palpebral fissure widened 1.0 and 6.0 mm in two patients who had preoperative and postoperative measurements. CONCLUSION: In Duane syndrome patients, severe cocontraction, globe retraction, and limitation to adduction may improve if the lateral rectus muscle is maximally recessed or its insertion is inactivated from the globe. Partial transposition of the vertical rectus muscles augmented with Foster sutures improved the angle of esotropia in primary position and abduction. Medial rectus muscle recession is indicated when the passive forced duction test reveals moderate-to-severe restriction to abduction.

Adolescent↗

Video-assisted living donor hemihepatectomy through a 12-cm incision for adult-to-adult liver transplantation.

OBJECTIVES: There has been remarkable progress in recent technical innovations for laparoscopic hepatectomy. However, a laparoscopic procedure rarely has been indicated for donation of the liver in living-related liver transplantation (LRLT). Here, we described the technique and the outcome of video-assisted donor hepatectomy (VADH) for adult-to-adult LRLT. METHODS: For 13 donors in adult-to-adult LRLT, 3 types of major hepatectomy--right hemihepatectomy (3), and left hemihepatectomy, with or without the caudate lobe (10)--were performed through video-assisted procedures; surgical manipulation via ports or via a 12-cm incision and viewing through a laparoscope or through incision were combined and used. RESULTS: VADH was completed in 13 donors, with a median operation time of 363 +/- 33 minutes and a median blood loss of 302 +/- 191 mL. No complications specific to video-assisted procedures, postoperative bile leak, or bleeding were observed. The restoration of the liver function was smooth, and the use of an analgesic (median: 1.2 times) was reduced, compared with the historical control (median: 3.8 times) that underwent a standard donation of the liver. Currently, all donors are healthy and have returned to their previous activities. The grafts have been functioning well, excluding 3 recipients who succumbed to serious complications unrelated to the video-assisted procedure. CONCLUSION: We have shown a new method of VADH through a 12-cm laparotomy for adult-to-adult LRLT. This technique is as feasible as standard open donor hepatectomy, with less pain and with improved postoperative symptoms.

Adult↗

Outcomes of cataract surgery in monocular patients.

PURPOSE: To determine the best corrected visual acuity (BCVA) outcomes and surgical complications in a consecutive series of functionally monocular patients who had phacoemulsification and intraocular lens (IOL) implantation. SETTING: University-based referral practice, Los Angeles, California, USA. METHODS: The medical records of 93 monocular patients who had phacoemulsification and IOL implantation between December 1991 and February 1999 were reviewed. Patients were considered to be monocular if Snellen BCVA in their fellow eye (the eye not having surgery) was worse than 20/200. Recorded were demographic information, anesthesia method, additional surgery, IOL model, preoperative and postoperative BCVA, reason for poor vision in the unoperated eye, co-morbidities in the operated eye, intraoperative and postoperative complications, length of follow-up, and postoperative procedures. RESULTS: Mean patient age was 72 years and mean follow-up, 6 months. Thirteen patients (14%) were monocular from complications of surgery. Eighty patients (86%) were monocular because of medical eye conditions. Ocular co-morbidities were present in 75% of eyes having surgery. Preoperative median BCVA was 20/60. Median BCVA was 20/30 by the final scheduled follow-up examination and 20/25 by the final follow-up examination. Thirty-seven percent of eyes were correctable to 20/20 or better, and 67% were correctable to 20/40 or better. Two eyes lost BCVA by the final scheduled postoperative examination, and 3 eyes lost BCVA by the final follow-up examination. Final BCVA of worse than 20/40 was always related to preexisting macular or optic-nerve pathology. There were 16 intraoperative or early postoperative complications. The most common (3 eyes) was posterior capsule plaque that could not be removed by capsule polishing. Twenty eyes (22%) had subsequent surgical procedures, the most common being laser posterior capsulotomy. No patient who was monocular from surgical complications had similar problems with second-eye cataract surgery. CONCLUSIONS: Best corrected visual acuity, the standard measure of efficacy in cataract surgery, improved a median of 3 to 4 Snellen lines. The safety of the procedure, as evidenced by the small number of patients who lost BCVA or experienced surgical complications, was satisfactory. Functionally monocular patients are likely to have ocular co-morbidities, some of which may limit the final visual outcome of surgery.

Adult↗

Subconjunctival versus intrascleral mitomycin-C in trabeculectomy.

BACKGROUND AND OBJECTIVE: A randomized prospective comparison of sub-Tenon's administration of mitomycin-C (MMC) with intrascleral administration of MMC in trabeculectomy was performed to determine whether intrascleral application is superior to the standard sub-Tenon's application. PATIENTS AND METHODS: Twenty-four eyes of 23 patients were randomized to treatment with MMC (0.27 mg/ml for 5 minutes). The route of administration was subconjunctival in group A and intrascleral in group B. Preoperative and postoperative visual acuity (VA), intraocular pressure (IOP), number of medications, and complications were compared between the two groups. RESULTS: There was no statistically significant difference between the two groups preoperatively regarding VA, IOP, and number of medications. Postoperatively, IOP and number of medications required had decreased significantly in both groups. A significantly larger number of postoperative procedures were required in group B to control IOP (group A = 3, group B = 14, P = .002). CONCLUSION: Both methods of MMC application significantly decreased the IOP and the number of medicines required to control IOP after trabeculectomy. The intrascleral use of MMC resulted in the need for more surgery postoperatively (P = .002).

Aged↗

[Abdominal pain in patients undergoing radical cystectomy for bladder cancer].

OBJECTIVE: To analyze the incidence and causes of emergency admission for abdominal pain in patients submitted to radical cystectomy for bladder cancer. METHODS: A retrospective study was conducted on 176 patients who had undergone radical cystectomy for bladder cancer. The preoperative treatment, urinary diversion procedure, postoperative tumor stage, complications and adjuvant treatment required were analyzed. The patients had a follow-up of 43.7-58.3 months (mean 51). RESULTS: 18 patients required admission for abdominal pain; 7 for complete bowel obstruction, 5 for partial obstruction of the intestine and 6 for intestinal fistula (4 to the neobladder, 1 to the urethra, and 1 to the vagina). In 6 patients abdominal pain was caused by recurrence of a pelvic tumor. Seven patients required emergency surgery and 5 elective surgery. CONCLUSIONS: 10.5% of the patients who had undergone radical cystectomy for bladder cancer had at least one episode of abdominal pain requiring admission to hospital. During the first 6 months postoperatively, partial obstruction of the intestine was the most common cause of abdominal pain, and thereafter, complete bowel obstruction and intestinal fistula to the neobladder. Abdominal pain was caused by recurrence of a pelvic tumor in 33% of the patients and by a benign condition in the remaining patients. We found no risk factors related with the preoperative treatment, tumor, urinary diversion procedure or early complications that may predispose to episodes of abdominal pain.

Abdominal Pain↗

[Preventive radical surgery of C-cell carcinoma in MEN-II syndrome based on genetic screening].

Between April 1986 and July 1995 121 patients have been operated on for C-cell carcinoma with 70 (57.9%) patients presenting a sporadic type and 51 (42.1%) a hereditary type of disease (46/38% MEN IIa and 5/4.1% MEN IIb). Indication for operation in patients with familial disease (MEN II) was in 9 patients (18%) detection of a mutation in the ret protooncogen (group I), in 27 patients (53%) a pathologic biochemical screening (pentagastrin stimulation) (group II), and in 15 patients (29%) the first manifestation in a family (index) (group III). Distribution of stages showed a stage I (T1 N0 M0) in 8/9 (89%) in group I, in 17/27 (63%) in group II, and in 0/15 in group III. In 8 from 9 patients with genetic indication a multifocal microcarcinoma and in one patient a cell-cell hyperplasia could be demonstrated. Accordingly the rate of curative operations with postoperative normalization of basal and pentagastrin stimulated calcitonin levels was 100% (9/9) in group I, 59% (16/27) in group II, and 7% (1/15) in group III. The mean age was 14 (median 12) years in group I, 26 (median 24) years in group II, and 43 (median 40) years in group III). In patients with presymptomatic screening (genetic and biochemical) a thyroidectomy including lymph node dissection of the central compartment was performed as a standard procedure. Postoperative complication rate showed a recurrent nerve palsy of 0 in group I and 4% in group II and a hypoparathyroidism of 0 in group I and 4% in group II. The detection of a mutation correlated with positive histological findings of the disease in all patients. The prophylactic radical operation on the basis of a genetic screening proved to be a safe procedure with curative intention. The early age of manifestation underlines the importance of the genetic screening and the early indication for operation.

Adolescent↗