[Treatment of macroglossia due to acromegaly]. / Behandeling van macroglossie ten gevolge van acromegalie.

A 61-years-old woman had macroglossia due to acromegaly with complaints of dyspneu at a lying sleeping position and complaints of speech and dysphagia. At the age of 55 years she was diagnosed with acromegaly induced by a adenoma of the pituitary gland, which had been removed surgically. The treatment of macroglossia included tongue reduction, removal of the remaining mandibular teeth with severe periodontal attachment loss, huge reduction of the residual alveolar ridge, immediate implant insertion, fabrication of implant-supported overdentures, and logopedic treatment. The speech improved significantly and the patient could sleep again in a lying position. Clinically, acromegaly is diagnosed on clinical signs, such as the morphology and the protrusion of the tongue. Often, macroglossia is a secondary symptom of a systemic disease, needing causal treatment. If surgical reduction of the tongue is indicated, also enlargement of the oral cavity should be considered.

Prospective comparison of gastric secretory function after gastrectomy with either Billroth II or Roux-en-Y anastomosis.

In order to delineate the role of enterogastric reflux in changes of postoperative gastric secretory functions, 22 patients with peptic ulcers, who were randomly assigned to partial gastrectomy without vagotomy with either Billroth II or Roux-en-Y anastomosis, were prospectively studied before and 6 months after surgery. Preoperatively, there were no significant differences in gastric secretory functions between the two groups of 11 patients. Postoperatively, median fasting bile acids in the stomach increased in the Billroth II patients from 0.35 to 16.10 mumol/hr (p less than 0.01), but significantly decreased in the Roux-en-Y patients from 0.30 to 0.10 mumol/hr (p less than 0.05), which indicated adequate prevention of enterogastric reflux after the Roux-en-Y procedure. Gastrectomy resulted in significant reductions of median values of basal acid output (4.6 vs 0.6 mmol/hr, p less than 0.01, and 4.2 vs 0.4 mmol/hr, p = 0.02), peak acid output (31.6 vs 4.2 mmol/hr, p less than 0.01, and 38.7 vs 4.5 mmol/hr, p less than 0.01), serum pepsinogen A (121 vs 86 micrograms/L, p less than 0.01, and 92 vs 45 micrograms/L, p less than 0.01), meal-stimulated serum gastrin secretion (1472 vs 199 pM.60 min, p less than 0.0001, and 1017 vs 199 pM.60 min, p less than 0.0001) in the patients with Billroth II and Roux-en-Y anastomosis, respectively. There were, however, no significant differences in gastric secretory parameters between the two groups when studied 6 months after surgery. Therefore it is concluded that after gastrectomy, enterogastric reflux does not affect the secretory function of the gastric remnant within the first 6 months after surgery.

Gastric emptying after partial gastrectomy without vagotomy with primary Roux-en-Y or Billroth II anastomosis.

Several clinical studies have indicated that gastric emptying is delayed in patients with Roux-en-Y biliary diversion with vagotomy. In order to determine whether Roux-en-Y diversion without vagotomy also induces delayed gastric emptying, we have compared the effect of gastrectomy without vagotomy with either Billroth II or Roux-en-Y anastomosis on gastric emptying of a liquid and solid meal in 22 peptic ulcer patients. The emptying half-times (t1/2) for solid food were not significantly different, 54; 24-122 min (median and range) in the 11 patients with Billroth II gastrectomy, 68; 44-189 min in the 11 patients with Roux-en-Y gastrectomy, and 83; 27-114 min in 11 normal control subjects. However, the lag phase was significantly (p less than 0.01) shorter in the patients with Billroth II gastrectomy (5; 0-43 min) and Roux-en-Y gastrectomy (4; 2-12 min) than in the control subjects (10; 4-22 min). The t1/2's for the fluid meal were similar, 7; 4-49 min after Billroth II gastrectomy, 8; 4-32 min after Roux-en-Y gastrectomy, and 9; 3-20 min in the control subjects. In all subjects the lag phase for the fluid meal was very short, ranging from 0 to 3 min. It is concluded that Roux-en-Y diversion per se does not delay gastric emptying in man.

Quality of life after operatively corrected high anorectal malformation: a long-term follow-up study of patients aged 18 years and older.

Fifty-eight patients (median age, 26.0 years; range, 18.1 to 56.9 years) with an operatively corrected high anorectal malformation were evaluated by questionnaire. No patient had normal continence for feces; however, 84% had a socially acceptable defecation pattern. The quality of life (QOL) and general and mental health perception of these patients were evaluated. For social functioning and health perception, items from the medical outcome study (MOS) were used. QOL and health perception were compared with those of the general population. Most aspects of QOL (corrected for age and gender) and mental health did not differ from those of the general population. However, the patient population had lower educational and general health levels (P < .01). Twelve percent felt restricted socially by their handicap, and 24% never had a lasting relationship. Of the patients who had a lasting relationship, 43% noted that the handicap had been disturbing in the relationship. Associated anomalies had no influence on QOL and health perception. QOL, education level, and relationships were affected by fecal incontinence. It is possible that more appropriate psychosocial support, eg, addressing the implications of the handicap on everyday life, would have a positive influence.

Atypical mycobacterial infection of the parotid gland.

A localized atypical mycobacterial infection of the major salivary gland is a rare disease. In this report the cases of three patients with this lesion are presented. The diagnosis was based on the clinical picture, skin testing with specific antigens, bacteriologic culture, and histopathologic findings. The patients were successfully treated by total parotidectomy with facial nerve preservation, which in our opinion is the therapy of choice in localized atypical mycobacterial infections.

Short-term results of gastrectomy with Roux-en-Y or Billroth II anastomosis for peptic ulcer. A prospective comparative study.

Since the Roux-en-Y anastomosis prevents the sequela of postoperative enterogastric reflux after gastrectomy, this approach has been advocated as the primary procedure in patients undergoing gastrectomy for peptic ulcer. We have prospectively followed for 2 years 22 patients, in whom gastrectomy was performed with, at random, either Roux-en-Y (n = 11) or Billroth II (n = 11) anastomosis. Two of the 11 patients who had received the Roux-en-Y procedure had anastomotic ulcers, leading to reresection in one of them. These two patients were found to have the highest values for basal and pentagastrin stimulated gastric acid output. After the Billroth II procedure a single patient had a small anastomotic ulcerative lesion. Apart from differences in intragastric bile acids (p less than 0.0001) and the gastritis activity score (p less than 0.01), no significant differences were found between the patients with Roux-en-Y and Billroth II anastomosis with respect to basal and pentagastrin-stimulated gastric acid secretion, basal, postprandial and bombesin-stimulated serum gastrin secretion, serum pepsinogen A and C concentrations, the serum pepsinogen A/C ratio, postprandial glucose, and for a modified Visick grading. From this small series we conclude that, as compared with the Billroth II-anastomosis, the Roux-en-Y procedure effectively prevents enterogastric reflux, and is associated with a higher gastritis activity score, but not with differences in gastric acid, gastrin, pepsinogens, or Visick grading. Furthermore, inadequate reduction of acid secretion in some patients after the Roux-en-Y procedure may lead to recurrent peptic ulcers.

Long-gap esophageal atresia: experience with reconstruction in 25 patients.

In a series of 183 patients with esophageal atresia in a period of 20 years, 25 had a "long-gap" esophageal atresia. In most of these cases we succeeded in saving the patients' own esophagus but at the cost of one to two years of intensive treatment. The overall survival was 80%. The result compares favorably with the results of esophageal replacement reported in the literature.

Esophageal stricture as a complication in Henoch-Schönlein purpura.

We report on a three-year-old boy with Henoch-Schönlein purpura developing an esophageal stenosis during severe clinical course of this disease. There are to date no reports on the development of such a complication. The possible pathogenesis in relation to stricture formation of ileum and ureter in Henoch-Schönlein's purpura is discussed.

[Acute appendicitis in children: serious complications when treatment is delayed]. / Appendicitis acuta bij kinderen: ernstige complicaties bij vertraging in de behandeling.

Three children, two boys aged 9 and 6 and a 12-year-old girl, had diffuse abdominal complaints, diarrhoea and a (sub)febrile temperature for several days. On admission, they were found to have a perforated inflamed appendix and peritonitis. Following asystole, intra-abdominal abscesses and an enterocutaneous fistula, the oldest boy showed good recovery after a hospital stay of two months; the girl recovered after one month in hospital following a psoas muscle abscess and two episodes of constrictive pericarditis with threatened tamponade. The younger boy was dead on arrival at the hospital. Appendicitis is not always easy to diagnose. An atypical presentation, very often with diarrhoea, can result in diagnostic delay. Early surgical consultation is mandatory in a child with progressive abdominal pain.

[Haemangiomas and congenital vascular malformations: their classification and diagnosis]. / Hemangiomen en congenitale vasculaire malformaties; classificatie en diagnostiek.

Mulliken and Glowacki's classification of peripheral blood- and lymph-vessel abnormalities is based on their clinical course and cellular characteristics, and is therefore clear to and readily usable by the practising physician. In order to make the diagnostic process more accessible, the Haemangiomas and Congenital Vascular Malformations Nijmegen working group has developed a system of diagnostic guidelines on the basis of this classification. The anamnesis should be directed at the following six distinguishing characteristics: presence of the anomaly at birth, growth, involution, change in volume, pain and outflow. The physical examination is directed at the following five characteristics: the possibility of emptying or pushing aside the anomaly, changes in volume during engorgement, murmur/'thrill'/pulsation, phleboliths, and hyper- or hypotrophy. If a diagnosis still cannot be made, then additional investigations may be carried out. Duplex scanning is usually sufficient for this purpose, after which the nature and extent of the malformation can be determined with MRI. On the basis of the results, the persons involved can be informed as to the prognosis of the malformation and a plan of treatment can be proposed.

[A child with traumatic hemobilia]. / Een kind met traumatische hemobilie.

A 4-year-old boy was hit by a car travelling at 40 km/h and was admitted 3.5 h later to the department of paediatric surgery. Because he was haemodynamically unstable and needed blood transfusion, the patient underwent an emergency operation. The liver was ruptured in the right lobe. A large haematoma was found in the serosa of the duodenum, along with a Meckel's diverticle, which was left in place. The liver rupture was covered and sealed. One month after the accident the patient was re-admitted, because of abdominal pain and gastrointestinal bleeding. The cause was thought to be the Meckel's diverticle, which was removed later. Two months after the trauma the patient was re-admitted with abdominal pain, again with haematemesis and melaena. The diagnosis of hemobilia was obtained with MRI and angiography, which revealed a ruptured pseudoaneurysm of the ramus dexter of the proper hepatic artery. The patient was successfully treated with embolization. The diagnostic delay was two months, which illustrates the importance of considering the possibility of the diagnosis hemobilia in case of gastrointestinal haemorrhage combined with biliary symptoms.

[Vesico-intestinal fissure: a multidisciplinary problem]. / Vesico-intestinale fissuur: een multidisciplinair probleem.

Vesico-intestinal fissure or exstrophy of the cloaca is a rare but serious birth defect of the urogenital tract and distal part of the digestive tract. The most important hallmarks are: bladder exstrophy, fusion between bladder and the exstrophied iliocoecal region, short blind-ending colon and imperforate anus. In addition the lower part of the body shows defects of the abdominal wall, symphysis, upper urinary tract, the internal and external genitals, lumbosacral spine, spinal cord and the lower extremities. It is just after 1960 that corrective surgery started to become successful. Because of the complexity of the anomaly it seems that only management by a team of specialists in a centre for pediatric surgery guarantees optimal treatment. The quality of live of these children can still be improved by application of the newest surgical techniques and excellent cooperation between the different involved specialists. The defects, surgical treatment and results in eight patients with vesico-intestinal fissure, seen at the Universitair Centrum voor Chirurgie bij Kinderen Nijmegen (UCCKN) from 1974 till 1989 inclusive, are presented.

A neonate with an intact congenital umbilical appendix: an alternative theory on the etiology of the appendico-umbilical fistula.

Neonatal umbilical anomalies usually represent remains of the vitelline duct or the allantois. We describe a case of an umbilical appendix in a neonate. The vermiform appendix was found to be positioned in the umbilical cord. In a brief literature review we found eight other reports concerning umbilical appendices. In this article we describe a possible embryological explanation for the development of an umbilical appendix, and discuss whether or not the appendiceal umbilical fistulae reported are congenital or iatrogenic. The possible association between an umbilical appendix and different forms of malpositioning and rotation of the gut is also discussed. Protrusion of the neonatal appendix into the umbilical cord represents a different entity of congenital anomalies. It is important to realize that, in the case of an unrecognized umbilical appendix, medical procedures (e.g., canulation or clamping of the umbilicus) may produce an iatrogenic appendico-umbilical fistula. Careful inspection and palpation of the umbilical cord prior to these procedures may prevent a fistula being created. Furthermore, because the possible association between umbilical appendices and different kinds of malpositioning of the gut is so far not wholly elucidated, we recommend further (radiological) investigation in each case of an umbilical appendix. Correct positioning of the bowel needs to be confirmed in order to rule out possible future complications.

Surgical treatment of hemangiomas and vascular malformations in functional areas.

Hemangiomas and vascular malformations (VM) in functional areas can be treated by a variety of methods. Because of the natural involution of hemangiomas, a non-agressive approach is recommended. Active therapy is necessary only in cases where a function is affected such as vision, respiration, hearing, and feeding. Psychological problems can be an indication for early excision, and psychological/cosmetic reasons in the presence of fibrofatty tissue residues when the hemangioma has been involuted for late excision. In contrast to hemangiomas, no involution is to be expected for VMs, so that therapy depends mainly on the occurrence of functional problems and/or serious complications. Surgical excision still has a place, however, the indications are limited.

Progressive pneumoperitoneum as preoperative preparation for large hernial repair in patients with severe chronic obstructive lung disease.

The results of operation in four patients with a large hernia associated with severe chronic obstructive lung disease show the advantages and limitations of a preparatory progressive pneumoperitoneum. If carefully employed, it seems possible to repair very large hernias in patients suffering from severe chronic obstructive lung disease.

Intestinal pseudo-obstruction syndrome in a child with myotonic dystrophy.

Gastrointestinal symptoms in myotonic dystrophy are increasingly observed, but major intestinal movement disorders such as intestinal pseudo-obstruction appear to be an infrequent complication. We describe a 13-year-old boy who, after appendectomy, developed intestinal pseudo-obstruction syndrome as the first clinical manifestation of myotonic dystrophy. He developed several similar episodes thereafter, which responded to conservative measures. When a child with myotonic dystrophy presents with an ileus, the diagnosis of intestinal pseudo-obstruction should be considered and therapy should then be conservative.

Effect of gastrectomy with either Roux-en-Y or Billroth II anastomosis on small-intestinal function.

This prospective study was undertaken in patients scheduled for gastrectomy for peptic ulcer disease to determine the effect of partial gastrectomy with either Roux-en-Y (n = 11) or Billroth II anastomosis (n = 11) on the function of the small intestine. Patients were studied before and at 6 months (blood and small-intestinal function tests) and at 24 months (blood tests) postoperatively. Median postoperative body weights at 6 months (70.5 kg; p less than 0.01) and 12 months (70.3 kg; NS) were lower than preoperatively (73.0 kg). Haemoglobin concentrations at 6 months (8.9 mM; p less than 0.01) and at 24 months (9.1 mM; p less than 0.05) were also significantly reduced compared with the preoperative value (9.5 mM). However, neither at 6 nor at 24 months postoperatively were there significant changes for serum iron, iron saturation, folic acid, vitamin B12, protein, albumin, alkaline phosphatase, and calcium concentrations. Whereas no significant deterioration of the absorption of D-xylose and vitamin B12 or of faecal fat excretion was observed, the orocoecal transit time was significantly shortened from 98 to 50 min (p less than 0.01), the expiratory hydrogen excretion after a 50-g oral glucose load was significantly increased from 8 to 54 ppm (p less than 0.01), as was indicanuria from 257 to 368 mumol/24 h (p less than 0.01). Apart from a lower serum iron concentration and iron saturation index in the Roux-en-Y patients 6 months postoperatively (p less than 0.05), no significant differences between the two types of anastomosis were observed. It is therefore concluded that both in patients with Roux-en-Y and in those with Billroth II anastomosis most abnormalities observed after gastrectomy are secondary to an accelerated small-intestinal transit.

Prospective study of the effect of gastrectomy with and without bile reflux on serum pepsinogens.

In order to prospectively determine the effect of gastrectomy with or without enterogastric reflux on serum pepsinogen concentrations, serum pepsinogen A, serum pepsinogen C and the pepsinogen A:C ratio were measured before, and 10 days and 6, 15 and 24 months after 2/3-3/4 distal gastrectomy in peptic ulcer patients with primary Roux-en-Y diversion (n = 11) or Billroth II reconstruction (n = 11). Gastrectomy induced early decreases in serum pepsinogen A from 100 +/- 12 to 66 +/- 7 micrograms/l (p less than 0.05) and from 111 +/- 11 to 82 +/- 20 micrograms/l (p = 0.05), serum pepsinogen C from 49 +/- 6 to 29 +/- 5 micrograms/l (p less than 0.05) and from 54 +/- 9 to 40 +/- 11 micrograms/l (p = 0.10) in patients with Roux-en-Y and Billroth II gastrectomy, respectively, but did not influence the pepsinogen A:C ratios. Serum pepsinogen A and the pepsinogen A:C ratio continued to decrease 6 months after surgery but no further significant reductions were observed 15 and 24 months postoperatively. Serum pepsinogen C concentrations did not significantly change during postoperative follow-up. Analyses of variances of serum pepsinogen A and the pepsinogen A:C ratio showed that the type of operation had no significant effect on the postoperative course. It is concluded that gastrectomy leads to early decreases in serum pepsinogen A and pepsinogen C levels followed by progressive further reductions of serum pepsinogen A and the pepsinogen A:C ratio for a period of 6 months postoperatively. These postoperative changes of serum pepsinogens are not due to increased enterogastric biliary reflux.