Does tranexamic acid reliably reduce blood loss in proximal femur fracture surgery?

PURPOSE: The aim of our study was to investigate the use of tranexamic acid in patients with proximal femoral fractures and compare the total blood loss, transfusion rates, complications, and the application method. METHODS: A retrospective single center cohort study (level I trauma center) with 1479 patients treated operatively for a proximal femoral fracture between January 2016 and June 2020 was performed. 1 g of tranexamic acid was applied (systemic, topic or combined application). Patient data, surgical procedure, complications, and mortality were assessed. Hemoglobin levels, blood loss and transfusion rates for patients with and without tranexamic acid and the application methods were compared. RESULTS: 667 femoral neck fractures, 701 pertrochanteric and 109 subtrochanteric fractures were included. Mean age was 80.8 years. 274 patients received tranexamic acid. At admission average hemoglobin was 12.2 g/l. Hemoglobin drop postoperatively was less after tranexamic acid (9.72 vs. 9.35 g/dl). Transfusion rates were lowered significantly by 17.1% after tranexamic acid. Blood loss was reduced for all patients after tranexamic acid independent of fracture morphology. The combination of 1 g i.v. and 1 g topical-applied tranexamic acid seems to be more effective. Complication rates did not differ. CONCLUSION: Tranexamic acid is effective in reducing blood loss and transfusion rates, without increasing the risk of thromboembolic events after proximal femoral fractures. For open reduction and nailing and arthroplasty in fracture setting combined topical and single i.v. application seems most effective and closed reduction with nailing can be treated by single dose i.v. application of 1 g tranexamic acid.

A recurrent single-amino acid deletion (p.Glu500del) in the head domain of ß-cardiac myosin in two unrelated boys presenting with polyhydramnios, congenital axial stiffness and skeletal myopathy.

BACKGROUND: Alterations in the MYH7 gene can cause cardiac and skeletal myopathies. MYH7-related skeletal myopathies are extremely rare, and the vast majority of causal variants in the MYH7 gene are predicted to alter the rod domain of the of ß-cardiac myosin molecule, resulting in distal muscle weakness as the predominant manifestation. Here we describe two unrelated patients harboring an in-frame deletion in the MYH7 gene that is predicted to result in deletion of a single amino acid (p.Glu500del) in the head domain of ß-cardiac myosin. Both patients display an unusual skeletal myopathy phenotype with congenital axial stiffness and muscular hypertonus, but no cardiac involvement. RESULTS: Clinical data, MRI results and histopathological data were collected retrospectively in two unrelated boys (9 and 3.5 years old). Exome sequencing uncovered the same 3-bp in-frame deletion in exon 15 (c.1498_1500delGAG) of the MYH7 gene of both patients, a mutation which deletes a highly conserved glutamate residue (p.Glu500del) in the relay loop of the head domain of the ß-cardiac myosin heavy chain. The mutation occurred de novo in one patient, whereas mosaicism was detected in blood of the father of the second patient. Both boys presented with an unusual phenotype of prenatal polyhydramnios, congenital axial stiffness and muscular hypertonus. In one patient the phenotype evolved into an axial/proximal skeletal myopathy without distal involvement or cardiomyopathy, whereas the other patient exhibited predominantly stiffness and respiratory involvement. We review and compare all patients described in the literature who possess a variant predicted to alter the p.Glu500 residue in the ß-cardiac myosin head domain, and we provide in-silico analyses of potential effects on polypeptide function. CONCLUSION: The data presented here expand the phenotypic spectrum of mutations in the MYH7 gene and have implications for future diagnostics and therapeutic approaches.

[Molecular medicine: pathobiochemistry as the key to personalized treatment of inherited diseases]. / Molekulare Medizin: Pathobiochemie als Schlüssel zur personalisierten Therapie vererbter Krankheiten.

Genetic defects are often still regarded as a life-long fate, which one has to cope with. It is true that in many cases an inherited disposition may lead to a severe disease; however, it is also true that the number of genetic defects with a treatment option is continuously increasing and in some of them the onset of disease symptoms can even be totally prevented. Knowledge of the precise molecular pathomechanism is often the basis for a treatment concept. Genome-wide sequencing has tremendously increased the possibility to identify a genetic defect and its broad application has meanwhile made a decisive contribution in routine diagnostics. After identifying a genetic alteration, it is still necessary to investigate the pathobiochemical consequences on the cellular and systemic level. This can be a time-consuming process since not all functional consequences can be immediately recognized. In the case of metabolic defects the treatment strategy can either be a supplementation of missing products or a removal of toxic substrates. The residual function of affected pathways can also often be improved. Recently, the direct correction of the affected genetic defects has become a treatment option for a selected number of diseases. As the first symptoms of disease usually occur early in life, pediatrics has a pioneering role in developing treatment strategies.

Treatment strategy and long-term functional outcome of traumatic elbow dislocation in childhood: a single centre study.

PURPOSE: Traumatic elbow dislocation (TED) is the most common injury of large joints in children. There is an ongoing debate on the optimal treatment for TED. We aimed to assess the functional outcome after operative and nonoperative treatment of TED. METHODS: We analysed the medical records of patients with TED treated at the University Children's Hospital, Basel, between March 2006 and June 2015. Functional outcome was assessed using the Mayo Elbow Performance Score (MEPS) and Quick Disabilities of the Arm, Shoulder and Hand (QuickDASH) Sport and Music Module score. These scores were compared between nonoperatively and operatively treated patients. RESULTS: A total of 37 patients (mean age 10.2 years, 5.2 to 15.3) were included. Of these, 21 (56.8%) children had undergone nonoperative treatment, with 16 (43.2%) patients having had operative treatment. After a mean follow-up of 5.6 years (1.2 to 5.9), MEPS and QuickDASH Sport and Music Module scores in the nonoperative group and operative group were similar: MEPS: 97.1 points (SD 4.6) versus 97.2 points (SD 2.6); 95% confidence interval (CI)-2.56 to 2.03); p = 0.53; QuickDASH Sport and Music Module score: 3.9 points (SD 6.1) versus 3.1 points (SD 4.6); 95% CI 2.60 to 4.17; p = 0.94. We noted no significant differences regarding the long-term functional outcome between the subgroup of children treated operatively versus those treated nonoperatively for TED with accompanying fractures of the medial epicondyle and medial condyle. CONCLUSION: Functional outcome after TED was excellent, independent of the treatment strategy. If clear indications for surgery are absent, a nonoperative approach for TED should be considered. LEVEL OF EVIDENCE: Level III - therapeutic, retrospective, comparative study.

Structure elucidation and quantification of the reduction products of anticancer Pt(iv) prodrugs by electrochemistry/mass spectrometry (EC-MS).

Pt(iv) prodrugs are a class of promising anticancer agents, which are activated by reduction to the active Pt(ii) species. Consequently, the reduction process is a crucial parameter. Herein, a new approach using electrochemistry (EC) coupled to liquid chromatography (LC) and electrospray ionization-mass spectrometry (ESI-MS) or inductively coupled plasma (ICP)-MS was applied. This enabled getting insights into the differences in the reduction and ligand release of platinum(iv) complexes with varying equatorial core structures.

MKS1 mutations cause Joubert syndrome with agenesis of the corpus callosum.

Joubert syndrome (JS) is a clinically and genetically heterogeneous ciliopathy characterized by episodic hyperpnea and apnea, hypotonia, ataxia, cognitive impairment and ocular motor apraxia. The "molar tooth sign" is pathognomonic of this condition. Mutations in the MKS1 gene are a major cause of Meckel-Gruber syndrome (MKS), the most common form of syndromic neural tube defects, frequently resulting in perinatal lethality. We present the phenotype and genotype of a child with severe JS and agenesis of the corpus callosum (ACC). In our patient, a next generation sequencing (NGS) approach revealed the following two variants of the MKS1 gene: first, a novel missense variant [ c.240G > T (p.Trp80Cys)], which affects a residue that is evolutionarily highly conserved in mammals and ciliates; second, a 29 bp deletion in intron 15 [c.1408-35_1408-7del29], a founder mutation, which in a homozygous state constitutes the major cause of MKS in Finland. We review the MKS1-variants in all of the eleven JS patients reported to date and compare these patients to our case. To our knowledge, this is the first patient with Joubert syndrome and agenesis of the corpus callosum where a potentially causal genotype is provided.

Difficulties in recognition of pyruvate dehydrogenase complex deficiency on the basis of clinical and biochemical features. The role of next-generation sequencing.

Pyruvate dehydrogenase complex (PDHc) defect is a well-known cause of mitochondrial disorders (MD) with at least six responsible genes (PDHA1, PDHB, DLAT, DLD, PDHX, PDP1). The aim of this work was to assess the diagnostic value of biochemical methods in recognition of PDHc defect in Polish patients with suspicion of MD. In the first step, Western blot of the E1α subunit was performed on 86 archive muscle bioptates with suspicion of MD. In the second step, Sanger PDHA1 sequencing was performed in 21 cases with low E1α expression. In the third step, 7 patients with negative results of PDHA1 sequencing were subjected to whole-exome sequencing (WES). This protocol revealed 4 patients with PDHA1 and one with DLD mutations. Four additional probands were diagnosed outside the protocol (WES or Sanger sequencing). The molecular characterization of PDHc defect was conducted in a total of 9 probands: 5 according to and 4 off the protocol. Additionally, two affected relatives were recognized by a family study. Altogether we identified seven different PDHA1 changes, including two novel variants [c.464T > C (p.Met155Thr) and c.856_859dupACTT (p.Arg288Leufs*10)] and one DLD variant. The lactate response to glucose load in the PDHA1 subset was compared to a subset of non PDHc-related MD. Opposite responses were observed, with an increase of 23% and decrease of 27%, respectively. The results show that determining lactate response to glucose load and muscle E1α expression may contribute to distinguishing PDHc-related and other MD, however, WES is becoming the method of choice for MD diagnostics.

Multi-scale imaging of anticancer platinum(iv) compounds in murine tumor and kidney.

Nano-scale secondary ion mass spectrometry (NanoSIMS) enables trace element and isotope analyses with high spatial resolution. This unique capability has recently been exploited in several studies analyzing the subcellular distribution of Au and Pt anticancer compounds. However, these studies were restricted to cell culture systems. To explore the applicability to the in vivo setting, we developed a combined imaging approach consisting of laser ablation inductively coupled plasma mass spectrometry (LA-ICP-MS), NanoSIMS and transmission electron microscopy (TEM) suitable for multi-scale detection of the platinum distribution in tissues. Applying this approach to kidney and tumor samples upon administration of selected platinum(iv) anticancer prodrugs revealed uneven platinum distributions on both the organ and subcellular scales. Spatial platinum accumulation patterns were quantitatively assessed by LA-ICP-MS in histologically heterogeneous organs (e.g., higher platinum accumulation in kidney cortex than in medulla) and used to select regions of interest for subcellular-scale imaging with NanoSIMS. These analyses revealed cytoplasmic sulfur-rich organelles accumulating platinum in both kidney and malignant cells. Those in the tumor were subsequently identified as organelles of lysosomal origin, demonstrating the potential of the combinatorial approach for investigating therapeutically relevant drug concentrations on a submicrometer scale.

[Motility disorders of the esophagus]. / Motilitätsstörungen des Ösophagus.

Motility disorders of the esophagus comprise a heterogeneous spectrum of diseases. Primary malformations of the esophagus are now amenable to improved surgical and gastroenterological therapies; however, they often lead to persistent long-term esophageal dysmotility. Achalasia originates from impaired relaxation of the gastroesophageal sphincter apparatus. Systemic diseases may give rise to secondary disorders of esophageal motility. A number of visceral neuromuscular disorders show an esophageal manifestation but aganglionosis rarely extends into the esophagus. The growing group of myopathies includes metabolic and mitochondrial disorders with increasing levels of genetic characterization and incipient emergence of therapeutic strategies. Esophagitis with an infectious etiology causes severe dysmotility particularly in immunocompromised patients. Immunologically mediated inflammatory processes involving the esophagus are increasingly better understood. Finally, rare tumors and tumor-like lesions may impair esophageal motor function.

[Laparoscopic "single-port" appendectomy in children]. / Laparoskopische "Single Port"-Appendektomie im Kindesalter.

BACKGROUND: In contrast to the laparoscopic three trocar-technique and to the single incision laparoscopic surgery (SILS), the "single-port" appendectomy (SPA) requires only one port with one integrated instrument channel. We report on our experience with this half-open surgical technique. PATIENTS / MATERIALS AND METHODS: Between September 2006 and August 2008 a total of 285 children underwent an appendectomy, 265 in SPA technique. Through a 10 mm subumbilical inserted ballon trocar, diagnostic laparoscopy was routinely performed and, afterwards, the appendix was grasped with a 450 mm forceps, exteriorised and dissected outside the abdomen as in open surgery. Patients with perforated appendicitis detected by preoperative ultrasonography were operated by open appendectomy. RESULTS: 94 % of the SPA were performed successfully with no conversion. In six patients (2.3 %), a second trocar was inserted, in seven children (2.6 %), an extension of the incision became necessary. There were three conversions (1.1 %) to open surgery. The median operating time was 50 min and the median length of hospital stay 4 days. Three children had postoperative wound infections (1.1 %). CONCLUSIONS: SPA is a safe alternative to conventional appendectomy techniques, in part also in cases of perforated appendicitis. The minimal scarring guarantees an attractive cosmetic result. The diagnostic laparoscopy enables one to obtain additional information. In the case of extended adhesions, an extension of the incision and / or the use of a second trocar may be helpful.

Leigh disease with brainstem involvement in complex I deficiency due to assembly factor NDUFAF2 defect.

Mitochondrial NADH: ubiquinone oxidoreductase (complex I) deficiency accounts for most defects in mitochondrial oxidative phosphorylation. Pathogenic mutations have been described in all 7 mitochondrial and 12 of the 38 nuclear encoded subunits as well as in assembly factors by interfering with the building of the mature enzyme complex within the inner mitochondrial membrane. We now describe a male patient with a novel homozygous stop mutation in the NDUFAF2 gene. The boy presented with severe apnoea and nystagmus. MRI showed brainstem lesions without involvement of basal ganglia and thalamus, plasma lactate was normal or close to normal. He died after a fulminate course within 2 months after the first crisis. Neuropathology verified Leigh disease. We give a synopsis with other reported patients. Within the clinical spectrum of Leigh disease, patients with mutations in NDUFAF2 present with a distinct clinical pattern with predominantly brainstem involvement on MRI. The diagnosis should not be missed in spite of the normal lactate and lack of thalamus and basal ganglia changes on brain MRI.

PDH E1ß deficiency with novel mutations in two patients with Leigh syndrome.

Most cases of pyruvate dehydrogenase complex (PDHc) deficiency are attributable to mutations in the PDHA1 gene which encodes the E(1)α subunit, with few cases of mutations in the genes for E(3), E3BP (E(3) binding protein), E(2) and E(1)-phosphatase being reported. Only seven patients with deficiency of the E(1)ß subunit have been described, with mutations in the PDHB gene in six of them. Clinically they presented with a non-specific encephalomyopathy. We report two patients with new mutations in PDHB and Leigh syndrome. Patient 1 was a boy with neonatal onset of hyperlactataemia, corpus callosum hypoplasia and a convulsive encephalopathy. After neurological deterioration, he died at age 5 months. Autopsy revealed the characteristic features of Leigh syndrome. Patient 2, also a boy, presented a milder clinical course. First symptoms were noticed at age 16 months with muscular hypotonia, lactic acidosis and recurrent episodes of somnolence and transient tetraparesis. MRI revealed bilateral signal hyperintensities in the globus pallidus, midbrain and crura cerebri. PDHc and E(1) activities were deficient in fibroblasts in patient 1; in patient 2 PDHc deficiency was found in skeletal muscle. Mutations in PDHA1 were excluded. Sequencing of PDHB revealed a homozygous point mutation (c.302T>C), causing a predicted amino acid change (p.M101T) in patient 1. Patient 2 is compound heterozygote for mutations c.301A>G (p.M101V) and c.313G>A (p.R105Q). All three mutations appear to destabilize the E(1) enzyme with a decrease of both E(1)α and E(1)ß subunits in immunoblot analysis. To our knowledge, these patients with novel PDHB mutations are the first reported with Leigh syndrome.

Danon disease: case report and detection of new mutation.

Danon disease is an X-linked disorder resulting from mutations in the lysosome-associated membrane protein-2 (LAMP2) gene. We report a male patient with skeletal myopathy, mental retardation, and massive hypertrophic obstructive cardiomyopathy necessitating heart transplantation. Immunohistochemistry of skeletal muscle and leukocytes, western blot analysis of leukocytes and cardiac muscle, flow cytometry, and DNA sequencing were performed. Muscle biopsy revealed autophagic vacuolar myopathy and lack of immunohistochemically detectable LAMP-2. Diagnosis of Danon disease was confirmed by western blot analysis of myocardial tissue and peripheral blood sample of the patient showing deficiency of LAMP-2 in myocardium and leukocytes. Moreover, absence of LAMP-2 in lymphocytes, monocytes and granulocytes was shown by flow cytometric analysis. Genetic analysis of the LAMP2 gene revealed a novel 1-bp deletion at position 179 (c.179delC) at the 3' end of exon 2, resulting in a frameshift with a premature stop codon.

Lack of complex I is associated with oncocytic thyroid tumours.

Oncocytic tumours are characterised by hyperproliferation of mitochondria. We immunohistochemically analysed all enzymes of the oxidative phosphorylation system in 19 oncocytic thyroid tumours. A specific lack of complex I was detected, which was expressed at <5% of the level determined in surrounding non-cancerous tissue.

Posttherapeutic (131)I SPECT-CT offers high diagnostic accuracy when the findings on conventional planar imaging are inconclusive and allows a tailored patient treatment regimen.

PURPOSE: The purpose of this prospective study was to determine the diagnostic impact and influence on patient treatment of posttherapeutic (131)I SPECT-CT when the findings on planar posttherapeutic whole-body scintigraphy (ptWBS) were inconclusive. MATERIALS AND METHODS: A total of 53 SPECT-CT scans were performed in 41 patients with thyroid cancer after high-dose (131)I therapy (2.944 to 7.526 GBq (131)I) because of diagnostic uncertainty on ptWBS. Physiological uptake in the salivary glands, gastric mucosa, gut, nasal mucosa, urinary tract and liver were considered to be normal. Any other foci of increased (131)I uptake, except iodine uptake clearly located in the thyroid bed, were considered to be abnormal. The data were evaluated on a lesion and a patient basis. RESULTS: Regarding neck lesions, SPECT-CT provided a diagnostic impact in 26/90 lesions (28.9%) and confirmed the diagnosis in 64/90 lesions (71.1%). On a patient basis, SPECT-CT changed N status in 12/33 patients (36.4%), provided a diagnostic impact in 21/33 patients (63.6%) and led to a treatment change in 8/33 patients (24.2%). Regarding lesions distant from the neck, SPECT-CT confirmed the diagnosis in 62/71 lesions (87.3%) and had a diagnostic impact in 9/71 lesions (12.7%). On a patient basis, SPECT-CT changed M status in 4/19 patients (21.1%), had a diagnostic impact in 14/19 patients (73.7%) and led to a treatment change in 2/19 patients (10.5%). Considering all patients, SPECT-CT led to a treatment change in 10/41 patients (24.4%). CONCLUSION: Integrated SPECT-CT is a useful tool, especially in cases of diagnostic uncertainty and helps to individualize patient management.

Evaluation of cast wedging in a forearm fracture model.

BACKGROUND: In paediatric traumatology fractures are commonly treated with a cast. In this course cast wedging is sometimes performed aiming to reduce the fracture angulation. However, the impact of various factors and measures such as cast material, optimal position of the wedge and wrist position were not assessed in a systematic manner. METHODS: A laser supported model was developed to evaluate the biomechanical processes of cast wedging manoeuvre in a model of a distal diaphyseal forearm fracture. Consecutive measurements were performed to find out the influence of wedge position, cast material and wrist position. FINDINGS: The result of the manoeuvre was revealed to be independent of the cast material (plaster of Paris vs. synthetic cast) used. The optimal position for placing the wedge was shown to be on the concave side of the cast at the level of the fracture. The result of a cast wedging manoeuvre in a dorsally displaced forearm fracture can be optimized with the wrist held in extension. INTERPRETATION: The cast wedging model is not a meticulous copy of the human anatomy but it allows some basic studies on cast wedging technique. The results that can be achieved are similar to the experiences of practical paediatric traumatology. Furthermore the present model may be beneficial for use in education and training programs.

Improvement of renal split function in hydronephrosis with less than 10 % function.

PURPOSE: The split function (SF) of hydronephrotic kidneys may dramatically decrease in the presence of severe and persistent obstruction, necessitating surgical correction. The indication for pyeloplasty versus nephrectomy is mainly based on the results of repeated renal ultrasonography (US) and MAG3-diuretic nephrography (DNG) with SF. Nephrectomy is usually recommended if SF is less than 10 %. However, recent studies with long-term follow-up indicate that even with an initial SF of < 10 %, a significant improvement of SF may be seen when pyeloplasty is performed so that nephrectomy may not be justified. PATIENTS AND METHODS: Case histories of 32 children with pre- or postnatally diagnosed severe hydronephrosis were retrospectively analyzed. Surgical correction was indicated if US revealed progredient hydronephrosis (anteroposterior diameter > 20 mm) and/or DNG demonstrated a loss of SF < 40 % and/or severe obstruction, respectively. Postoperative follow-up examinations included renal US after 3, 6 and 12 months, as well as DNG after 12 months. RESULTS: Thirty-two children (25 male, 7 female) underwent Anderson-Hynes pyeloplasty (AHP). At the time of operation, the mean age of the patients was 33 months (1 - 156 months). Patients were divided into 3 groups according to the initial SF: group I, 21 patients with SF > 40 %; group II, 7 patients with moderately impaired SF between 10 - 40 %; group III, 4 patients (aged 1 - 137 months) with a SF of < 10 %. In 2 patients, percutaneous nephrostomy (PCN) was performed, followed by AHP. Thirty patients underwent AHP without preceding PCN. In group III, SF increased from < 10 % preoperatively to 21 %, 27 %, 45 %, and 53 % postoperatively, respectively. In all patients, postoperative DNG demonstrated a significant improvement of SF from 41 %, on average, preoperatively (range 0 - 64 %) to 47 %, on average, postoperatively (range 17 - 60 %). CONCLUSION: Long-term follow-up confirms that the prognosis for renal function is excellent in patients with moderately reduced SF. The significant improvement of SF 12 months after AHP in all patients with a poor SF of less than 10 % supports our approach of performing pyeloplasty in patients even with an initial SF of < 10 %, which is in contrast to common practice.

Hyperbaric oxygen therapy (HBO) for the treatment of an epidural abscess in the posterior fossa in an 8-month-old infant.

Epidural abscesses in children are extremely rare, especially in the posterior fossa. In some cases antibiotic therapy and surgical drainage are insufficient for complete healing. We present the case of an 8-month-old boy who developed an epidural abscess in the posterior fossa after repeated surgical procedures for retrocerebellar arachnoid cysts and hydrocephalus. We decided to use adjuvant hyperbaric oxygen therapy (HBO) to avoid removal of the bone and the existing ventriculoperitoneal shunt. In this way osteomyelitis, potentially leading to bone removal and shunt infection, could be prevented. HBO is a relatively safe, noninvasive and cost-effective therapy to improve healing of chronic and deep-seated wound infections. To our knowledge HBO has never been used before in such a young child in neurosurgery. Multidisciplinary management is recommended to optimize treatment.

Pulmonary function following completion of Minimally Invasive Repair for Pectus Excavatum (MIRPE).

UNLABELLED: Pulmonary function testing remains part of the routine preoperative investigations in patients with pectus excavatum, although there is evidence that reduced exercise capacity is predominantly due to impaired cardiovascular performance rather than ventilatory limitation. AIM OF THE STUDY: To evaluate the change of pulmonary function in patients after completion (metal bar removal) of minimally invasive repair for pectus excavatum compared to the preoperative functional results. PATIENTS AND METHODS: All patients who underwent minimally invasive repair for pectus excavatum (MIRPE) between February 2000 and June 2006 and subsequently had their metal bars removed were examined. Pulmonary function tests were performed as part of the routine pre- and postoperative protocol. Vital capacity (VC), forced expiratory volume in one second (FEV1), total lung capacity (TLC), residual volume (RV) and maximal expiratory flow rate at 25 % (MEF25) were evaluated. In addition, comparisons were made between patients with a low ( 6) pectus severity index (PSI) to establish the relative pre- to postoperative change of the above-mentioned lung function parameters. RESULTS: Fifteen patients were included in the study (14 males, 1 female). The median age at surgery was 15.9 years. The metal bar(s) were removed after a median of 37 months following the initial operation. Lung function tests were performed at a median of 32 days preoperatively and 129 days after completion of the procedure. Preoperative lung function values lay below population averages. The majority of patients had either a restrictive, obstructive or combined pulmonary disorder. Improvement was observed in all lung function parameters after metal bar removal compared to preoperative values and was significant for VC, FEV1 and the RV/TLC ratios. With regard to the severity of the deformity, although not statistically significant, patients with a higher PSI (> 6) appeared to benefit particularly from the operation. CONCLUSION: Our findings indicate that pulmonary function improves in patients after completion of minimally invasive repair of pectus excavatum. However, a review of the literature suggests that, in all probability, following surgery, patients benefit more with regard to enhanced cardiovascular performance than from improved pulmonary limitations. The value of routine testing of pre- and postoperative lung function in patients with pectus excavatum is questionable.